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Gastroenterology Nutrition 26 February 2014 Parenteral nutrition 26 February 2014 Haematology Blood group incompatibilities 26 February 2014 Coagulopathy 26 February 2014 Coagulopathy supporting evidence 26 February 2014 Polycythaemia 26 February 2014 Thrombocytopenia 26 February 2014 Transfusion of red blood cells 26 February 2014 Transfusion of red blood cells supporting evidence 26 February 2014 Vitamin K Infection BCG immunisation 20 February 2014 BCG and unknown maternal HIV status in postnatal ward 20 February 2014 Cytomegalovirus (CMV) 20 February 2014 Cytomegalovirus (CMV) supporting evidence 20 February 2014 Conjunctivitis 20 February 2014 Group B streptococcus disease 20 February 2014 Group B streptococcus disease supporting evidence
20 February 2014 Hepatitis B and C 20 February 2014 Hepatitis B and C supporting evidence 20 February 2014 Herpes simplex 20 February 2014 Human Immunodeficiency Virus (HIV) supporting evidence 20 February 2014 Infection in first 72 hours of life 20 February 2014 Infection in first 72 hours of life supporting evidence 20 February 2014 Infection - late onset 20 February 2014 Infection - late onset supporting evidence 20 February 2014 Methicillin-resistant Staphylococcus Aureus (MRSA) 20 February 2014 Palivizumab 20 February 2014 Palivizumab supporting evidence 13 February 2014 Syphilis - babies born to mothers with positive serology 13 February 2014 TB in pregnancy - investigation and management 13 February 2014 Varicella 13 February 2014 Varicella supporting evidence 13 February 2014
Practical Procedures Arterial line insertion 5 February 2014 Arterial line sampling 5 February 2014 Cannulation
5 February 2014 Chest drain insertion 5 February 2014 Consent 5 February 2014 Exchange transfusion 5 February 2014 Extravasation injuries 5 February 2014 Long line insertion (peripherally sited) 5 February 2014 Long line insertion (peripherally sited) supporting evidence 5 February 2014 Prostaglandin infusion 4 February 2014 Prostaglandin infusion supporting evidence 4 February 2014 Skin biopsy for inborn errors of metabolism 4 February 2014 Skin care 4 February 2014 Skin care supporting evidence 4 February 2014 Transillumination of the chest 4 February 2014 Umbilical artery catheterisation and removal 4 February 2014 Umbilical venous catheterisation and removal 4 February 2014 Venepuncture 4 February 2014 Venepuncture supporting evidence
Renal
Renal failure 4 February 2014 Renal failure supporting evidence 4 February 2014
Respiratory Apnoea and bradycardia 29 January 2014 Chest physiotherapy 29 January 2014 Chest physiotherapy Supporting Evidence 29 January 2014 Chronic lung disease 29 January 2014 Chronic lung disease Supporting Evidence 29 January 2014 CPAP (Continous Positive Airway Pressure) 29 January 2014 HFNC (High Flow Nasal Cannulae) Supporting Evidence 29 January 2014 HFOV (High Frequency Oscillatory Ventilation) 29 January 2014 HFOV (High Frequency Oscillatory Ventilation) Supporting Evidence 29 January 2014 Intubation 21 January 2014 Intubation Supporting Evidence 21 January 2014 Intubation - Difficult 21 January 2014 Intubation - Difficult Supporting Evidence 21 January 2014 Nitric oxide 21 January 2014 Nitric oxide Supporting Evidence 21 January 2014 Oxygen on discharge 21 January 2014 Oxygen saturation targets 21 January 2014
Oxygen saturation targets Supporting Evidence 21 January 2014 Persistent pulmonary hypertension of the newborn (PPHN) 21 January 2014 Persistent pulmonary hypertension of the newborn (PPHN) Supporting Evidence 21 January 2014 Pulmonary haemorrhage 21 January 2014 Surfactant replacement therapy 21 January 2014 Surfactant replacement therapy Supporting Evidence 21 January 2014 Synchronous positive pressure ventilation (SIPPV) 21 January 2014 Transcutaneous CO2 and O2 21 January 2014 Transcutaneous CO2 and O2 Supporting Evidence 21 January 2014 Ventilation (conventional) 21 January 2014 Ventilation (volume-targeted) 21 January 2014 Ventilation (volume-targeted) Supporting Evidence 21 January 2014
Screening Antenatal ultrasound abnormalities 21 January 2014 Bloodspot tests 21 January 2014 Bloodspot test Supporting Evidence 21 January 2014 Cranial ultrasound scans 21 January 2014 Disorders of sexual development 21 January 2014 Examination of the newborn
21 January 2014 Examination of the newborn Supporting Evidence 21 January 2014 Hearing screening 21 January 2014 Hearing screening Supporting Evidence 21 January 2014 Pulse-oximetry (universal) screening 21 January 2014 Retinopathy of prematurity (ROP) 21 January 2014 Retinopathy of prematurity (ROP) Supporting Evidence 21 January 2014
NEW Surgical Guidelines for 2013-15 Recycling of stoma losses via a mucus fistula Supporting Evidence 21 January 2014 Recycling of stoma losses via a mucus fistula 21 January 2014 Rectal washout using syringe method Supporting Evidence 21 January 2014 Rectal washout using syringe method 21 January 2014 Oesophageal atresia/Replogle tubes Supporting Evidence 21 January 2014 Oesophageal atresia/Replogle tubes 21 January 2014 Intra-abdominal cysts Supporting Evidence 21 January 2014 Intra-abdominal cysts 21 January 2014 Inguinal hernia Supporting Evidence 21 January 2014 Inguinal hernia 21 January 2014 Gastrointestinal stomata Supporting Evidence 21 January 2014 Gastrointestinal stomata
21 January 2014 Exomphalos - initial management 21 January 2014 Broviac line insertion 21 January 2014 Ano-rectal malformation in NNU before transfer Supporting Evidence 21 January 2014 Ano-rectal malformation in NNU before transfer 21 January 2014
Developmental Care Environment and Noise on the Neonatal Unit and Supoorting Evidence Kangaroo Care and Supporting Evidence Positioning and Handling and Supporting Evidence Feedback and New Guidelines Admission and Discharge Cardiovascular Cardiac Murmurs and Supporting Evidence Cyanotic Congenital Heart Disease including Hypoplastic Left Heart Syndrome (HLHS) and other duct-dependent lesions diagnosed antenatally and Supporting Evidence ECG Abnormalities and Supporting Evidence Heart Failure and Supporting Evidence Hypotension and Supporting Evidence Patent Ductus Arteriosus (PDA) and Supporting Evidence Pericardiocentesis and Supporting Evidence Critical Care Extreme Prematurity and Supporting Evidence Hypothermia and Supporting Evidence Pain and Stress and Supporting Evidence Preterm Care ('Golden Hour') and Supporting Evidence Resuscitation and Supporting Evidence
Endocrine/Metabolism Hyperglycaemia and Supporting Evidence Hyperkalaemia and Supporting Evidence Hypernatraemic Dehydration and Supporting Evidence Hypoglycaemia and Supporting Evidence Hypothyroidism and Supporting Evidence Intravenous Fluid Therapy and Supporting Evidence Metabolic Disorders (inborn errors of metabolism) and Supporting Evidence Gastroenterology Breastfeeding and Supporting Evidence Breast Milk Expression and Supporting Evidence Breast Milk Handling and Storage and Supporting Evidence Enteral Feeding and Supporting Evidence Gastro-Oesophageal Reflux (GOR) and Supporting Evidence Gastroschisis and Supporting Evidence Jaundice and Supporting Evidence Liver Dysfunction in Preterm Infants and Supporting Evidence Necrotising Enterocolitis (NEC) and Supporting Evidence Nutrition and Supporting Evidence Parenteral Nutrition (PN) and Supporting Evidence
CARDIAC MURMURS
Cardiac murmur detected at routine postnatal examination
Oximetry on right arm and leg Check femoral pulses SpO2 95%
Symptoms Tachypnoea or abnormal breathing pattern Not taking feeds Slow to feed (>30 min) Sweaty or tachypnoeic during feeds Colour change Signs of congenital heart disease Central cyanosis Tachypnoea Other respiratory distress Tachycardia Hyperdynamic precordium Abnormal volume or character of pulse Hepatomegaly Abnormal heart sounds Heart murmur Absence of femoral pulses
SpO2 <95%
Absent femorals
Transfer to NNU for ECG, CXR and echo (if available). Contact cardiologist
Discharge
Abnormal
Inform parents to watch for poor feeding, poor weight gain, breathing difficulties, colour change
barely audible soft but easily audible moderately loud, no thrill louder, with thrill audible with stethoscope barely on the chest audible with stethoscope off the chest
Cyanotic 2011-13
INVESTIGATIONS
Chest X-ray oligaemia/plethora/congenital anomaly classic appearance (e.g. boot shaped heart) is unusual Blood gas including lactate Echocardiogram 4-limb BP (>20 mmHg difference between an upper and lower limb is abnormal) Pre- and postductal saturations (saturation of <95% in both limbs or >3% difference is significant) Nitrogen washout test (carries risk of duct closure: discuss with consultant first) to differentiate between respiratory (parenchymal) and cardiac cause of cyanosis including PPHN baseline saturation (and blood gas if arterial line in-situ) place baby in 100% ambient oxygen for 10 min if there is respiratory pathology, saturations should rise to >95%
IMMEDIATE MANAGEMENT
Resuscitation Call consultant A cardiac baby presenting collapsed and/or cyanosed is a challenging neonatal emergency
Cyanotic 2011-13
Airway Intubate and ventilate all babies presenting profoundly cyanosed or collapsed If apnoea occurs secondary to a prostaglandin infusion, intubate baby but do not alter infusion Maintain ductal patency Open duct with dinoprostone (prostaglandin E2, prostin E2) Start at 5 nanogram/kg/min, may be increased to 40 nanogram/kg/min on advice of cardiologist if dinoprostone not available, use prostaglandin E1 (Alprostadil); see local formulary Make fresh solution every 24 hr Be vigilant: 10% will develop apnoeas on this low dose regimen Ventilation Indications Significant apnoeas Congestive cardiac failure Respiratory failure Technique Avoid hyperventilation, which can increase pulmonary blood flow initial settings: PEEP 45 cm H2O and FiO2 0.21, adjusted accordingly Aim for: PaCO2 57 KPa PaO2 46 KPa pH 7.357.40 SpO2 7590%, although many will run higher in room air Inotropes If signs of peripheral underperfusion persist, arrange local echocardiography (if available) to assess contractility if confirmed, start dobutamine 510 microgram/kg/min by continuous IV infusion if no improvement to peripheral circulation, consider nitroprusside 0.58 microgram/kg/min by continuous IV infusion after discussion with cardiac team Monitor Monitor blood pressure invasively using a peripheral arterial cannula, not UAC Titrate infusion to keep to SpO2 >7590% need to balance pulmonary and systemic circulations: too high an SpO2 compromises LV output and worsens hypotension Assess cardiac output, likely to be low when: tachycardia persists BP remains low acidosis persists lactate elevated or rising poor peripheral perfusion When cardiac output low: ensure prostaglandin infusion adequate ensure adequate intravascular volume correct anaemia inotropes (dobutamine) may be required for tissue oxygen delivery and hypotension (as usually started for elevated lactate and reduced perfusion long before hypotension arises)
Cyanotic 2011-13
Discuss further management and transfer with regional cardiac centre Babies who respond to a prostaglandin infusion do not need to be transferred out-ofhours Appropriately skilled medical and nursing staff are necessary for transfer
Intubation Do not intubate routinely for transfer An intubated baby requires a cardiac centre ITU bed Intubate if: continuing metabolic acidosis and poor perfusion long-distance transfer necessary significant inotropic support in sick baby needed apnoea recommended by cardiac team
HYPOPLASTIC LEFT HEART SYNDROME (HLHS) (AND OTHER DUCT-DEPENDENT LESIONS DIAGNOSED ANTENATALLY) BACKGROUND
Malformation affecting left ventricle, aorta and valves Degree of hypoplasia varies considerably and, in many cases, lesion is dynamic with progression during pregnancy Recently palliative/corrective surgery (Norwood procedure and its modifications) has become increasingly successful in prolonging survival with a view to later heart transplantation
ANTENATAL MANAGEMENT
Record management plan in mothers notes and in antenatal counselling letter (with additional copy of both on neonatal unit) After delivery and stabilisation at local maternity unit, arrange non-urgent transfer to regional cardiac surgery centre for full cardiology assessment If closed or small (restrictive) atrial septum puts fetus at risk of severe pulmonary congestion and hypoxaemia shortly after birth, urgent septostomy immediately after delivery at maternity unit may be recommended, despite a generally poor outcome
Postnatal diagnosis Some babies will present when duct closes, anytime during neonatal period and early infancy, particularly where a left heart lesion has developed later in gestation It is important to consider diagnosis of HLHS in any baby presenting with shock
Cyanotic 2011-13
MANAGEMENT
Aim to maintain patency of (or open a closed) ductus arteriosus, and optimise systemic perfusion In HLHS, single right ventricle supplies blood to systemic and pulmonary circulations . Excess flow in one circulation will lead to a significant decrease in blood supplied to other circulation Greatest risk is excessive blood flow through pulmonary circulation with systemic underperfusion, presenting with: tachypnoea; poor peripheral perfusion; and metabolic acidosis Poor peripheral perfusion can lead to cerebral injury and necrotising enterocolitis (NEC)
Resuscitation Neonatal team must be present at delivery to ensure baby establishes effective respirations and becomes pink Although no increased requirement for resuscitation, it is extremely important to avoid hypoxia and hyperoxia If stable, allow short cuddle with parents before transfer to neonatal unit, but keep warm to avoid hypothermia Admit to unit for routine monitoring and investigations including blood gases and avoid acidosis
FURTHER MANAGEMENT
See Cyanotic congenital heart disease section of this guideline
ECG ABNORMALITIES
SINUS TACHYCARDIA
Recognition and assessment Sinus rhythm (P wave precedes every QRS complex) with a heart rate above normal limit for age and gestation Causes Fever Infection Low haemoglobin Pain Hypovolaemia Hyperthyroidism Myocarditis Drugs (e.g. caffeine and salbutamol) Management Treat the cause
SINUS BRADYCARDIA
Recognition and assessment Sinus rhythm (P wave precedes every QRS complex) with a heart rate below normal limit for age and gestation Differential diagnosis Hypoxia (most likely cause) Vagal stimulation (ET tube touching carina) Post-intubation Hypovolaemia Hypothermia Metabolic derangement Immediate management Manage airway and breathing Adjust ET position, if too low If post-intubation, use atropine (see Neonatal Formulary) Correct hypovolaemia Correct metabolic derangement If persistent, obtain 12-lead ECG
Investigations 12-lead ECG Measure QTc interval on ECG during period of sinus rhythm Echocardiogram to rule out structural abnormality of heart Immediate treatment Seek advice from paediatric cardiologist
SUPRAVENTRICULAR TACHYCARDIA
Recognition and assessment Rapid regular tachyarrhythmia Heart rate >230/min ECG: P waves commonly absent. When present, almost always have a different morphology narrow QRS complex in fast sinus tachycardia, P waves can be very difficult to see look for delta waves consistent with Wolff-Parkinson-White syndrome as this affects the choice of anti-arrhythmic agent used by cardiologists at Birmingham Childrens Hospital (BCH) Symptoms and signs Persistent SVT can cause haemodynamic compromise Investigations 12-lead ECG to document SVT: if not definite SVT, treat for cause of sinus tachycardia (e.g. fluid for hypovolaemia) Immediate management Assess airway, breathing and circulation Check for signs of cardiac failure Vagal manoeuvre such as applying ice pack to face Adenosine IV bolus use central venous access or IV access in a bigger vein (antecubital fossa) connect 3-way connector to end of cannula/catheter establish patency of IV access connect syringe with adenosine to one port and sodium chloride 0.9% flush to another port run ECG strip give adenosine as a quick bolus and push the bolus of sodium chloride 0.9% at the end quickly document change in cardiac rhythm on ECG Adenosine dosage Start with 150 microgram/kg IV bolus if no response, increase by 50 microgram/kg Repeat every 12 min maximum dose 300 microgram/kg If no response, discuss with BCH cardiologists regarding DC shock Subsequent management Echocardiogram to assess ventricular function and presence of congenital heart disease Correct electrolyte and metabolic imbalance, if present Discuss with paediatric cardiology centre for further management or earlier if necessary
VENTRICULAR TACHYCARDIA
Recognition and assessment Heart rate >200/min
Immediate management Manage airway and breathing Correct hypoxia Correct electrolyte disturbance Discuss with paediatric cardiology centre Consider synchronised cardioversion (in very fast heart rates, defibrillators cannot synchronise with the patient and unsynchronised will be required) if intubated, with analgesia Amiodarone 5 mg/kg over 30 min IV (repeat if necessary) If no response, lidocaine 0.51 mg/kg IV. May be repeated after 5 min
TACHYARRHYTHMIA
True heart rate? Is baby crying/in pain? Check airway and breathing Check saturation Consider arterial/capillary gas Check perfusion Check blood pressure Manage airway and breathing Correct hypoxia Correct electrolyte disturbance 12-lead ECG 12 lead ECG
Narrow QRS complex Absent/abnormal P wave vagal manoeuvres adenosine discuss with paediatric cardiologist consider synchronised cardioversion
Broad QRS complex Abnormal P wave discuss with paediatric cardiologist synchronised cardioversion
HEART FAILURE
DEFINITION
Congestive cardiac failure occurs when heart unable to pump sufficient blood to meet metabolic demands of body tissues cause may be cardiac or non-cardiac
Causes Non-cardiac Sepsis Asphyxia Anaemia Polycythaemia Fluid overload AV malformation Bronchopulmonary dysplasia (BPD) Cardiac Left ventricular outflow tract (LVOT) obstruction Left-to-right shunt (see Increased left to right shunt below) Arrhythmia Left ventricular outflow tract obstruction (LVOT) Hypoplastic left heart syndrome Critical aortic stenosis Coarctation Interrupted aortic arch Clinical differentiation between an obstructed systemic circulation and severe sepsis is extremely difficult as a murmur and weak pulses can be common to both. In infant in extremis, presence of abnormal pulses alone is sufficient indication to start a prostaglandin infusion until a cardiac lesion has been excluded by echocardiography
INVESTIGATIONS
Blood gas including lactate Chest X-ray cardiomegaly and pulmonary oedema Echocardiogram Four-limb BP (a difference of >20 mmHg between an upper and lower limb is significant) Pre- and postductal saturations postductal saturations can be considerably lower than preductal in aortic arch defects (a difference of >3% is significant)
IMMEDIATE TREATMENT
If left sided obstructive lesion suspected, treat with inotropes and use diuretics cautiously
Resuscitation Airway Intubate and ventilate babies presenting collapsed or with obvious cyanosis in association with cardiac failure Routine intubation not indicated If apnoea occurs secondary to a prostaglandin infusion, intubate baby but do not alter infusion Breathing See Ventilation guideline Ventilate with PEEP 56 cm Adjust ventilation to maintain: PaCO2 56 kPa pH 7.4 Circulation Vascular access with 2 IV cannulae or umbilical venous catheter (UVC) See Umbilical venous catheterisation guideline Presence of cyanosis and a murmur suggest baby likely to respond to prostaglandin infusion Prostaglandin infusion to maintain ductal patency open duct with dinoprostone (prostaglandin E2, prostin E2), see Neonatal Formulary. Start at 5 nanogram/kg/min, may be increased to 40 nanogram/kg/min, but only on advice of cardiologist alprostadil (prostaglandin E1 can also be used. Start at 5 nanogram/kg/min, may be increased but only on advice of cardiologist Monitor blood pressure invasively using a peripheral arterial cannula, not umbilical arterial catheter (UAC) need to balance pulmonary and systemic circulations: too high an SpO2 compromises LV output and worsens hypotension seek advice from cardiologist
Cardiac output Assess cardiac output, it is likely to be low when: tachycardia persists BP remains low acidosis persists high lactate peripheral perfusion poor ensure prostaglandin infusion adequate When cardiac output low: ensure adequate intravascular volume correct anaemia dobutamine will be required for poor perfusion
Intubation An intubated baby requires a cardiac centre ITU bed: do not intubate routinely for transfer
Intubate if: continuing metabolic acidosis and poor perfusion long-distance transfer necessary inotropic support needed apnoea occurring recommended by cardiac team
MANAGEMENT
If in cardiac failure, give immediate dose of diuretic May require maintenance diuretics (discuss with cardiologist) usually furosemide 1 mg/kg and amiloride 100 microgram/kg orally Discuss with cardiac centre for definitive management and follow-up
Hypotension 2011-13
HYPOTENSION
Hypovolaemia is an uncommon cause of hypotension in the preterm newborn. Excessive volume expansion can increase mortality
DEFINITION
Thresholds for intervention Aim to maintain mean arterial BP >gestational age in weeks If mean BP unavailable or pulse pressure wide (e.g. PDA), aim to maintain systolic BP >gestational age in weeks in first 24 hr, and >30 mmHg thereafter Aim for even higher mean arterial blood pressure in case of persistent pulmonary hypertension of newborn see PPHN guideline
IMMEDIATE TREATMENT
Aim of treatment is to improve organ perfusion, not to correct a BP reading Seek senior advice throughout Transilluminate chest to exclude pneumothorax Fluid Not >10 mL/kg unless there is evidence of fluid/blood loss and hypovolaemia If clinical condition poor, BP very low, or mother has been treated with IV antihypertensive agent, give inotrope (see below) after fluid bolus Which fluid? Use sodium chloride 0.9% 10 mL/kg over 30 min EXCEPT when there is: coagulopathy with bruising: give fresh frozen plasma 10 mL/kg over 30 min (see Coagulopathy guideline ) Acute blood loss: give packed cells 10 mL/kg over 30 min Reassess clinically within 30 min of each bolus
Hypotension 2011-13
If hypotension persists after fluid bolus, seek senior advice start inotropes
Inotropes If evidence of poor perfusion, give dobutamine as initial agent commencing at 10 microgram/kg/min. If no response after 20 min, increase to 15 microgram/kg/min Always use dobutamine as first inotrope for pulmonary hypertension Dobutamine will have now improved organ perfusion, but if mean BP remains low, commence dopamine at 5 microgram/kg/min, increasing by 5 microgram/kg/min increments in 30 min intervals to a maximum of 20 microgram/kg/min where possible, perform echocardiogram to assess response to dopamine at doses >10 microgram/kg/min If hypotensive but peripheral perfusion good or bounding, as in sepsis, consider dopamine as initial inotrope after fluid bolus, as this will cause vasoconstriction that will help alleviate poor vascular tone seen with sepsis
How Dopamine must be given centrally because: can cause tissue injury if given into liver or extravasation injury if given peripherally Dobutamine is best given centrally, although can be given peripherally Continuing hypotension If hypotension persists despite these measures: give hydrocortisone 1 mg/kg IV followed by 1 mg/kg IV 812 hrly for 23 days as necessary seek senior advice throughout Refractive hypotension Seek senior advice before starting adrenaline infusion. Depending on individual circumstances, discuss alternative agents (e.g. noradrenaline, vasopressin) If acidotic with severe hypotension, but not hypovolaemic Give adrenaline 100300 nanogram/kg/min (see Neonatal Formulary for instructions on making up solution) Monitor limb perfusion and urine output If cooling, refer to Cooling guideline and avoid vasoconstrictive agents which further reduce peripheral perfusion DO NOT USE ADRENALINE in cooled infants
MONITORING
If pneumothorax suspected, transilluminate Chest X-ray: if intubated urgent, if respiratory status worsening look for air leak or overinflation Blood gases Check effective delivery of drugs: record volume in syringe hourly check for leaks ensure correct position of umbilical venous catheter (UVC) delivering inotropes
SUBSEQUENT MANAGEMENT
If being given morphine, reduce dose if possible
Hypotension 2011-13
If ventilated, try to reduce mean airway pressure without compromising chest inflation and oxygenation If poor response to above measures: echocardiogram to assess for myocardial dysfunction/congenital heart disease
Weaning inotropes Wean dopamine or dobutamine in 5 microgram/kg/min decrements as tolerated and directed by senior advice
PDA 2011-13
INVESTIGATIONS
Palpation of femoral pulses Pre and post ductal SpO2 monitoring Chest X-ray: cardiomegaly pulmonary plethora Echocardiography not essential but advisable because duct-dependent cardiac lesion can be difficult to detect clinically important, if considering treatment with prostaglandin inhibitor, to assess cardiac anatomy and ductal status
PDA 2011-13
IMMEDIATE TREATMENT
Restrict fluid intake to <150 mL/kg/day Prostaglandin inhibitor to initiate ductal closure Indometacin or ibuprofen follow your units practice Contraindications to indometacin or ibuprofen Renal impairment Urine output <1 mL/kg/hr Platelet count <50 109/L Suspected necrotising enterocolitis Dose If not contraindicated, give loading dose of indometacin 100 microgram/kg IV over 2030 min or ibuprofen 10 mg/kg IV administered in accordance with Neonatal Formulary
SUBSEQUENT MANAGEMENT
Check renal function and platelets before each dose Repeat echocardiogram after completing the course Feeds can be initiated and continued at a routine rate
Monitoring pharmacological treatment Check: urine output >1 mL/kg/day feed tolerance FBC platelets >50 109/L renal profile: an increase in urea and creatinine can be expected after administration of indometacin/ibuprofen but urine output is of more clinical value. Discuss with senior colleague Persistence or recurrence of murmur Does not necessarily indicate return of PDA Echocardiogram sometimes demonstrates a physiological left branch pulmonary stenosis: common after ductal closure and not clinically significant Surgical referral Consider surgical ligation if: pharmacological closure contraindicated or ineffective obvious cardiac compromise it is not possible to extubate baby or there has been post-extubation failure there have been pulmonary haemorrhages or other symptoms suggestive of ductal steal
Pericardial 2011-13
PERICARDIOCENTESIS
INDICATION
Consider drainage of a pericardial effusion only if there is cardiovascular compromise
PERICARDIAL EFFUSION
Causes Neonatal hydrops Extravasation of TPN from migrated long lines Complication of central venous catheters Clinical signs Tachycardia Poor perfusion Soft heart sounds Increasing cardiomegaly Decreasing oxygen saturation Arrhythmias Sudden collapse in baby with long line or umbilical venous catheter (UVC) in situ Investigations Chest X-ray: widened mediastinum and enlarged cardiac shadow Echocardiogram (if available)
EQUIPMENT
Sterile gown and gloves Sterile drapes Dressing pack with swabs and plastic dish 22/24 gauge cannula 510 mL syringe with 3-way tap attached Cleaning solution as per unit policy - suggest aqueous chlorhexidine 0.5%/isopropyl alcohol 70% (Hydrex), diluted 50:50 with Normasol for infants <26 weeks gestation
PROCEDURE
Consent and preparation If time allows, inform parents and obtain consent (verbal or written) If skilled operator available, perform under ultrasound guidance In an emergency situation, the most experienced person present performs procedure without delay and without ultrasound guidance Ensure baby has adequate analgesia Drainage Maintain strict aseptic technique throughout Clean skin around xiphisternum and allow to dry Attach needle to syringe and insert just below xiphisternum at 30 to skin and aiming towards left shoulder Continuously aspirate syringe with gentle pressure as needle inserted. As needle enters pericardial space there will be a gush of blood or air Send aspirated fluid for microbiological and biochemical analysis Withdraw needle
AFTERCARE
Cover entry site with clear dressing (e.g. Tegaderm/Opsite)
EXTREME PREMATURITY
INTRODUCTION
Outcomes for premature babies at borderline of viability generally improve with each additional week of gestational age estimation of gestational age confirmed by ultrasound when carried out in first trimester of pregnancy is generally reliable Discussion with parents, before birth if possible, should precede any action
MANAGEMENT
An experienced paediatrician to be present at delivery of extremely premature babies (<28 completed weeks gestation) and make confirmatory assessment of gestational age and condition of baby
24 weeks gestation Unless baby has a severe abnormality incompatible with any significant period of survival, initiate intensive care and admit to neonatal intensive care unit <24 weeks gestation Discuss with parents national and local statistical evidence for survival in babies with range of disabilities found in this age group explain that statistics indicate most babies born <24 weeks gestation will die Between 24 weeks, 0 days and 24 weeks, 6 days gestation Unless parents and clinicians agree that, in view of babys condition (or likely condition) it is not in his/her best interests to start intensive care, provide full, invasive, intensive care and support from birth and admit to neonatal intensive care unit Between 23 weeks, 0 days and 23 weeks, 6 days gestation Give precedence to parents wishes regarding resuscitation and invasive intensive care treatment. However, when condition indicates baby will not survive for long, clinicians are not legally obliged to proceed with treatment that is wholly contrary to their clinical judgement, if they consider treatment would be futile as a first step, determine whether baby is suffering, whether any suffering can be alleviated, and likely burden placed on baby by intensive care treatment where parents would prefer clinical team to make decision about initiation of intensive care, clinicians must determine what constitutes appropriate care where it has not been possible to discuss a babys treatment with mother and, where appropriate, her partner, before the birth, clinical team should consider offering full invasive intensive care until babys condition and treatment can be discussed with parents Between 22 weeks, 0 days and 22 weeks, 6 days gestation Standard practice should be not to resuscitate a baby and this would normally not be considered or proposed If parents request resuscitation, and reiterate this request, discuss risks and long-term outcomes with an experienced neonatologist before attempting resuscitation and offering intensive care Treating clinicians must all agree that this is an exceptional case where resuscitation is in a babys best interests Below 22 weeks gestation Resuscitation should never occur in routine clinical practice any attempt to resuscitate babies born at this gestational age should take place only within the context of an approved research study When intensive care not given, clinical team must provide palliative care until baby dies
EXTREME PREMATURITY Supporting information Most babies born <24 weeks gestation are expected to die, but outcome improves with each additional week of gestational age? A retrospective, 5 year case-control review of 237 infants with gestational ages < 27 weeks (Genzel-Boroviczeny, 2010) found that, despite successful resuscitation, infants between 23 and 26 weeks had a very poor prognosis for survival when presenting with bradycardia, cyanosis and no respiratory efforts (1-min Apgar=1) at birth. Initiating active treatment for an infant at 23 weeks with bradycardia and apnoea was almost always unsuccessful (with a 1min Apgar score of 1, a male infant at 23 weeks and 500g had a mortality rate of 92%), whereas by 26 weeks gestation, the chances of survival were higher than the probability of death.
.nhs.uk
Genzel-Boroviczeny O, Hempelman J, Zoppelli L, et al. Predictive value of the 1-min Apgar score for survival at 23-26 weeks gestational age. Acta Paediatr 2010;99:1790-4 Evidence Level: III Last amended January 2011
AIM
To stabilise and perform all procedures required by the baby within the first hour of life
BEFORE DELIVERY
Nurses Identify nurse responsible for admission and redistribute existing babies Ensure incubator set up and pre-warmed with humidity set at maximum Check monitor and appropriate connections Set oxygen saturation limits at 9295% Ensure ventilator and NeopuffTM plugged in and checked Ensure appropriate size face masks available Prepare suction and catheters Ensure transport incubator pre-warmed and cylinders full Ensure endotracheal tube (ETT) sizes 2.5 and 3.0 are available Set up trolley for umbilical arterial catheter (UAC) and umbilical venous catheter (UVC) beside incubator Prepare infusion fluids for UAC and UVC Take resuscitation bag and saturation monitor to delivery Doctors/ANNPs Registrar or experienced ANNP is responsible for early care of babies <28 weeks gestation counsel parents appropriate to gestation <26 weeks, discuss delivery with consultant, who will attend whenever possible Prescribe infusions for UAC and UVC Check resuscitaire in delivery suite ensure overhead heater switched on and set to maximum set peak inspiratory pressure (PIP) at 20 cmH2O and FiO2 at 0.21 check saturation monitor and probe available Prepare plastic bag
AFTER DELIVERY
Nurses Keep baby warm with plastic bag and hat Assist with resuscitation Accurate time-keeping including resuscitation and procedures Attach oxygen saturation probe to right hand Assist with ETT fixation Set up transport incubator and transfer baby to it Ensure baby labels in place before transport Ensure midwives have taken cord gases Transfer baby to neonatal unit (NNU) Doctors/ANNPs Competent practitioner, ANNP or middle grade doctor to attend If normal delivery and baby breathing, delay clamping of cord for 12 min providing baby can be kept warm If operative or instrumental delivery, cut cord immediately and take baby to resuscitaire Place baby in plastic bag Cover babys head with appropriate size warmed hat Assess colour tone, heart rate and breathing If baby is breathing regularly, commence CPAP at 5 cmH2O If baby is not breathing regularly, give 5 inflation breaths at 2025 cmH20 using T piece and face mask monitor response by checking colour, breathing and heart rate if baby does not start to breath, ventilate at 20/5 if heart rate not above 100 bpm or falls, observe chest movement and if poor, increase pressures to 25/5 observe chest movement throughout and, if obvious and heart rate good, reduce pressures when heart rate >100 bpm or chest movement seen, check saturation monitor and adjust FiO2 aiming to bring saturations close to NLS guidance If continued IPPV necessary, intubate If unit policy is to give surfactant on labour ward ensure that ETT is not inserted too far and is securely fixed before administering surfactant Check baby once placed in transport incubator and review air entry colour heart rate saturation Complete joint resuscitation record and obtain signature from maternity team Show baby to parents Senior member of staff to talk briefly to parents Transfer baby to NNU
Hypothermia 2011-13
HYPOTHERMIA
DEFINITION
Skin temperature <36.0OC on admission from delivery suite
ASSESSMENT
Babies at risk Preterm <30 weeks gestation Low birth weight <1500 g Sick baby Small for dates Consequences (<36.0OC) Hypoglycaemia Metabolic acidosis Hypoxia with increased oxygen demands Consequences (<35.5OC) Increased metabolic rate Clotting disorders Shock Apnoea Intraventricular haemorrhage Persistent fetal circulation Decreased surfactant production and function Causes of heat loss Radiation: heat lost to cooler objects in the room in cold environment, whether in incubator or not, excessive heat may be lost in excessively hot environment or in direct sunlight, baby could overheat in incubator Conduction: heat lost to cooler surfaces on which baby is placed Convection: heat lost due to drafts Evaporation: heat lost through water evaporating from skin
PREVENTION
Delivery suite Keep room 2328OC and free from draughts, especially when babies are due to be delivered Babies <30 weeks Dry head and put on hat Do not dry remainder of baby Place in polythene bag immediately and keep inside bag until placed in pre-heated prehumidified incubator. Do not cover the polythene bag during transfer Other babies Use pre-warmed towel, dry immediately after delivery Discard towel and wrap in another pre-warmed towel and blanket Ensure room warm enough to enable skin-to-skin contact and early breastfeeding Cover exposed skin with warm blanket Neonatal unit Keep at 2425OC to avoid cooling from radiant heat loss, and misting (condensation) in incubators Keep incubators and cots away from windows to prevent radiation heat loss Nurse babies requiring intensive care in pre-warmed incubator
Hypothermia 2011-13
Babies <29 weeks must have humidification >60% for first 2 weeks Incubator temperature (OC) 35 34 33.5 33.2 33 32.5
Incubator temperature during first 3 days Birth weight (g) 1000 1500 2000 2500 3000 4000
Babies <1000 g require even higher temperatures, occasionally >37OC If babys temperature remains within normal limits for 24 hrs, reduce incubator temperature by 1OC according to babys needs When babys weight reaches about 1600 g, or according to local practice, transfer to open cot Rainout may occur if the difference between temperature in incubator and room temperature is >5OC: ensure room temperature kept at locally agreed level
Babies not at risk of hypothermia If not requiring observation of respiratory status or excessive invasive procedures, babies may be: dressed kept wrapped placed in a cot Mild hypothermia can be managed with the addition of: hats cot lids heated mattresses If babys temperature <36.0OC consider: use of incubator, if available increasing humidity, if appropriate for gestational age bubble wrap
HYPOTHERMIA Supporting Information Placing the baby in a polythene bag prevents heat loss pending transfer to an incubator? A Cochrane systematic review (McCall, 2010) found that Plastic wraps or bags were effective in reducing heat losses in infants < 28 weeks' gestation (4 studies, n = 223; WMD 0.68 C; 95% CI 0.45, 0.91), but not in infants between 28 to 31 week's gestation. McCall EM, Alderdice F, Halliday HL, et al. Interventions to prevent hypothermia at birth in preterm and/or low birthweight infants. Cochrane Database of Systematic Reviews 2010, Issue 3. Art. No.: CD004210 Evidence Level: I Heated mattresses are useful in the treatment of mild hypothermia? A retrospective review (Ibrahim, 2010) studied the occurrence of hypothermia in 105 babies born before, and 124 born after the introduction of heated gel mattresses. Four (3.3%) babies were hypothermic (temperature <36 degrees C) at admission when the mattresses were used, compared to 21 (22.6%) babies during the period it was not (p < 0.001). Hyperthermia (temperature >37 degrees C) rose from 30.1% prior to use of gel mattresses to 49.6% when they were used (p = 0.004). Ibrahim CP, Yoxall CW. Use of self-heating gel mattresses eliminates admission hypothermia in infants born below 28 weeks gestation. Eur J Pediatr 2010;169:795-9 Evidence Level: IV Last amended October 2010
PRINCIPLES OF ANALGESIA
Prophylactic or symptomatic Use stepwise approach with agents of increasing potency
Topical pain relief Lidocaine (plain, not with adrenaline) 1% 0.3 mL/kg maximum per dose effective as a topical anaesthesia
Indications Insertion of a chest drain Insertion of a peritoneal drain Avoid EMLA cream: evidence of efficacy in neonate is inconsistent Paracetamol Orally or rectally: absorbed more quickly if given via oral route Table 2 Route Oral Rectal
Morphine sulphate oral liquid (Oramorph) Particularly useful for chronic pain relief and for terminal care Large doses such as 100 microgram/kg 6-hrly can cause hypotension and respiratory depression
Diamorphine Loading dose for ventilated babies in serious pain, 180 microgram/kg IV over 30 min Maintenance 15 microgram/kg/hr IV infusion Larger doses than this are no more effective but are associated with more adverse effects Effects can be reversed by naloxone 100 microgram/kg IV Morphine Loading dose for sedation whilst ventilated 120 microgram/kg IV over 5 min; for severe pain 240 microgram/kg Maintenance 1020 microgram/kg/hr IV Can cause hypotension, bradycardia and respiratory depression Half-life between 1 and 6 hr: increases with decreasing gestational age Effects can be reversed by naloxone 100 microgram/kg IV as a bolus
SEDATION
Sedation has no analgesic effect. Always use in conjunction with appropriate analgesia Chloral hydrate Can be useful in irritable fatigued child/cerebral irritation Has long half life and can accumulate, leading to hypotension and respiratory depression single dose treatment: 45 mg/kg orally (75 mg/kg for term infants for single procedure, e.g. CT) sustained sedation: 30 mg/kg 6-hrly orally if used regularly, review after 12 days
PRETERM CARE (GOLDEN HOUR) Supporting information Evidence pertinent to this guideline may be found in the supporting information for the following Neonatal Guidelines: Resuscitation Hypothermia Ventilation Cannulation Infection
Naloxone should not be administered to infants whose mothers abuse narcotics? A single case study (Gibbs, 1989) recorded generalised convulsions unresponsive to diazepam in a naloxone-treated baby born to a heroin user maintained throughout her pregnancy on methadone. The authors concluded that, as convulsions due to neonatal abstinence syndrome do not appear until at least 48 hours after birth, the symptoms in this case were due to naloxone administration.
Gibbs J, Newson T, Williams J, et al. Naloxone hazard in infant of opioid abuser. Lancet. 1989;2:15960
Evidence Level: V Should resuscitation be carried out with 21% or 100% oxygen? Guidelines from the Resuscitation Council (see above) give no direction on this question. A systematic review and meta-analysis of 10 studies in a total of 2,133 infants (Saugstad, 2008) found that those given 21% oxygen (n=1,082) had reduced mortality compared to those (n=1,051) given 100% oxygen (RR 0.69; 95% CI 0.54 0.88). A systematic review of 2,011 infants from 7 controlled trials (Rabi, 2007) found a statistically significant reduction in mortality in the room air group at 1 week (OR 0.70, 95% CI 0.50 - 0.98) and at 1 month (OR 0.63, 95% CI 0.42 - 0.94). A randomised study in 44 preterm infants (Ezaki, 2009) found reduced oxidative stress in the group given 21% oxygen, compared to the group given 100% oxygen.
Ezaki S, Suzuki K, Kurishima C, et al. Resuscitation of preterm infants with reduced oxygen results in less oxidative stress than resuscitation with 100% oxygen. J Clin Biochem Nutr 2009;44:111-8 Rabi Y, Rabi D, Yee W. Room air resuscitation of the depressed newborn: a systematic review and metaanalysis. Resuscitation 2007;72:35363 Saugstad OD, Ramji S, Soll RF, et al. Resuscitation of newborn infants with 21% or 100% oxygen: an updated systematic review and meta-analysis. Neonatology 2008;94:176-82
Resuscitation 2011-13
RESUSCITATION
Check equipment daily, and before resuscitation Follow Resuscitation Council UK Guidelines www.resus.org.uk
Cord clamping It is current NLS recommendation that cord is not clamped immediately In uncompromised infants a delay in cord clamping of at least one minute is recommended After discussion in Staffordshire, Shropshire & Black Country resuscitation group it has been decided any time a neonatologist is called for fetal reasons, do not delay cord clamping
ASSESS
Assess colour, tone, breathing and heart rate If baby very floppy and heart rate slow, assist breathing immediately Reassess every 30 sec throughout resuscitation process If help required, request immediately If baby not breathing adequately by 90 sec, assist breathing
CHECK AIRWAY
For baby to breathe effectively, airway must be open To open airway, place baby supine with head in neutral position If very floppy, consider chin support or jaw thrust while maintaining the neutral position
IMMEDIATE TREATMENT
Airway Keep head in neutral position Use T-piece and soft round face mask, extending from nasal bridge to chin Give 5 inflation breaths, sustaining inflation pressure (Table 1) for 23 sec for each breath Give PEEP of 5 cm H2O Begin inflation breaths in air Table 1: Inflation pressure (avoid using pressure higher than recommended) Term infant Preterm infant 30 cm of water 2025 cm of water
No chest movement Ask yourself: Is head in neutral position? Is a jaw thrust required? Do you need a second person to help with airway to perform a jaw thrust?
Resuscitation 2011-13
Is there an obstruction and do you need to look with a laryngoscope and suck with a largebore device? Consider placing oro-pharyngeal (Guedel) airway under direct vision using laryngoscope Is inflation time long enough? if no chest movement occurs after alternative airway procedures above have been tried (volume given is a function of time and pressure), a larger volume can be delivered if necessary by inflating for a longer time (34 sec) Attach saturation monitor to right hand see Saturation monitoring for guidance on saturation targets
Endotracheal intubation Indications Severe hypoxia (e.g. terminal apnoea or fresh stillbirth) Stabilisation of airway Extreme prematurity Congenital diaphragmatic hernia Safe insertion of tracheal tube requires skill and experience If you cannot insert a tracheal tube within 30 sec, revert to mask ventilation Breathing Most babies have a good heart rate after birth and establish breathing by 90 sec if not breathing adequately give 5 inflation breaths, preferably using air at pressures in Table 1 heart rate should rapidly increase as oxygenated blood reaches heart Do not move onto ventilation breaths unless you have a heart rate response OR you have seen chest movement Review assessment after inflation breaths Is there a rise in heart rate? Is there chest movement with the breaths you are giving? If no spontaneous breathing, but chest movement has been obtained, perform 30 sec of ventilation breaths, given at a rate of 30 breaths per min (1 sec inspiration) Table 2: Outcome after 30 sec of ventilation breaths Heart rate Breathing Increases Not started breathing Action
Provide 3040 breaths/min Where available, use PEEP at 5 cm water with T-piece system
<60
If baby is floppy with slow heart rate and there is chest movement, start cardiac compressions with ventilation breaths immediately after inflation breaths Increase inspired oxygen concentration every 30 sec by 30% e.g. 306090% depending on response see Saturation chart
Resuscitation 2011-13
Chest compression Use if heart rate approximately <60 beats/min (do not try and count accurately as this will waste time) Figure 1 Figure 2 Start chest compression only after successful inflation of lungs
Pictures taken from NLS manual and Resuscitation Council (UK) and reproduced with their permission
Ideal hold (figure1/figure 2) Circle chest with both hands so that thumbs of both hands can press on the sternum just below an imaginary line joining the nipples with fingers over babys spine Alternative hold (less effective) Compress lower sternum with fingers whilst supporting babys back. The alternative hand position for cardiac compressions can be used when access to the umbilicus for UVC catheterisation is required, as hands around the chest may be awkward
Resuscitation 2011-13
Action Compress chest quickly and firmly to reduce the antero-posterior diameter of the chest by about one-third, followed by full re-expansion to allow ventricles to refill remember to relax grip during IPPV, and feel for chest movement during ventilation breaths, as it is easy to lose neutral position when cardiac compressions are started Co-ordinate compression and ventilation to avoid competition. Aim for 3:1 ratio of compressions to ventilations, and 90 compressions and 30 breaths (120 events) per min Blood If there is evidence of fetal haemorrhage, consider giving O negative emergency blood Resuscitation drugs Always ask about drugs taken recently by, or given to mother Consider drugs only if there is an undetectable or slow heartbeat despite effective lung inflation and effective chest compression Umbilical venous catheter (UVC) preferred venous access Adrenaline 1:10,000 10 microgram/kg (0.1 mL/kg) IV If this dose is not effective, consider giving 30 microgram/kg (0.3 mL/kg) after sodium bicarbonate has been given Adrenaline should only be given via the ET tube if venous access is taking time to achieve; it should not delay intravenous access and treatment; the dose is 0.51.0 mL/kg of 1 in 10,000. Sodium bicarbonate 4.2% 12 mmol/kg (24 mL/kg) IV (never give via ET tube) Glucose 10% 2.5 mL/kg IV slowly over 5 min Sodium chloride 0.9% 10 mL/kg IV Naloxone Consider only after ventilation by mask or endotracheal tube has been established with chest movement seen and heart beat >100 beats/min If mother has been given pethidine within 24 hr of delivery, give IM naloxone: 100 microgram (0.25 mL) for small prem babies 200 microgram (0.5 mL) for all other babies Do not give naloxone to babies born to mothers who abuse narcotics
WHEN TO STOP
If no sign of life present after 10 min of continuous good quality resuscitation, outlook is poor with few survivors, majority will have cerebral palsy and learning difficulties If no sustained spontaneous breathing 30 min after a heart rate has been established, majority also have poor prognosis Continue resuscitation until a senior neonatologist advises stopping
Resuscitation 2011-13
MONITORING
Saturation monitoring Oxygen monitoring is activated when paediatrician/2nd pair of hands arrives. In the meantime, the person initiating resuscitation carries out all the usual steps in resuscitation Do not stop resuscitation for a saturation probe to be attached Attach saturation monitor to the right hand once five inflation breaths have been given Saturations should spontaneously improve as Table 3 Table 3 Time (min) 2 3 4 5 10 Acceptable pre-ductal saturations (%) 60 70 80 85 90
Air to oxygen If inflation breaths have been successful and chest movement seen but heart rate and/or colour/saturations (if available) not improved: in term babies increase oxygen to 30% in preterm babies increase oxygen to 30% If no response, increase by increments of 30% every 30 sec i.e.: Term air 306090/100% Preterm air 30 6090/100% Reduce oxygen if saturation levels as per Boost recommendations when oxygen saturation levels at 9195% for preterm <30 weeks or >95% for term at 10 min of life (otherwise Resus Council target sats should be followed to stop babies being too pink too early on and being at risk of O2 toxicity) Preterm deliveries >26 weeks gestation do not require routine intubation if respiratory effort good these babies can receive PEEP 5cm H20 via mask ventilation with oxygen supplementation as appropriate on the resuscitaire and PEEP support on transfer to NICU If respiratory effort is poor, at any point, or babys condition deteriorates, intubate and ventilate as per BAPM guidance
DOCUMENTATION
Make accurate written record of facts (not opinions) as soon as possible after the event Record when you were called, by whom and why condition of baby on arrival what you did and when you did it timing and detail of any response by baby date and time of writing your entry a legible signature
COMMUNICATION
Inform parents what has happened (the facts)
Birth
AT
30 sec
ALL
If gasping or not breathing Open airway Give 5 inflation breaths Consider SpO2 monitoring 60 sec
STAGES
Re-assess If no increase in heart rate, look for chest movement
ASK:
If chest not moving Re-check head position Consider 2-person airway control and other airway manoeuvres Repeat inflation breaths Consider SpO2 monitoring Look for a response
Acceptable Pre-ductal SpO2 2 min 3 min 4 min 5 min 10 min 60% 70% 80% 85% 90%
DO
When chest moving If heart rate not detectable or slow (<60/min), start chest compressions 3 compressions to each breath
YOU NEED
Re-assess heart rate Every 30 sec If heart rate not detectable or slow (<60/min), consider venous access and drugs
HELP?
HYPERGLYCAEMIA
DEFINITION
Blood glucose >12 mmol/L, and/or presence of >2+ glycosuria on dipstick testing Do not take sample from an infusion line that has glucose running through it
CLINICAL FEATURES
Dehydration Acidosis ketoacidosis does not occur in babies Poor weight gain Babies at high risk Ill, unstable Preterm (especially <28 weeks) Small for gestational age (<3rd centile) Receiving total parenteral nutrition (TPN) Receiving corticosteroids, caffeine
MONITORING
Twice-daily monitoring Check blood glucose at least twice a day in: unstable or acutely ill babies (RDS, septicaemia, NEC) Daily monitoring Check blood glucose at least once a day in babies: <32 weeks gestation for first week receiving TPN with severe unexpected dehydration or metabolic acidosis with poor weight gain while receiving >120 kcal/kg/day Babies treated with corticosteroids Check urine for glycosuria daily Check blood glucose if >2+ glucose in urine
TREATMENT
Discontinue or decrease medications that worsen hyperglycaemia Suspected infection Hyperglycaemia in baby with previously stable blood glucose may be early indicator of infection After taking appropriate cultures, treat empirically Fluids Correct dehydration by increasing daily fluid intake: use sodium chloride 0.9% If baby receiving >6 mg glucose/kg/min, decrease concentration of glucose infused or replace some of the glucose intake with sodium chloride 0.9% using following formula: glucose infusion rate (mg/kg/min) = % glucose fluid volume (mL/kg/day) divided by 144 these measures often bring blood glucose down substantially hypoglycaemia can occur following overzealous glucose restriction Insulin Consider if: baby remains hyperglycaemic (blood glucose >12 mmol/L), and/or presence of >2+ glycosuria on dipstick testing and when control not obtained with above methods or a higher carbohydrate intake than infant will tolerate is necessary for nutritional reasons
Seek senior advice about commencing insulin therapy See Administration of Actrapid insulin below Follow Neonatal Formulary for insulin infusion rates Check blood glucose concentrations regularly during insulin infusion. Treating clinician to decide on frequency, based on stability of glycaemia If unable to wean off insulin after 2 weeks, transient neonatal diabetes is likely; consult a paediatric endocrinologist
Example Actrapid insulin to be infused at 0.1 mL/hr through a peripheral cannula (including a T adapter) + octopus extension set Total lumen capacity: 0.5 mL + 0.34 mL = 0.84 mL Time delay: 0.84 mL/0.1 mL/hr = 8.4 hr* Lumen capacity of IV sets** IV product Extension set with T adapter SL Ready set Double lumen umbilical catheter Triple lumen extension tube with Bionector (octopus) Product manufacturer Venisytems Vygon Vygon Vygon Product code F89805014 836.201 1274.14 841.364 Priming volume (lumen capacity) 0.5 mL 0.85 mL 0.26 mL 0.34 mL (per lumen)
*It may be possible to reduce delay by priming some or all of the IV assembly with infusion fluid, if safe from interactions with drugs infused simultaneously through same extension set and with permission of the medical team i.e. SpR or consultant only **Information above was obtained from the manufacturers of the IV sets currently in use. If a new product is introduced or a new manufacturer used for an existing product, priming volume may require updating
Evidence Level: IV Treating hyperglycaemia has a beneficial effect on mortality and morbidity? A Cochrane Systematic Review of 2 trials in 47 infants (Bottino, 2009) found the evidence was insufficient to answer this question and called for more and larger trials to be conducted.
Bottino M, Cowett RM, Sinclair JC. Interventions for treatment of neonatal hyperglycemia in very low birth weight infants. Cochrane Database of Systematic Reviews 2009, Issue 1. Art. No.: CD007453
Hyperkalaemia 2011-13
HYPERKALAEMIA
CAUSES
Renal failure: secondary to hypoxic ischaemic encephalopathy (HIE), sepsis and hypotension, or structural abnormalities Cellular injury with potassium release e.g. large intraventricular haemorrhage Very low-birth-weight babies without renal failure (non-oliguric hyperkalaemia) in first 12 48 hr + Excess K in IV solutions Endocrine (congenital adrenal hyperplasia)
INVESTIGATIONS
If sample haemolysed, repeat and send free-flowing venous sample or arterial sample If potassium >6.0 mmol/L, connect to cardiac monitor
IMMEDIATE TREATMENT
Serum potassium >6.0 mmol/L (stable with normal ECG) + Stop all K IV solutions or oral supplements Reconfirm hyperkalaemia Institute continuous ECG monitoring
Hyperkalaemia 2011-13
Serum potassium >7.0 mmol/L without ECG changes As above Give salbutamol 4 microgram/kg IV in glucose 10% over 510 min: effect evident within 30 min but sustained benefit may require a repeat infusion after at least 2 hr if IV access difficult, give nebulized salbutamol 2.5 mg as a single dose (difficult to administer if ventilated and not formally evaluated in neonates) and repeat if necessary Give intravenous insulin 0.5 units/kg in glucose 10% (made up to 2.5 mL and given over 30 min): very effective and has an additive effect with salbutamol Repeat U&E + Repeat insulin infusion as necessary until K <7 mmol/L Monitor blood glucose every 15 min for first 2 hr during and after infusion aim for blood glucose 47 mmol/L Serum potassium >7.5 mmol/L with ECG changes As above, but first institute emergency measures below: give 10% calcium gluconate 2 mL/kg IV over 510 min flush line with sodium chloride 0.9% or preferably use a different line give IV sodium bicarbonate (1 mmol/kg over 2 min) this is effective even in patients who are not acidotic (2 mL of 4.2 % sodium bicarbonate = 1 mmol) Further treatments: discuss with consultant A cation-exchange resin, such as calcium resonium (500 mg/kg rectally, with removal by colonic irrigation 812 hrly, repeat every 12 hr. Dose can be doubled at least once to 1 g/kg in severe hyperkalaemia). Useful for sustained reduction in serum potassium but takes many hours to act and is best avoided in sick preterms who are at risk of necrotising enterocolitis (NEC) If severe hyperkalaemia persists despite above measures in term babies with otherwise good prognosis, contact renal team for consideration of dialysis Exchange transfusion using fresh blood or washed red blood cells provides another + strategy for sustained and reliable reduction in serum K concentration see Exchange transfusion guideline
SUBSEQUENT MANAGEMENT
Recheck serum K 46 hrly; when arrhythmias present with renal failure, monitor hourly Monitor urine output and maintain good fluid balance If urine output <1 mL/kg/hr, unless baby volume depleted, give IV furosemide 1 mg/kg until volume corrected Treat any underlying cause (e.g. renal failure)
+
Evidence Level: II (for inhaled salbutamol) Is rectal calcium resonium a safe treatment in neonates? Intestinal perforation has been reported in infants treated with exchange resin enemas (Grammatikopoulos, 2003; Bennett, 1996), although these may have been spontaneous rather than as a result of the treatment. Nausea and vomiting are common side effects of oral administration, but changing to the rectal route is less effective (Helfrich, 2001). Only one randomised trial of resins in the treatment of hyperkalaemia in neonates has been identified (Hu, 1999). 40 VLBW infants were randomised to receive either glucose/insulin infusion (n=20) or kayexalate resin enema (n=20). Duration of hyperkalaemia was significantly shorter (26.4 +/- 14.9 vs 38.6 +/-13.3 hours) in the insulin group. An appropriately-sized randomised trial is necessary to evaluate the risks and benefits of this treatment in premature infants (Grammatikopoulos, 2003).
Bennett LN, Myers TF, Lambert GH. Cecal perforation associated with sodium polystyrene sulfonate-sorbitol enemas in a 650 gram infant with hyperkalemia. Am J Perinatol 1996;13:167-70 Grammatikopoulos T, Greenough A, Pallidis C, et al. Benefits and risks of calcium resonium therapy in hyperkalaemic preterm infants. Acta Paediatr 2003;92:118-27 Helfrich E, de Vries TW, van Roon EN. Salbutamol for hyperkalaemia in children. Acta Paediatr 2001;90:1213-6 Hu PS, Su BH, Peng CT, et al. Glucose and insulin infusion versus kayexalate for the early treatment of non-oliguric hyperkalaemia in very-low-birth-weight infants. Acta Paediatr Taiwan 1999;40:314-8
Evidence Level: II (For no evidence of benefit of resins over glucose/insulin) Evidence Level: V (For case report evidence of harm from resins)
Do some VLBW infants without renal failure suffer from hyperkalaemia? Both renal and non-renal causes of neonatal hyperkalaemia have been suggested (Singh, 2002), and the cause of the condition is generally held to be multi-factorial (Ditzenberger, 1999). One study of 48 infants (Fukuda, 1989) implicated metabolic acidosis and catabolic state, but another, in 33 infants (Stefano, 1993), found no difference in muscle protein catabolism between 12 infants with hyperkalaemia and 21 without. In a study of 18 VLBW infants (Gruskay, 1988) no differences in renal glomerular function were noted in 8 who developed hyperkalaemia and 10 who did not. Inability to regulate potassium balance, as a result of immature distal tubule function, may result in hyperkalaemia in the absence of renal failure (Mildenberger, 2002; Lorenz, 1997; Matsuo, 1995; Sato, 1995).
Ditzenberger GR, Collins SD, Binder N. Continuous insulin intravenous infusion therapy for VLBW infants. J Perinat Neonat Nurs 1999;13:70-82 Fukuda Y, Kojima T, Ono, A, et al. Factors causing hyperkalemia in premature infants. Am J Perinatol 1989;6:76-9 Gruskay J, Costarino AT, Polin RA, et al. Nonoliguric hyperkalemia in the premature infant weighing less than 1000 grams. J Pediatr 1988;113:381-6 Lorenz JM, Kleinmann LI, Markarian K. Potassium metabolism in extremely low birth weight infants in the first week of life. J Pediatr 1997;131:81-6 Matsuo Y, Hasegawa K, Doi Y, et al. Erythrocyte sodium-potassium transport in hyperkalaemic and normokalaemic infants. Eur J Pediatr 1995;154:571-6 Mildenberger E, Versmold HT. Pathogenesis and therapy of non-oliguric hyperkalaemia of the premature infant. Eur J Pediatr 2002;161:415-22 Sato K, Kondo T, Iwao H, et al. Internal potassium shift in premature infants: cause of nonoliguric hyperkalemia. J Pediatr 1995;126:109-13 Singh BS, Sadiq HF, Noguchi A, et al. Efficacy of albuterol inhalation in treatment of hyperkalemia in premature neonates. J Pediatr 2002;141:16-20 Stefano JL, Norman ME. Nitrogen balance in extremely low birth weight infants with nonoliguric hyperkalemia. J Pediatr 1993;123:632-5
Evidence Level: IV What level of hyperkalaemia should prompt treatment? The criteria on which to treat hyperkalaemia have ranged from 6.8 to 7.5 mmol/L, but 6.5 mmol/L may be a better level at which to begin treatment, as rhythm disturbances are to be expected above 7.0 mmol/L (Grammatikopoulos, 2003). If treatment is not initiated until symptoms appear (or the serum level exceeds 7.0 mmol/L), the potential for success is reduced (Ditzenberger, 1999). Mortality rates may be as high as 80% once arrhythmias have appeared (Singh, 2002).
Ditzenberger GR, Collins SD, Binder N. Continuous insulin intravenous infusion therapy for VLBW infants. J Perinat Neonat Nurs 1999;13:70-82 Grammatikopoulos T, Greenough A, Pallidis C, et al. Benefits and risks of calcium resonium therapy in hyperkalaemic preterm infants. Acta Paediatr 2003;92:118-27 Singh BS, Sadiq HF, Noguchi A, et al. Efficacy of albuterol inhalation in treatment of hyperkalemia in premature neonates. J Pediatr 2002;141:16-20
Hypernatraemia 2011-13
HYPERNATRAEMIC DEHYDRATION
DEFINITION
In breast fed babies Serum sodium >145 mmol/L mild: 146149 mmol/L moderate: 150169 mmol/L severe: >170 mmol/L
AIM
To prevent/treat hypernatraemic dehydration while encouraging breastfeeding Other causes of hypernatraemia Diarrhoea Excessive perspiration Renal dysplasia Obstructive uropathy Osmotic diuresis Diabetes insipidus Idiopathic causes Infection (meningitis, encephalitis) Improperly mixed formula Sodium bicarbonate or sodium chloride administration
PREVENTION
Babies at high risk Born to primiparous women Maternal prolonged second stage of labour >1 hr Caesarean section with delayed initiation of feeding Maternal breast abnormalities (flat, inverted nipples)/surgery Use of labour medications Maternal illness, haemorrhage Preterm <37 weeks Maternal obesity Maternal diabetes Action Identify babies at risk Immediate skin-to-skin contact at birth and breastfeed within 1 hr of life Offer breastfeeding assistance within 6 hr of life Ensure babies feed at least 6 times within 24 hr If baby reluctant to feed, express breast milk (see Breast milk expression guideline) and offer by cup or syringe Calculate required volume of feeds using local guidelines Avoid bottle feeding and dummies Avoid early discharge in at-risk babies Assess baby to ensure feeding adequate assess feeding, number of wet nappies and stools using following table
Hypernatraemia 2011-13
Day Wet nappies Stool 12 >2/day >1/day 34 >3/day >2 /day, changing in colour and consistency 56 >5/day >2/day, yellow in colour Weigh between 72 and 96 hr Refer all who have lost more than 10% weight weight loss % = weight loss (g)/birth weight (g) 100 Symptoms and signs Lethargy Irritability: fussy or unsettled during breastfeeding Prolonged feeding >45 min Delayed change from meconium to transitional stools Demanding <6 feeds in 24 hr Reduced urinary frequency Weight loss Jaundice Tremor Increased tone Fever Doughy skin Seizures (usually during rehydration) Fullness of anterior fontanelle Physical examination can be unremarkable usual signs (sunken fontanelle, reduced skin turgor) of dehydration can be absent Complications Venous and arterial thrombosis Subdural and capillary haemorrhage Cerebral oedema Seizures (especially following rehydration) Apnoea and bradycardia Cognitive and motor deficits Hypertension Cerebral infarction Death Investigations U&E Calcium Total bilirubin Blood glucose CRP Blood culture
Hypernatraemia 2011-13
MANAGEMENT
Mild hypernatraemia
Hypernatraemia 2011-13
Baby admitted to neonatal unit
Signs of shock?
Mild hypernatraemia
Unwell baby No
Well baby
Resuscitate with 10 mL/kg sodium chloride 0.9% Tolerate enteral feed
Put baby to breast Top up with EBM/formula at 100 mL/kg/day via cup or syringe If cup and syringe feeds not tolerated, pass NG tube and administer feeds (breast milk and/or formula milk) If total enteral feeds not tolerated, give IV fluids to make up deficit
Yes
No Start IV fluids 100 mL/kg/day If blood glucose >2.5 mmol, use sodium chloride 0.9% If blood glucose <2.5 mmol, use sodium chloride 0.45% and glucose 5% or 10% Subtract resuscitation fluid from calculated maintenance fluid Wean off formula feeds or IV fluids on to breast or EBM as tolerated by baby
U&E and blood glucose 4-hrly + + Aim for rate of fall in Na of 0.5 mmol/L/hr. If Na falling any faster, reduce rate of rehydration If severe hypernatraemia, contact consultant If low blood glucose, see Hypoglycaemia guideline + If renal failure or Na >170 mmol/L, discuss with paediatric nephrologist Monitor 4-hrly: temperature heart rate blood pressure Keep strict fluid balance chart Monitor weight daily Aim to correct dehydration over 4872 hr or slower in severe cases Do not correct hyperglycaemia with insulin, this can reduce plasma osmolality rapidly and precipitate cerebral oedema Once sufficient EBM, aim to establish breastfeeding and reduce top-up Neurodevelopmental follow-up for all babies with moderate and severe hypernatraemia Encourage mother to continue expressing breast milk in addition to breastfeeding
Evidence Level: III Weighing babies early (72-96 hrs after birth) helps to prevent hypernatraemic dehydration? A study of outcomes pre- and post- the introduction of a policy of weighing newborns at 72-96 hrs after birth (Iyer, 2008) found 60 cases of hypernatraemic dehydration: 23 before and 37 after introduction of the policy. After the policy, there was earlier recognition (median 3 vs 6 days), lower percentage weight loss (11% vs 15%), smaller increase in sodium (147 vs 150 mmol/l), and higher breastfeeding rate at discharge (73% vs 22%) and 8 weeks (57% vs 22%). All the differences were significant (p<0.01). There was one death in the pre-policy group, and none in the post-policy group.
Iyer NP, Srinivasan R, Evans K, et al. Impact of an early weighing policy on neonatal hypernatraemic dehydration and breast feeding. Arch Dis Child 2008;93:297-9
Hypoglycaemia 2011-13
HYPOGLYCAEMIA
DEFINITION
Asymptomatic hypoglycaemia Low blood glucose concentrations are common in preterm and small-for-gestational-age infants No simple correlation between blood glucose concentration and neuroglycopenia Blood glucose >2.0 mmol/L is safe Symptomatic hypoglycaemia Blood glucose <2.6 mmol/L and any of the following symptoms, provided these resolve once hypoglycaemia has been corrected: convulsions abnormal neurological behaviour, including hypotonia and poor response to stimulation apnoea Symptoms cannot be attributed to hypoglycaemia if they persist after adequate treatment. Jitteriness alone does not constitute symptomatic hypoglycaemia
PREVENTION
Keep all infants warm Feed all high-risk infants within 2 hr of birth if possible In all starved high-risk infants, institute IV infusion of glucose 10% (see below)
At-risk infants Perform blood glucose estimation in: Small for dates Preterm Any ill infant Infant of diabetic mother Beckwith-Wiedemann syndrome Haemolytic disease of the newborn Severe fluid restriction Monitor all at-risk infants using near-patient monitoring First 24 hr, 34 hrly Second 24 hr, 46 hrly Then as necessary Babies requiring TPN, at least daily Babies fed enterally, immediately before feeds Always verify a low near-patient test result (<2.6 mmol/L) in a symptomatic baby by sending a sample for laboratory blood glucose estimation
MANAGEMENT
Never use concentrated (>20%) glucose solutions in babies Asymptomatic hypoglycaemia in at-risk infant Correct hypothermia (see Hypothermia guideline) Feeding Increase frequency and/or volume of feeds milk is more beneficial than glucose 10% as it is more energy dense (70 kcal/100 mL vs40 kcal/100 mL) and contains fats that promote ketoneogenesis and glucose uptake Calculate exact amount of milk baby needs and ensure calculated amount given
Hypoglycaemia 2011-13 seek advice from breastfeeding advisor, if available if baby not taking full amount, top-up with nasogastric feeding If breast milk not adequate, top-up feeding via cup with term formula first, then, if next blood glucose still <2.6, preterm formula
IV glucose Give IV glucose if: unable to increase/tolerate feed frequency or volume or intensive feeding does not produce normoglycaemia Give glucose 6 mg/kg/min (glucose 10% at 90 mL/kg/day) by IV infusion Continue enteral feeding during IV infusion Once normoglycaemia achieved, wean from infusion as tolerated Monitor Repeat glucose measurement after 1 hr. If low, check laboratory blood glucose If infant develops symptoms or becomes profoundly hypoglycaemic (<1.1 mmol/L), follow Symptomatic hypoglycaemia protocol (below) immediately Symptomatic hypoglycaemia Aim for blood glucose >2.6 mmol/L IV glucose Give glucose 10% 2.5 mL/kg by IV bolus over 5 min into peripheral vein and follow with an infusion of at least 90 mL/kg/day (~6 mg/kg/min), that may be increased if necessary Continue enteral feeds Monitor and adjust Record whether symptoms respond to treatment within 30 min: very important for definitive diagnosis of symptomatic hypoglycaemia Re-check blood glucose after 30 min. If still low, give another 2.5 mL/kg bolus and increase infusion rate to 120 mL/kg/day (8.3 mg/kg/min) except on day 1 when increase glucose concentration of infusion Record response and repeat glucose measurement in 1 hr. If still low, check blood glucose by laboratory estimation and, if hypoglycaemia confirmed, increase delivered glucose content by increasing either volume (to 150 mL/kg/day), or concentration (12.520%). Glucose 20% is commercially available but please follow local policy (Table 1) Give glucose 12.520% centrally as extremely hypertonic. If UVC used, ensure tip not near liver Failure to respond If >12 mg/kg/min of glucose required to achieve normoglycaemia, hyperinsulinaemic state is very likely. Obtain blood sample at time of hypoglycaemia for simultaneous measurement of: blood glucose plasma insulin and serum C-peptide free fatty acids ketones cortisol growth hormone acylcarnitines collect next passed urine for organic acid analysis Administer glucagon 200 microgram/kg (maximum 1 mg) IM, SC, or IV. Check blood glucose within 30 min of glucagon administration, and houly thereafter until stable If persistent hyperinsulinism suspected, seek advice from paediatric endocrinologist/metabolic paediatrician, and consider early transfer to a unit specialising in the management of such infants
Hypoglycaemia 2011-13
Routine addition of glucose polymers (e.g. Maxijul) not recommended. If decision made to use Maxijul, discuss with paediatric dietitian. Beware risk of necrotising enterocolitis Table 1: Prescription to make up 50 mL of varying concentrations of glucose solution Infusion concentration (%) Volume of 10% glucose (mL) Volume of 50% glucose (mL) 12 47.5 2.5 15 44.0 6.0 17 41.0 9.0 20 37.5 12.5 Step down Once blood glucose normal, wean infant on to milk feeds either continuously or by hourly boluses, followed by normalising to 3-hrly feeds when blood glucose stable
MONITORING
Hypoglycaemic patients: if symptomatic, every 30 min if asymptomatic, every hour continue until normoglycaemic Once normoglycaemic, 34 hrly until 24 hr has elapsed since last hypoglycaemic episode
When to investigate further Persistent recurrent hypoglycaemia, especially in low-risk baby Unexpectedly profound hypoglycaemia in a well baby Hypoglycaemia in association with metabolic acidosis Hypoglycaemia in association with other abnormalities: midline defects micropenis exomphalos erratic temperature control Family history of SIDS, Reyes syndrome, or developmental delay Investigations Paired insulin and glucose estimations while hypoglycaemic (hyperinsulinism confirmed if insulin >10 picomol/L when glucose <2 mmol/L or glucose/:insulin ratio <0.3) Urinary ketones and organic acids Serum C-peptide Plasma cortisol and growth hormone Plasma amino acids Plasma acylcarnitine Free and total carnitines
Symptomatic
Symptomatic
>2.60 mmol/L
>2.0 mmol/L
NO
NO
YES
YES
>2.6 mmol/L
NO
YES
Monitor pre-feed blood glucose and Continue feeding No more blood glucose monitoring
necessary unless symptomatic decrease IV fluids gradually
Hypothyroidism 2011-13
HYPOTHYROIDISM
SCREENING
Perform neonatal screening blood test at 58 days old for: congenital hypothyroidism (CH) phenylketonuria sickle cell disease medium chain acyl-co A dehydrogenase deficiency cystic fibrosis In preterm infants, perform on day 6 and repeat at 3640 weeks gestational age Screening relies on measurement of raised blood spot TSH duty clinical biochemist will notify designated consultant or on-call consultant if blood spot TSH >20 mU/L on first testing or 10 mU/L on two occasions
IMMEDIATE MANAGEMENT
Informing diagnosis If screening test result indicates congenital hypothyroidism, a well-informed healthcare professional (community midwife, neonatal outreach nurse, health visitor or GP) must inform parents face-to-face do not communicate an abnormal result on Friday, Saturday or just before a weekend if consultant meeting cannot be arranged within next 24 hr provide parents with information leaflet congenital hypothyroidism suspected (available from www.newbornbloodspot.screening.nhs.uk/cms.php?folder=2458 Consultant meeting Consultant to arrange to meet parents on same or next day to: explain abnormal result examine baby using screening laboratory proforma as an aide-mmoire look for other abnormalities (10% in CH versus 3% in normal baby), congenital heart disease (pulmonary stenosis, ASD and VSD) is commonest anomaly commence babys treatment stress importance of daily and life-long treatment provide parent information leaflet (available from www.gosh.nhs.uk/gosh_families/information_sheets/congenital_hypothyroidism/congenita l_hypothyroidism_families_booklet.pdf Document discussion and management plan and follow up and send to GP and parents Complete and return data form to clinical biochemist at screening laboratory Obtain further diagnostic tests Baby 1 mL venous blood in heparinised container for FT4 and TSH send repeat dried blood spot card to screening laboratory ultrasound or radionuclide scan of thyroid, preferably within 7 days of starting levothyroxine Mother take 3 mL venous blood from mother into a heparinised container for FT4, TSH and thyroid antibodies
TREATMENT
Start treatment with levothyroxine after obtaining confirmatory blood tests. Do not wait for results unless transient hypothyroidism suspected. Treatment must start before 21 days of age, and preferably by 18 days after discussion with paediatric endocrinologist, consultant may withhold treatment if transient hypothyroidism suspected Starting dose levothyroxine 10 microgram/kg/day with a maintenance regimen to maintain serum FT4 in upper half of normal range, particularly for infants with severe hypothyroidism (e.g. athyrotic, ectopic or those with initial FT4 <5.5 nmol/L)
Hypothyroidism 2011-13
Adjustment required depending on thyroid function test results Tablets are 25 microgram strength it is not necessary to divide tablets for intermediate dose as half life >1 week administer intermediate dose, such as 37.5 microgram, as 25 and 50 microgram on alternate days Crush required levothyroxine dose using tablet crusher (if tablet crusher not available, between 2 metal spoons) and mix with a little milk or water, using teaspoon or syringe do not add to bottle of formula suspensions not advised due to variable bioavailability repeat dose if baby vomits or regurgitates immediately Record date treatment commenced Provide parents with 28 day prescription for levothyroxine Arrange continued prescription with GP, emphasising need to avoid suspensions
FOLLOWUP
Arrange follow up after commencement of hormone replacement therapy as follows: 2 weeks, 6 weeks, 3 months, 6 months, 9 months, 1 yr, 18 months, 2 yrs, 30 months, 3 yr, yearly thereafter At each clinic visit: physical examination, including height, weight and head circumference developmental progress blood sample for thyroid function test (FT4, FT3 and TSH, just before usual daily medication dose) request as FT4 priority, then TSH
Interpretation of thyroid function test results Analyte FT4 (pmol/L) Age 05 days 514 days 14 days2 yr 014 days 15 days2 yr Concentration 1752 1230 1225 110 3.68.5
TSH (mU/L)
Aim for FT4 towards upper limit of normal range at higher concentrations of FT4, normal concentrations of T3 (produced by peripheral conversion) are achieved if FT4 concentration satisfactory but with significantly raised TSH, consider noncompliance TSH concentration does not always normalise under 6 months and may be slightly raised up to 3 yr of age in absence of non-compliance, probably due to reset feedback mechanism Overtreatment may induce tachycardia, nervousness and disturbed sleep patterns, and can produce premature fusion of cranial sutures and epiphyses
AFTERCARE
Reassure parents that baby will grow into healthy adult with normal intelligence Stress importance of regular treatment. As half-life is long, it is not necessary to give an extra tablet next day if a day's treatment missed Give details of: British Thyroid Foundation, 2nd floor, 3 Devonshire Place, Harrogate HG1 4AA 01423 709707/709448 http://www.btf-thyroid.org/ www.bsped.org.uk
Screening In preterm infants, blood testing should be performed on day 6 and repeated at 36-40 weeks gestational age? Revised UK guidelines published in April 2005 (see above) made this recommendation, but with the proviso that it should be kept under review. A study (Korada, 2008) compared baseline readings of thyroid stimulating hormone (TSH) in 2238 preterm infants with second samples taken from 2039 infants. No infant with a normal TSH concentration on first sampling was found to have a reading of > 10mU/l on second sampling. The authors concluded that repeat sampling may not be required with a lower screening threshold of 6 mU/l.
Korada M, Pearce MS, Ward Platt MP, et al. Repeat testing for congenital hypothyroidism in preterm infants is unnecessary with an appropriate thyroid stimulating hormone threshold. Arch Dis Child Fetal Neonatal Ed 2008;93:F286-8
Evidence Level: IV Immediate management Infants with congenital hypothyroidism have an increased incidence of other abnormalities? A study of registry data in the US (Kumar, 2009) showed that cchildren with congenital hypothyroidism had a significantly increased risk of congenital renal and urological anomalies (OR 13.2; 95% CI 10.6-16.5). The other significantly increased defects in congenital hypothyroidism were cardiac, gastrointestinal, and skeletal. Analysis of matched data confirmed an increase of congenital renal and urologic anomalies (OR 4.8; 95% CI 3.7-6.3).
Kumar J, Gordillo R, Kaskel FJ, et al. Increased prevalence of renal and urinary tract anomalies in children with congenital hypothyroidism. J Pediatr 2009;154:263-6
Evidence Level: IV Treatment A starting dose of 10 mcg/kg/d of thyroxine is appropriate? Do higher dose regimens result in adverse effects on memory, attention or behaviour? What constitutes optimal TH therapy is not yet certain (Rose, 2006). This dose is at the lower end of the range recommended by current American Academy of Pediatrics guidelines (Rose, 2006). These advise a starting dose of 10-15 mcg/kg/d, depending on the severity of the initial hypothyroidism. When a higher starting dose (12-17 mcg/kg/d) is used, serum T4 normalises in 3 days and TSH returns to the target range within 2 weeks (Bakkar, 2002). However, evaluation of cognitive outcome is important after use of this increased dose (Rose, 2006). A cohort based follow up study of 49 young adults with early treated congenital hypothyroidism compared these with 41 matched sibling controls (Oerbeck, 2005). At age 20, those subjects
given high dose (>/= 7.8 mcg/kg/d) therapy displayed no adverse effects on higher order cognitive skills, compared to those on low dose (<7.8 mcg/kg/d) treatment. The high dose group did, however, exhibit significant differences on some measures of memory, attention (distractibility) and behaviour. The authors concluded that their findings supported the use of higher dose treatment, but acknowledged that only 12 of their 49 subjects had been given doses of >10 mcg/kg/d, and that definite answers to the outcome in high dose treatment groups await further studies. The largest study to date looking at these outcomes was a systematic review of 14 cohort studies in 1321 patients (Hrytsiuk, 2002).This concluded that The evidence for an effect of starting doseon cognitive development, growth, or behavior is too weak to justify recommendations in favor of high- or standard-dose regimens. The most severely hypothyroid infants are at risk for a 5-20 point decrease in IQ, and may benefit from a starting dose of 12-17 mcg/kg/d (LaFranchi, 2007). A Cochrane Systematic Review of a single trial in 47 infants (Ng, 2009) concluded that there was insufficient evidence with which to answer this question.
Bakkar B, Kempers MJ, DeVijlder JJ, et al. Dynamics of the plasma concentrations of TSH, FT4 and T3 following thyroxine supplementation in congenital hypothyroidism. Clin Endocrinol 2002;57:529-37 Hrytsiuk I, Gilbert R, Logan S, et al. Starting dose of levothyroxine for the treatment of congenital hypothyroidism: a systematic review. Arch Pediatr Adolesc Med 2002;156:485-91 LaFranchi SH, Austin J. How should we be treating children with congenital hypothyroidism? J Pediatr Endocrinol Metab 2007;20:559-78 Ng SM, Anand D, Weindling AM. High versus low dose of initial thyroid hormone replacement for congenital hypothyroidism. Cochrane Database of Systematic Reviews 2009, Issue 1. Art. No.: CD006972 Oerbeck B, Sundet K, Kase BF, et al. Congenital hypothyroidism: no adverse effects of high dose thyroxine treatment on adult memory, attention, and behaviour. Arch Dis Child 2005;90:132-7 Rose SR, Brown RS, Foley T, et al. Update of newborn screening and therapy for congenital hypothyroidism. Pediatrics 2006;117:2290-303
Evidence Level: II (for no early administration of sodium) What is the evidence for appropriate volume replacement on day 1,2,3, etc? A Cochrane review of 4 trials involving a total of 414 infants (Bell, 2001) advises that fluid be restricted to amounts that meet physiological needs without allowing significant dehydration. This would typically result in a significantly lower risk of mortality (RR 0.52, 95% CI 0.28-0.96). This amount must be flexible, taking into account ambient humidity and gestational/postnatal age, but would be in the range of 30-60 ml/kg/day plus estimated insensible water loss (Armon, 2008; Modi, 2004). Given adequate hydration, stepwise increments on subsequent days following birth should not be necessary unless accompanied by a clinically relevant increase in nutrition (Modi, 2004).
Armon K, Riordan A, Playfor S, et al. Hyponatraemia and hypokalaemia during intravenous fluid administration. Arch Dis Child 2008;93:285-7 Bell EF, Acarregui MJ. Restricted versus liberal water intake for preventing morbidity and mortality in preterm infants. The Cochrane Database of Systematic Reviews 2001, Issue 3. Art. No.: CD000503 Modi N. Management of fluid balance in the very immature neonate. Arch Dis Child Fetal Neonatal Ed 2004;89:F108-11
Evidence Level: I Can chronic lung disease, necrotising enterocolitis or patent ductus arteriosus (PDA) be caused by fluid overload, rather than inappropriate sodium supplementation? There is evidence that a positive water and sodium balance with expansion of the extracellular space in preterm infants increases morbidity (Bell, 2001). In this Cochrane Review, restricted water intake significantly reduced the risk of PDA (RR 0.40, 95% CI 0.26-0.63, NNT 5.3) and necrotising enterocolitis (RR 0.30, 95% CI 0.13-0.71, NNT 11.9), although not of chronic lung disease (RR 0.80, 95% CI 0.56-1.14). A recent review (Lorenz, 2004) concluded that, based on a metaanalysis of 3 RCTs, higher fluid intakes did not significantly increase the risk of chronic lung
disease. A further retrospective study in 204 extremely low birth weight infants (Stephens, 2008) confirmed the association of high fluid intake (>170 ml/kg(-1)/day(-1)) with increased risk of PDA on day 2 (OR 1.014; 95% CI 1.001 1.028) and day 3 (OR 1.022; 95% CI 1.004 1.040). Findings from 2 RCTs on sodium supplementation were contradictory.
Bell EF, Acarregui MJ. Restricted versus liberal water intake for preventing morbidity and mortality in preterm infants. The Cochrane Database of Systematic Reviews 2001, Issue 3. Art. No.: CD000503 Lorenz JM. Fluid and electrolyte therapy and chronic lung disease. Curr Opin Pediatr 2004;16:152-6 Stephens BE, Gargus RA, Walden RV, et al. Fluid regimens in the first week of life may increase risk of patent ductus arteriosus in extremely low birth weight infants. J Perinatol 2008;28:123-8
Evidence Level: I Should infants receiving phototherapy be given extra fluids? Earlier studies of phototherapy (e.g. Wu, 1985) showed increased insensible water loss during the process. This led to recommendations for fluid supplementation in infants undergoing phototherapy, and a survey in 1996 (Hansen, 1996) recorded 74% of responding neonatal ICUs following this policy. Later studies have produced contradictory results, however, with some suggesting that the earlier findings may have been due to heat stress and that phototherapy in a thermally stable infant does not increase fluid loss (Kjartansson, 1992i &ii), and another recording a 20% increase in transepidermal water loss despite tight control of both skin temperature and relative humidity (Grunhagen, 2002). If these results are accepted, an increase in maintenance fluids of 0.35 mL/kg/h is indicated to correct the deficit. Two further examples of the more recent studies (Maayan, 2001; Wananukul, 2001) agree with Grunhagen that fluid loss is increased, even in thermally stable infants. A randomised study in 74 term neonates with severe hyperbilirubinaemia (Mehta, 2005) found that fluid supplementation decreased the rate of exchange transfusion (RR 0.30; 95% CI 0.140.66) and the duration of phototherapy (52 +/- 18 hours vs 73 +/- 31 hours; P = .004).
Grunhagen DJ, de Boer MG, de Beaufort AJ, et al. Transepidermal water loss during halogen spotlight phototherapy in preterm infants. Pediatr Res 2002;51:402-5 Hansen TW. Therapeutic approaches to neonatal jaundice: an international survey. Clin Pediatr 1996;35:309-16 Kjartansson S, Hammarlund K, Sedin G. Insensible water loss from the skin during phototherapy in term and preterm infants. Acta Paediatr 1992;81:764-8 Kjartansson S, Hammarlund K, Riesenfeld T, et al. Respiratory water loss and oxygen consumption in newborn infants during phototherapy. Acta Paediatr 1992;81:769-73 Maayan MA, Yosipovitch G, Hadad E, et al. Transepidermal water loss and skin hydration in preterm infants during phototherapy. Am J Perinatol 2001;18:393-6 Mehta S, Kumar P, Narang A. A randomized controlled trial of fluid supplementation in term neonates with severe hyperbilirubinemia. J Pediatr 2005;147:781-5 Wananukul S, Praisuwanna P. Transepidermal water loss during conventional phototherapy in nonhemolytic hyperbilirubinemia term infants. J Med Assoc Thai 2001;84(Suppl 1):S46-S50 Wu PY, Hodgman JE, Kirkpatrick BV, et al. Metabolic aspects of phototherapy. Pediatrics 1985;75:427-33
IV Fluids 2011-13
MONITORING
Weigh On admission Daily for intensive care babies: twice daily if fluid balance is a problem use in-line scales if available Serum sodium Daily for intensive care babies If electrolyte problems or <26 weeks, measure twice daily admission electrolytes reflect maternal status: need not be acted upon but help to interpret trends serum urea not useful in monitoring fluid balance: reflects nutritional status and nitrogen load Serum creatinine Daily for intensive care babies Reflects renal function over longer term trend is most useful tends to rise over first 23 days gradually falls over subsequent weeks absence of postnatal drop is significant Urine output Review 8-hrly for intensive care babies 24 mL/kg/hr normal hydration <1 mL/kg/hr requires investigation except in first 24 hr of life >67 mL/kg/hr suggests impaired concentrating ability or excess fluids
NORMAL REQUIREMENTS
Humidification If <29 weeks, humidify incubator to at least 60% If ventilated or on CPAP ventilator, set humidifier at 39OC negative 2 to ensure maximal humidification of inspired gas
IV Fluids 2011-13
Normal fluid volume requirements Fluid volume (mL/kg/day) Day of life 1 2 3 4 <1000 g 90 120 150 150 1000 g 60 90 120 150
Day 1 glucose 10% Day 2 glucose 10% and potassium 10 mmol in 500 mL if birth weight <1000 g, use TPN (with potassium 2 mmol/kg/day) add sodium only when there is diuresis, or weight loss >6% of birth weight Day 3 glucose 10%, sodium chloride 0.18% and potassium 10 mmol in 500 mL or TPN (with potassium 2 mmol/kg/day and sodium 4 mmol/kg/day) After Day 4 glucose 10% (with maintenance electrolytes adjusted according to daily U&E) or TPN Fluid volume requirements are a guide and can be increased faster or slower depending on serum sodium values, urine output and changes in weight Infants receiving phototherapy may require extra fluids depending on type of phototherapy
IV Fluids 2011-13
Excessive IV fluids and failure to excrete fetal ECF Management Reduce fluid intake to 75% of expected Inappropriate ADH Clinical features Weight gain, oedema, poor urine output Serum osmolality low (<275 mOsm/kg) with urine not maximally dilute (osmolality >100 mOsm/kg Management Reduce fluid intake to 75% of expected Consider sodium infusion only if serum sodium <120 mmol/L Risk of accidental hypernatraemia when using 30% sodium chloride. Use with caution and always dilute before use Acute renal failure Management Reduce intake to match insensible losses + urine output Seek advice from senior colleague Excessive renal sodium losses Management If possible, stop medication (diuretics, caffeine) that causes excess losses Check urinary electrolytes + Calculate fractional excretion of sodium (FE Na %): + FE Na = [(urine Na plasma creatinine)/(urine creatinine plasma Na)] 100% normally <1% but in sick preterm infants can be up to 10% affected by sodium intake: increased intake leads to increased fractional clearance if >1%, give sodium supplements Calculate sodium deficit = (135 plasma sodium) 0.6 weight in kg replace over 24 hr unless sodium <120 mmol/L or symptomatic (apnoea, fits, irritability) initial treatment should bring serum sodium up to about 125 mmol/L Use sodium chloride 30% (5 mmol/mL) diluted in maintenance fluids
Adrenal insufficiency Clinical features Hyperkalaemia Excessive weight loss Virilisation of females Increased pigmentation of both sexes Ambiguous genitalia Management Seek consultant advice
IV Fluids 2011-13
Inadequate intake Clinical features Poor weight gain and decreased urinary sodium Management Give increased sodium supplementation If taking diuretics, stop or reduce dose Excessive sodium intake leading to water retention Clinical features Inappropriate weight gain Management Reduce sodium intake
IV Fluids 2011-13
Compositions of commonly available solutions FLUID Na mmol/L Sodium chloride 0.9% 150 Glucose 10% Glucose 10%/sodium 30 chloride 0.18% Albumin 4.5% 150 Sodium chloride 0.45% 75
K mmol/L 1 -
Cl mmol/L 150 30 75
Useful figures Sodium chloride 30% = 5.13 mmol/mL each of Na and Cl Sodium chloride 0.9% = 0.154 mmol/mL each of Na and Cl Potassium chloride 15% = 2 mmol/mL each of K and Cl Calcium gluconate 10% = 0.225 mmol/mL of Ca Sodium bicarbonate 8.4% = 1 mmol/mL each of Na and bicarbonate Sodium chloride 0.9% 1 mL/hr = 3.7 mmol Na in 24 hr Osmolality Serum osmolality = 2(Na + K) + glucose + urea (normally 285295 mOsmol/kg) + + Anion gap = (Na + K ) (Cl + HCO3) normally 717 mmol/L Normal urine: osmolality 100300 mOsmol/kg, specific gravity 10041015 Neonates can dilute urine up to 100 mOsmol/kg, but can concentrate only up to 700 mOsmol/kg Glucose To make glucose 12.5%, add 30 mL of glucose 50% to 470 mL of glucose 10% To make glucose 15%, add 60 mL of glucose 50% to 440 mL of glucose 10% Glucose 20% is commercially available Glucose 10% with sodium chloride 0.18% and 10 mmol potassium chloride is not commercially available but can be made up using 3 mL sodium chloride 30% and a 500 mL bag of glucose 10% with 10 mmol potassium chloride
Metabolic 2011-13
METABOLIC DISORDERS
(INBORN ERRORS OF METABOLISM) RECOGNITION
Early recognition of inborn errors of metabolism (IEM) and prompt management are essential to prevent death or neurodisability diagnosis of IEM in neonates is often delayed owing to non-specific nature of clinical presentation, and unfamiliarity with diagnostic tests seek advice from local and regional clinical chemistry services
Consider inborn errors of metabolism at same time as common acquired conditions, such as sepsis Differential diagnosis Presentation Encephalopathy without metabolic acidosis Common conditions Urea cycle disorders Maple syrup urine disease (MSUD) Zellwegers syndrome Non-ketotic hyperglycinaemia (NKHG) Molybdenum cofactor deficiency Organic acidaemias (propionic, methylmalonic, isovaleric) Glutaric acidaemia type II Galactosaemia Tyrosinaemia Neonatal haemochromatosis Zellwegers syndrome a 1-antitrypsin deficiency Smith-Lemli-Opitz syndrome Fatty acid oxidation disorders (MCAD) Congenital disorders of glycosylation CDG 1b Fatty acid oxidation disorders Galactosaemia Glycogen storage disorders Fructose-1, 6-bisphosphatase deficiency With raised plasma lactate pyruvate dehydrogenase (PDH) deficiency pyruvate carboxylase deficiency respiratory chain disorders organic acidaemias With normal plasma lactate organic acidaemias GM1 gangliosidosis Mucopolysaccharidosis type VII and IV Gauchers Niemann-Pick A & C I-Cell disease MSUD Isovaleric acidaemia or Glutaric acidaemia type II
Liver dysfunction
Hypoglycaemia
Metabolic acidosis
Non-immune hydrops
Metabolic 2011-13
Cataracts
Dislocated lens Congenital anomalies hypotonia, epicanthal folds, Brushfield spots, simian creases, large fontanelle, renal cysts hypertelorism, low set ears, high forehead, abdominal wall defects, large kidneys appearance similar to fetal alcohol syndrome facial dysmorphism, cleft palate, poly- or syndactyly, congenital heart disease Agenesis of corpus callosum Apnoea or periodic breathing in term infant Respiratory alkalosis in a tachypnoeic baby Jaundice (particularly conjugated) and liver dysfunction Hypoglycaemia in a low-risk infant, or persistent/recurrent, with neurological symptoms Metabolic acidosis with increased anion gap Persistent vomiting Hiccoughing
Zellwegers syndrome Glutaric acidaemia type II PDH deficiency Smith-Lemli-Opitz syndrome NKHGPDH deficiency NKHG Hyperammonaemia Galactosemia Tyrosinaemia a 1-antitrypsin deficiency Fatty acid oxidation defects Glycogen storage disorders Galactosaemia Organic acidaemias Hyperammonaemia NKHG
Specific indicators Clinical context Unexplained and mysterious deterioration of baby (can be as short as 12 hr but more commonly at 48 hr) Family history Known metabolic disorders Unexplained neonatal or infant deaths Parental consanguinity Obstetric history Acute fatty liver of pregnancy and HELLP syndrome in index pregnancy may point towards long chain fatty acid oxidation defect in neonate Non-specific indicators suggestive of metabolic disorder in an encephalopathic baby Encephalopathy in low-risk infant, or onset after period of normality Fluctuating consciousness and muscle tone Changes in muscle tone: axial hypotonia with limb hypertonia normal tone in comatose baby Abnormal movements: myoclonic or boxing movements tongue thrusting lip smacking True seizures occur late in metabolic encephalopathies except in NKHG
Metabolic 2011-13
INITIAL INVESTIGATIONS
Whenever IEM suspected, perform required investigations without delay Seek early advice about appropriate investigations and management from inherited metabolic diseases (IMD) team at tertiary metabolic centre
Urine Smell Ketostix: presence of large amounts of urinary ketones is always abnormal in neonates and suggests IEM, especially organic acidaemias Reducing substances: use Clinitest: urinary dipsticks are specific for glucose and miss galactose in babies with galactosaemia Freeze 1520 mL urine for amino and organic acid analysis Blood FBC, U&E, infection screen Glucose Blood gas Ammonia Lactate Total and conjugated bilirubin, liver function tests including clotting studies Acylcarnitines, including free and total carnitine Uric acid Amino acids Imaging Cranial ultrasound Ophthalmic examination
SPECIFIC INVESTIGATIONS
Jaundice Blood Galactosaemia screen (urinary reducing substances can be negative after short period of galactose exclusion) Ferritin Very long chain fatty acids a 1-antitrypsin (quantitative) 7-dehydrocholesterol Transferrin isoelectric focusing Urine Succinylacetone Skin (and liver) biopsy after discussion with metabolic team Encephalopathy Paired blood and CSF glycine CSF lactate Very long chain fatty acid profile Urine for orotic acid Urine: Sulfitest for sulphite oxidase deficiency Hypoglycaemia (most informative when obtained at time of hypoglycaemia) Plasma non-esterified fatty acids
Metabolic 2011-13
-hydroxybutyrate RBC galactosaemia screen Insulin and C-peptide Acylcarnitine profile, free and total carnitine Cortisol, growth hormone Urine for organic acids
Post-mortem (plan how best to use these precious samples in consultation with IMD team) Plasma (25 mL), urine (1020 mL) and CSF (1 mL) frozen at 20 C Red cells: blood (5 mL) in lithium heparin stored at 4 C (fridge) Blood (5 mL) in EDTA: stored at 4 C for DNA analysis Tissue biopsies skin: store in viral culture medium or saline at 4 C (fridge) muscle and liver: take within 1 hour of death, snap freeze in liquid nitrogen Post-mortem examination
IMMEDIATE MANAGEMENT
Commence emergency management of suspected IEM while awaiting results of initial investigations Attend to Airway, Breathing and Circulation; ventilate if necessary Omit all protein intake, including TPN and lipid Commence intravenous glucose infusion to provide 68 mg glucose/kg/min start insulin infusion if hyperglycaemic (>15 mmol/L) or catabolic if hypertonic glucose infusion necessary, insert central line Correct dehydration, acid-base and electrolyte disturbances Cover for infection Control seizures (avoid sodium valproate) Consider transfer to tertiary metabolic centre if stable and appropriate
SPECIFIC MANAGEMENT
Must be led by IMD team Use following as guide to general principles of management
Neonatal hyperammonaemia A medical emergency requiring prompt intervention to lower ammonia concentration Renal replacement therapy (haemofiltration more efficient than peritoneal dialysis) Sodium benzoate Sodium phenylbutyrate L-arginine L-carnitine Organic acidaemia Reduce/stop protein intake Hypertonic glucose infusion +/- insulin L-carnitine Glycine Biotin Fatty acid oxidation disorders Avoid prolonged fast
Metabolic 2011-13
L-carnitine
Lactic acidosis Dichloroacetate Biotin L-carnitine thiamine Galactosaemia Dietary exclusion of galactose For further Information on inherited metabolic diseases, www.bimdg.org.uk>Guidelines> then emergency protocols and follow through
LOCAL CONTACT
For specialist advice, consult Birmingham Childrens Hospital metabolic team early
Nutrition 201315
NUTRITION
AIMS
To achieve growth and nutrient accretion similar to intrauterine rates To achieve best possible neurodevelopmental outcome To prevent specific nutritional deficiencies
NUTRITIONAL REQUIREMENTS
Daily recommended intake of nutrients for stable/growing preterm babies Preterm baby Preterm baby Nutrient Term baby <1000 g 10001800 g (Tsang/ESPGHAN) (Tsang/ESPGHAN) Energy (kcal/kg) 95115 130150 110135 Protein (g/kg) 2 3.84.5 3.44.2 Sodium (mmol/kg) 1.5 35 35 Potassium (mmol/kg) 3.4 23 23.5 Calcium (mmol/kg) 3.8 2.55.5 2.55.5 Phosphate (mmol/kg) 2.1 24.5 1.94.5
FEEDING GUIDE
Nutrient composition of commonly used milks per 100 mL Fortified breast Preterm Mature breast milk (Nutriprem breast milk milk (>2 wk) BMF) 70 69 85 Energy (kcal) 1.8 1.3 2.6 Protein (g) 1.3 0.7 2.2 Sodium (mmol) 0.55 0.55 2.2 Calcium (mmol) 0.5 0.5 1.9 Phosphorus (mmol) (based on 2013 datacards) SMA Gold Prem 1 82 2.2 1.9 2.5 2.0 Nutriprem 1 80 2.6 3.0 2.4 2.0
Route of administration Most babies <34 weeks cannot co-ordinate sucking, swallowing and breathing effectively and must be tube fed use gastric feeding with either nasogastric or orogastric tube Feeding schedules Gastrointestinal tolerance of feeding is very variable and concomitant illness can impose additional constraints, manage feeding on an individual basis See algorithm: Initiating and advancing enteral feeds
Nutrition 201315
Initiating and advancing enteral feeds Make every effort to use mothers expressed colostrum and breast milk Commence feeding as soon after birth as possible following individual clinical assessment High risk of NEC* Gestation 29+133+6 Gestation >34 weeks
1020 mL/kg/day trophic feeds Maintain trophic feeds as long as clinically indicated
Step 3
Continue to increase by 10 mL/kg twice in 24 hr as hourly feeds until 150180 mL/kg Only increase beyond 180 mL/kg after growth assessment
Babies can move between risk categories following individual clinical assessment *High risk definition: <29 weeks gestation or <1200 g birth weight Absent or reversed end diastolic flow in <34 weeks or <1501 g birth weight Re-establishment of feeds following necrotising enterocolitis (NEC ) Post-surgery for congenital gut abnormality or abdominal wall defects Caution initiating feeds in the following Ibuprofen or surgical ligation for patent ductus arteriosus (during treatment) Complex congenital cardiac disease Dexamethasone treatment Unstable/hypotensive ventilated babies Perinatal hypoxia-ischaemia with significant organ dysfunction Requiring full or partial exchange transfusion Type of milk Breast milk Wherever possible, use expressed breast milk for initiation of enteral feeds. Breast milk remains the ideal milk for term and preterm babies and should be strongly recommended Feeds to an initial volume of 150 mL/kg increasing to 180200 mL/kg as indicated by weight gain and volume tolerance
Nutrition 201315
Donor milk In the absence of a mothers own expressed breast milk, consider donor milk as the next milk of choice for babies at higher risk of NEC Indication for use: mothers milk unavailable gestational age <29 weeks birth weight <1200 g previous proven NEC Due to poor nutritional profile of donor milk it is wise to restrict use to establishing feeds in high risk babies with the gradual introduction of alternative feeds one week after full volumes achieved Slow change to different type of milk feed Occasionally, it may be necessary to change from one type of milk feed to another. Most from donor/mothers expressed breast milk to preterm formula. Ensure this is done slowly to ensure baby tolerates the change in feed Day 1: 75% feeds with current milk, 25% with new milk (i.e. 3 old feeds : 1 new feed) Day 2: 50% feeds with current milk, 50% with new milk (i.e. 2 old feeds : 2 new feeds) Day 3: 75% feeds with new milk, 25% with current milk (i.e. 1 old feed : 3 new feeds) Day 4: 100% new milk NB: It is also acceptable practice during the slow change to mix the milks together rather than using separately (see above) Breast milk fortifier (Nutriprem BMF/SMA BMF) When milk tolerated at 150 mL/kg for 48 hr and >10 days old, add fortifier (human breast milk fortifier) Observe for signs of feed intolerance (abdominal distension, vomiting, increased aspirates, change in stool frequency) for next 24 hr Thereafter, increase volume of milk gradually to 180200 mL/kg/day as necessary Preterm milk formula (Nutriprem 1/SMA Gold Prem 1) Indicated for babies born <2 kg and <34 weeks gestation Initially increase feeds to 150 mL/kg/day If necessary, increase to 180 mL/kg/day as indicated by weight gain Nutrient additives Exclusively breastfed babies <35 weeks gestation once full enteral feeds established 0.6 mL Abidec (NB: contains peanut oil) Joulies phosphate 0.675 mmol 12-hrly adjusted according to serum phosphate and alkaline phosphatase levels, and urinary reabsorption of phosphate folic acid 50 microgram daily until discharge unless prescribed breast milk fortifier Formula feed babies <35 weeks gestation, after first week once full enteral feeds established 0.3 mL Abidec (NB contains peanut oil) unless baby receiving Nutriprem 2 or SMA 2 Iron At 28 days of age and only for exclusively breastfed babies <2.3 kg at birth, start sodium feredetate (e.g. Sytron) once daily: <1.5 kg, 0.5 mL 1.5 kg, 1 mL Babies fed preterm formula or preterm follow on milk do not need iron supplements
Nutrition 201315
If feeding on preterm milk and gaining 1520 g/kg/day, change to a nutrient enriched post- discharge formula (e.g. Nutriprem 2 or SMA Gold Prem 2) If feeding on a preterm milk and gaining <15 g/kg/day, refer to dietitian
Specialised term formulas Use specialised formulas only where absolutely necessary and always under the direction of a paediatric or neonatal dietitian
MONITORING
Monitoring of gastrointestinal tolerance, growth and biochemical balance is critical in nutritional management of preterm babies Clinical monitoring Daily assessment of gastrointestinal tolerance: gastric residues stool frequency abdominal examination as appropriate Anthropometry Monitor weight daily for first few days to assist with fluid management see Intravenous fluid therapy guideline once clinically stable, measure weight twice weekly weight gain of 1520 g/kg/day is adequate in growing phase 2530 g/day is adequate weight gain if weight >2.0 kg Measure head circumference weekly to assess cerebral growth Document weight and head circumference regularly on growth chart. Measure length weekly Biochemical monitoring In sick or very premature babies, measure plasma urea, electrolytes, calcium, phosphate and albumin twice daily for initial few days. Reduce frequency depending on clinical stability Monitor glucose closely in initial few days once clinical stability and full enteral feeds achieved, carry out these tests at least once a week in very low birth weight (VLBW) babies Check urine weekly for excretion of sodium and phosphate
COMMON PROBLEMS
Poor growth Babies with weight gain <15 g/kg/day require further assessment Ensure baby receiving adequate nutrition (energy intake >120 kcal/kg/day; protein 3.3 g/100 kcal). Calculate energy and protein intake per kg/day Check for following factors, that may affect growth: clinical illness (e.g. UTI) medications (diuretics) steroid treatment can delay growth in length for up to 34 weeks after stopping increased energy requirement resulting from respiratory/cardiac disorders hyponatraemia (serum Na should be 132 mmol/L) hypophosphatemia (maintain serum PO4 at 2 mmol/L) anaemia Excessive weight gain Babies with weight gain >25 g/kg/day require further assessment Ensure measurement not spurious and not related to catch-up growth after a period of poor weight gain Evaluate for fluid retention and its causes consider diuretics in presence of oedema If receiving >150 kcal/kg/day, reduce energy intake
Nutrition 201315
if applicable, change feed under direction of paediatric dietitian, or decrease volume of feeds
Feeding intolerance Intolerance to feeding is common among small preterm babies and most will have episodes requiring either temporary discontinuation of feeding or delay in advancing feeds Carefully observe for signs of necrotising enterocolitis (NEC) including abdominal distension, discolouration, blood in stools, metabolic acidosis see Necrotising enterocolitis guideline Gastric aspirates If aspirates non-bilious and less than half the volume of previous feed, they can be replaced and feeding continue while observing baby closely If aspirates bilious or more than half previous feed volume, consider withholding feeds on that occasion and assess for any signs of NEC Patent ductus arteriosus (PDA) Preterm babies with PDA have decreased blood-flow in descending aorta and increased risk of NEC. ibuprofen is also associated with decreased gastrointestinal blood-flow. Observe closely for feeding intolerance and signs of NEC As increased IV fluid rates are associated with PDA, avoid any increase >150 mL/kg/day Cautiously increase feeds while receiving ibuprofen
POST-DISCHARGE NUTRITION
Nutrients vitamins and iron Breast milk Babies <35 weeks gestation, give multivitamins (Abidec) 0.6 mL) until 12 months old Babies <2.3 kg at birth, give sodium feredetate (e.g. Sytron) once daily: <1.5 kg, 0.5 mL 1.5 kg, 1 mL discontinue once mixed feeding established Term formulas Babies <35 weeks gestation, give multivitamin (abidec) 0.3 mL until 12 months old Give sodium feredetate (e.g. Sytron) once daily: <1.5 kg, 0.5 mL 1.5 kg, 1 mL discontinue once mixed feeding established Follow-on preterm formula Consider post-discharge follow-on preterm milk (e.g. Nutriprem 2, SMA 2) in premature babies with: chronic lung disease restricted intake (e.g. congenital heart disease) poor growth For term babies with increased energy demands or reduced intake, liaise with dietitian regarding use of a higher energy formula
PARENTERAL NUTRITION
DEFINITION
Parenteral nutrition (PN) or total parenteral nutrition (TPN) is the intravenous infusion of some or all nutrients for tissue maintenance, metabolic requirements and growth promotion in babies unable to tolerate full enteral feeds Seek advice from your local PN pharmacist
INDICATIONS FOR PN
Short-term supply of nutrients Extremely low birth weight (<1000 g) and/or gestation <29+0 (national CQUIN) Very low birth weight (<1500 g) AND clinically unstable, absent/reversed end-diastolic flow or full enteral feeds seem unachievable by day 5 Any baby on inotropic support Necrotising enterocolitis (1014 days) Temporary feeding intolerance Prolonged non-use of gastrointestinal (GI) tract >2 weeks Usually commenced in surgical centre before transfer back to Neonatal Unit: relapsing or complicated necrotising enterocolitis (NEC) surgical GI disorders (e.g. gastroschisis, large omphalocoele) short bowel syndrome
by 2 each day
Birth weight 2.55 kg 2.55 kg Day 1 Amino acid 1 (g/kg/day) Carbohydrate 614 (g/kg/day) (based on maintenance) fluid volume Fat (g/kg/day) 1
Day 3 2.5 14
Day 4 2.5 14
3.5
Electrolyte and other nutrient requirements Birth weight <2.5 kg Na (mmol/kg/day) K (mmol/kg/day) Ca (mmol/kg/day) PO4 (mmol/kg/day) Mg (mmol/kg/day) Peditrace (mL/kg/day) Solvito N (mL/kg/day) Vitilipid (infant) 3 (range 24)** 2.5 1 1.5 0.2 0.5 (day 1) 1 (day 2 onwards) 1 4 mL/kg/day Birth weight 2.55 kg 3 (range 24) 2.5 1 1 0.2 0.5 (day 1) 1 (day 2 onwards) 1 10 mL daily (total)
** Do not add supplemental sodium on days 12 if <32 weeks before naturesis has occurred (measure urine Na levels daily) Glucose maximum concentration Peripheral PN 1012% Central PN up to 2025% (may rise occasionally) Volume Maintenance up to 150 mL/kg/day (see Intravenous fluid therapy guideline for fluid requirement) maximal fluid volume varies with individual management Remember to account for volume, electrolyte and glucose content of other infusions (e.g. UAC/UVC fluid, inotropes, drugs) Calories Healthy preterm requires 50 kcal/kg/day for basal energy expenditure (not growth) and 11.5 g protein to preserve endogenous protein stores; more is required for growth, particularly if unwell 60 kcal/kg/day will meet energy requirements during sepsis 90 kcal/kg/day and 2.73.5 g protein will support growth and positive nitrogen balance 120 kcal/kg/day may be required for a rapidly growing preterm baby
NUTRITIONAL SOURCES
Glucose (provides 3.4 kcal/g) Initiated at endogenous hepatic glucose production and utilisation rate of 46 mg/kg/min; [810 mg/kg/min in extremely low-birth-weight (ELBW) babies]. Osmolality of glucose limits its concentration Protein (provides 4 kcal/g) At least 1 g/kg/day in preterm and 2 g/kg/day in ELBW decrease catabolism 33.5 grams protein/kg/day and adequate non-protein energy meets requirements for anabolism
Fat (provides 9 kcal/mL) Fat of 33.5 g/kg/day is usually sufficient 4 g/kg/day only in very preterm with normal triglycerides not septic, not on phototherapy fat should ideally provide 3540% of non-protein nitrogen calories To minimise essential fatty acid deficiency, hyperlipidaemia, bilirubin displacement, and respiratory compromise, lipid infusion rates 0.15 g/kg/hr are recommended to run throughout 24 hr in babies, maximal removal capacity of plasma lipids is 0.3 g/kg/hr delivery of 3 g/kg/day of a 20% lipid emulsion equates to an infusion rate of 0.125 g/kg/hr Energy Carbohydrate (glucose) and fat (lipid emulsions) provide necessary energy to meet the demands and, when provided in adequate amounts, spare protein (amino acids) to support cell maturation, remodelling, growth, activity of enzymes and transport proteins for all body organs PN requirement for growth 90115 kcal/kg/day Electrolytes Sodium, potassium, and chloride dependent on obligatory losses, abnormal losses and amounts necessary for growth, and can be adjusted daily If baby <32 weeks, do not add sodium until they have started their naturesis, monitored by daily urine Na+ Babies given electrolytes solely as chloride salts can develop hyperchloraemic metabolic acidosis (consider adding acetate to PN, where available) Monitor serum phosphate twice weekly. Aim to maintain at around 2 mmol/L Vitamins Vitamin and mineral added according to best estimates based on limited data
SPECIAL NEEDS
Hyperglycaemia If hyperglycaemia severe or persistent, start insulin infusion see Administration of actrapid insulin (soluble insulin) in Hyperglycaemia guideline Osteopenia If baby at risk of, or has established osteopenia, give higher than usual intakes of calcium and phosphorus consult dietitian and/or PN pharmacist regarding prescribing information permissible concentrations depend on amino acid and glucose concentrations in PN solution Metabolic acidosis For management of metabolic acidosis, add acetate as Na or K salt if available: ask local PN pharmacist choice of salt(s) will depend on serum electrolytes Increase calories for: infection chronic lung disease healing growth babies who have experienced intrauterine growth retardation (IUGR) Decrease calories for: sedation mechanical ventilation
MONITORING
Daily Fluid input Fluid output Energy intake Protein Non-protein nitrogen Calories Urine glucose Blood glucose (if urine glucose positive) Urine electrolytes Weight Length Head circumference FBC Na K Glucose Urea Creatinine Albumin Bone chemistry Bilirubin ** Blood gas (arterial or venous) Serum TG** Magnesium Zinc**
Weekly
* Initially daily and decrease frequency once stable unless indicated for other birth weight or gestationspecific guidance See Intravenous fluid therapy guideline ** In prolonged PN >2 weeks
COMPLICATIONS
Catheter-related: (see Long line insertion guideline) Peripheral catheters: extravasations and skin sloughs Septicaemia Electrolyte abnormalities Electrolyte and acid-base disturbances Metabolic Hyper/hypoglycaemia, osmotic diuresis Metabolic bone disease: mineral abnormalities (Ca/PO4/Mg) Hyperlipidaemia and hypercholesterolaemia Conjugated hyperbilirubinaemia PN-associated cholestatic hepatitis Can occur with prolonged PN (>1014 days) probably due to combination of PN hepato-toxicity and reduced oral feeding often transient usually manifests as rising serum bilirubin (with increased conjugated component) and mildly elevated transaminases leads to deficiencies of fatty acids and trace minerals even small enteral feeds will limit or prevent this problem add trace minerals to PN consider other causes of hyperbilirubinaemia (PN-induced cholestasis is diagnosis of exclusion) e.g. CMV, hypothyroidism if stools acholic (putty grey) or very pale, refer urgently to liver unit
WEANING PN
When advancing enteral feedings, reduce rate of PN administration to achieve desired total fluid volume Decrease the aqueous and fat portions by 90% and 10% respectively for each volume of PN reduced e.g. if reducing PN by 1 mL/hr, reduce Vamin by 0.9 mL and Intralipid by 0.1 mL Assess nutrient intake from both PN and enteral feed in relation to overall nutrition goals
GENERAL
Advise mothers to: bath or shower daily wash hands thoroughly with soap and running water before expressing gently massage breast and stimulate nipple to trigger milk ejection reflex before milk expression
MILK COLLECTION
Sterilise milk collection utensils before use Commence milk collection as soon as possible following delivery (preferably within 6 hr) Frequency of expression should be 812 times/24 hr (not leaving a gap of longer than 6 hr overnight) Teach all mothers hand expression Use hand expression to express colostrum and collect the milk obtained via a syringe When milk obtained is sufficient enough to remove easily from a storage container, teach mothers to use an electric breast pump Encourage simultaneous (double) pumping of both breasts Ensure mother has a properly fitting breast shield (funnel), size is determined by comfort
TECHNIQUE
Ensure mother seated in comfortable straight backed chair and keep clothing away from breast while milk is being expressed Support breast from underneath with fingers flat on ribs and index finger at junction of breast and ribs with nipple positioned centrally in shield (funnel) Adjust suction control for comfort Use light pressure to obtain patent seal between breast and shield. Firm pressure will inhibit milk flow by compressing ducts Empty breasts as thoroughly as possible since fat content increases as breast is drained If using a single pump, mothers should switch to second breast when milk flow slows Use a new bottle for each expression Leave a space of 12 cm at the top of each bottle to allow for expansion during freezing Following expression, wash equipment in hot soapy water with a bottle brush before sterilization Encourage mothers to practice kangaroo care also known as skin-to-skin holding See Kangaroo care guideline
Problems related to milk expression Sore nipples: centre milk expression shield try a variety of shield sizes check pump vacuum stop pump before removing shields do not use plastic-backed breast pads change breast pads frequently
Too little milk Increase kangaroo care (skin-to-skin) Express close to babys cot Check frequency and duration of pumping Check shield (funnel) size Encourage breast compression during expression Increase frequency of expression sessions Consider enhancing of prolactin secretion using domperidone Praise provision of expressed milk, no matter how small
ADMINISTRATION
Ensure there is a dedicated fridge and freezer for storage of milk on ward Add date and time bottles removed from freezer/opened to bottle label
ADVICE TO MOTHERS
Advise mothers to bath or shower daily do not wash breasts with bactericidal detergent or soap Before expressing milk, it is essential to wash hands thoroughly with soap and water and dry with a disposable towel Give all breastfeeding mothers the BLISS Preterm breastfeeding leaflet Emphasise to mothers the importance of washing all breast milk collecting equipment properly before disinfection wash equipment with detergent and hot water using bottle brush (not shared) and rinse well before disinfection discard bottle brushes on discharge
STORAGE
Where Store in refrigerator at 4oC. Freshly expressed breast milk can be stored for 48 hr before freezing Breast milk can be stored for 3 months in freezer without a defrost cycle if freezer has defrost cycle and milk appears frothy but does not smell rancid, it is safe to use Monitor fridge and freezer temperature using maximum/minimum thermometer that has been calibrated every 6 months How Place milk in sterile container with airtight lid Ensure bottles labelled appropriately Store labelled bottles in separate containers in fridge/freezer (individual containers must not hold bottles from more than one mother) Wash containers in fridge daily in warm soapy water, rinse well and dry thoroughly Clean container between each use Shake milk containers vigorously before use refrigerated milk separates with hind milk forming top layer
DEFROSTING
Use frozen milk in sequence of storage Defrost frozen milk in fridge If frozen milk needs to be thawed quickly, hold bottle under cold or tepid water shake frequently and do not allow water to enter bottle via cap Discard thawed milk (stored in a refrigerator) after 12 hr
USE
Once removed from fridge, fresh or defrosted milk must be used within 4 hr Fresh milk is preferable to thawed milk Change continuous tube feeding (tubing between nasogastric tube and pump) every 4 hr to minimise fat loss, position syringe delivering feed in semi-upright position
TRANSPORTATION OF MILK
Milk is often transported from: Mother's home to hospital transport in an insulated container that can be easily cleaned encourage mothers to use coolant block to maintain stable temperature Hospital to hospital use rigid container for easy cleaning (e.g. cool box) and fill empty space with bubble wrap
PRECAUTIONS
Wash hands thoroughly Cover cuts and abrasions and wear gloves if necessary
RECORD KEEPING
Label all bottles with babys printed hospital label containing: name and hospital number date and time of expression If mother expressing milk at home, provide supply of printed hospital labels Before giving breast milk, two members of staff must check label and cross-reference with babys identity bracelet to ensure milk is not given to wrong child See Breastfeeding guideline
Breastfeeding 2011-13
BREASTFEEDING
PRE-TERM INFANTS RATIONALE
Human milk is important in establishing enteral nutrition Any amount of mothers fresh breast milk is better than none Physician advocacy has a strong influence on intention to feed
IMPLEMENTATION
In pregnancy at high-risk of premature delivery, discuss feeding during antenatal period During mothers first visit to NICU, discuss value/benefits Document discussion in medical record Separate decision to provide a few weeks of pumped breast milk from the commitment to long-term, exclusive breastfeeding Praise efforts to provide expressed breast milk Ensure adequate discussion and provision of written information on hand-expression, and on mode and frequency of pump use
CONTRAINDICATIONS TO BREASTFEEDING
Infants with galactosaemia should not receive breast milk HIV in UK Always check maternal HIV status before breastfeeding Breastfeeding absolutely contraindicated (in UK) If you are concerned that mother intends to breastfeed, ensure an HIV specialist explains the risk to which infant will be exposed HIV in developing countries If returning to a developing country where there is no access to clean water, exclusive breastfeeding is safer than mixed Maternal medications Antimetabolites or cytotoxic drugs Radioisotope investigation (until isotope clears) See Medications and mothers milk by T W Haleor or Neonatal Formulary or BNF 61 page 19 A current, reliable reference for drugs and breastfeeding must be available on the neonatal unit
Breastfeeding 2011-13
Hepatitis B Risk of transmission can be almost totally eliminated by a combination of active and passive immunisation Breastfeeding is not contraindicated See Hepatitis B and C guideline Hepatitis C Transmission by breastfeeding theoretically possible but has not been documented Breastfeeding not contraindicated but inform mother that risks are unknown Varicella-zoster virus (VZV) Infants of mothers with active VZV can reduce risk by avoiding breastfeeding until mother is no longer infectious (5 days from onset of rash) Premature babies <1 kg, <28 weeks are considered high risk and should be given VZIG (see Varicella guideline) Herpes simplex type 1 Stop women with herpetic lesions on breast from breastfeeding or feeding expressed breast milk from affected side (until lesions have healed) cover active lesions elsewhere careful hand hygiene essential affected side cover and pump and discard milk (no breastfeeding) until lesions are clear unaffected side can breastfeed and use expressed breast milk Phenylketonuria (PKU) Breastfeeding not contraindicated in infants with PKU Screening service will contact paediatric dietitians directly Careful dietetic management necessary All infants should be under care of paediatric dietitians Radioactive diagnostic agents Women receiving radioactive diagnostic agents need to pump and discard milk for varying periods of time although most agents have very short plasma half-lives, seek advice from hospital nuclear medicine department Medications For medications that require caution with breastfeeding, see references given above check all maternal medication against texts above Social drugs Alcohol discourage more than limited consumption Nicotine nicotine concentration in breast milk increases immediately after smoking discourage mothers from smoking directly before breastfeeding or expressing
ENTERAL FEEDING SMALL-FOR-GESTATIONAL AGE AND VERY LOW BIRTH WEIGHT BABIES
See also Nutrition guideline
INDICATIONS
Small-for-gestational age = birth weight <10th centile Very low birth weight = <1500 g
PRINCIPLES
Early enteral feeds promote normal gastrointestinal structure and function, motility and enzymatic activity The intrauterine growth restricted (IUGR) baby of <34 weeks gestation is at greatest risk of necrotising enterocolitis (NEC) Enteral feeding may be a risk factor for necrotising enterocolitis, especially in premature babies, those with intrauterine growth restriction and absent or reversed end-diastolic flow on umbilical artery Doppler Delayed nutrition can result in growth restriction with long-term complications of short stature, poor organ growth and poorer neurological function Delayed feeding in sick baby, regardless of gestation, is appropriate. Decision to start enteral feeding should be made on daily ward round The evidence base for when to start and how fast to increase feeds is limited and metaanalyses can be inconclusive regarding implications for practice
Table 1: Starting and increasing feeds When to start feeds Group 1 Well >34 weeks gestation (small-forgestational age <10th centile) 3034 weeks gestation with no risk factors for gut problems 3034 weeks gestation with birth weight <10th centile and absent or reversed end diastolic flow on fetal Doppler <30 weeks gestation Full feeds on day 1 : follow Hypoglycaemia guideline Enteral feeds on day 1 Start at 1 mL/kg/hr Nil-by-mouth for 48 hr, then trophic feeds at 0.51 mL/kg/hr for 24 hr
2 3
Table 2: Fluid volume Fluid volume (mL/24 hr) Day of life 1 2 3 4 <1000 g 90 120 150 150 1000 g 60 90 120 150
Babies who are medically fit to take all their fluids enterally should do so. Do not use IV fluids as a substitute for breast milk in such babies
Stop feeds and seek medical review if Aspirates heavily bile stained Blood or mucus per rectum Abnormal abdominal examination (e.g. abdominal distension, discolouration, tenderness, poor bowel sounds, not passing stools >48 hr)
FREQUENCY OF FEEDS
Dependent on maturity and condition of baby In extremely premature baby, initial 12 hrly feeds are appropriate Once baby tolerating full feeds, increase feed interval
ROUTE OF FEEDING
In most premature babies: via nasogastric or orogastric tube Once baby more mature and able to suck, offer feeds by breast (see Progression to breastfeeding below), cup or bottle Encourage mothers who wish to breastfeed by starting skin-to-skin time
PROGRESSION TO BREASTFEEDING
Aim Safe progression to exclusive breastfeeding Principles Reaching an actual gestational age or body weight is not an indication for transition from nasogastric tube (NGT) feeding to breastfeeding Initiation of oral feeds should follow observations of babys behaviour e.g: tolerating bolus feeds swallowing secretions physiologically stable stable respirations (>70 breaths/min will inhibit oral response) able to demonstrate rhythmic non-nutritive sucking for approximately 5 min at least 3234 weeks gestation Early breastfeeding attempts are gradual and not expected to result in full feeding immediately increase breastfeeds gradually from a few minutes at the breast to 1 full feed per day, in response to babys demands
Contraindications and special precautions See Breastfeeding guideline Maternal milk supply Ensure sufficient maternal breast milk and good lactation should fulfil babys total 24 hr requirement by 72 hr postnatal Process Skin-to-skin contact for extended periods as long as mother and baby can tolerate Non-nutritive sucking with a dummy Suckling at emptied breast Positioning at breast Attachment May need temporary use of premature nipple shields If baby awake, alert and able to demonstrate suckling, commence nutritive sucking at breast <5 min followed by supplementation with total volume of NGT/enteral feed 510 min followed by supplementation with three-quarters volume of NGT/enteral feed 1015 min followed by supplementation with half volume NGT/enteral feed 1520 min at the breast with no supplementation with NGT/enteral feeds Optimise milk transfer Offer the breast with the best flow first. Stimulate the let-down reflex before putting baby to breast e.g. hand or mechanical expression Progression to demand feeding Gradual progress from NGT enteral feeds to exclusive breastfeeding ensures nutritional needs are met and prevents baby from becoming overtired Increase volume of feeds before withdrawal of NGT Weight gain of 1015 g/kg/day must be achieved before changing to full breastfeeds Monitor weight, length and head circumference regularly to assess nutritional status and adequacy of feeding If poor weight gain, feed volume can be increased to a maximum of 200 mL/kg, if tolerated, or breast milk fortifier may be added
ENTERAL FEEDING FOR SMALL-FOR-GESTATIONAL AGE AND VERY LOW BIRTH WEIGHT BABIES Supporting information
Does delaying the introduction of progressive enteral feeding help prevent necrotising enterocolitis (NEC) in VLBW infants? A Cochrane Review of 5 RCTs in a total of 600 infants (Morgan, 2011) concluded that: Current trial data do not provide evidence that delayed introduction of progressive enteral feeds reduces the risk of NEC in VLBW infants. Delaying the introducing of progressive enteral feeds results in several days delay in establishing full enteral feeds but the clinical importance of this effect is unclear. Further RCTs are needed to give more precise estimates of the effect of delaying the introduction of enteral feeds on clinical outcomes in VLBW infants. Another review by the same authors reached similar conclusions about the slow advancement of enteral feed volumes and called for further large-scale trials. Morgan J, Young L, McGuire W. Delayed introduction of progressive enteral feeds to prevent necrotising enterocolitis in very low birth weight infants. Cochrane Database of Systematic Reviews 2011, Issue 3. Art. No.: CD001970
Morgan J, Young L, McGuire W. Slow advancement of enteral feed volumes to prevent necrotising enterocolitis in very low birth weight infants. Cochrane Database of Systematic Reviews 2011, Issue 3. Art. No.: CD001241
Does positioning have an effect on GOR? A systematic review of randomised controlled trials (Carroll, 2002) quotes a controlled prospective study of 9 infants with GOR (Orenstein, 1983) which found that positioning at a 60 degree elevation in an infant seat increased reflux compared with the prone position. A later study by the same author (Orenstein, 1990) found no significant difference between the flat and head-elevated prone positions. The supine reversed-Trendelenburg sleeping position, recommended by some (Taminiau, 1997), was found to increase acid reflux parameters in all 10 consecutively investigated infants in a Belgian study (Bagucka, 1999). A study of 18 preterm infants with GOR (Ewer, 1999) compared prone, left lateral and right lateral positions. Each position was used for 8 hours, with the order randomly assigned. The reflux index was significantly less in prone (6.3) and left lateral (11.0) positions compared to the right lateral (29.4). The left lateral position may be an acceptable alternative, in infants, to the prone position which has been associated with sudden infant death (Tobin, 1997; Vandenplas, 1997) and is recommended in US guidelines (Anon, 2001) for children older than one year. The effects of positioning have not been studied in children older than 1 year (Anon, 2001). A study in 22 premature infants (Corvaglia, 2007) found that oesophageal exposure to acid and nonacid GOR was lower in the prone (4.4% and 0.3%, respectively) and the left lateral (7.5% and 0.7%, respectively) positions than in the right lateral (21.4% and 1.2% respectively) and supine (17.6% and 1.3%, respectively) positions.
Anon. Guidelines for evaluation and treatment of gastroesophageal reflux in infants and children: recommendations of the North American Society for Pediatric Gastroenterology and Nutrition. J Pediatr Gastroenterol Nutr 2001;32(Suppl 2):S1-S31 Bagucka B, De Schepper J, Peelman M, et al. Acid gastro-esophageal reflux in the 10 degreesreversed-Trendelenburg-position in supine sleeping infants. Acta Paediatr Taiwan 1999;40:298-301 Carroll AE, Garrison MM, Christakis DA. A systematic review of nonpharmacological and nonsurgical therapies for gastroesophageal reflux in infants. Arch Pediatr Adolesc Med 2002;156:109-13 Corvaglia L, Rotatori R, Ferlini M, et al. The effect of body positioning on gastroesophageal reflux in premature infants: evaluation by combined impedance and pH monitoring. J Pediatr 2007;151:591-6 Ewer AK, James ME, Tobin JM. Prone and left lateral positioning reduce gastro-oesophageal reflux in preterm infants. Arch Dis Child Fetal Neonatal Ed 1999;81:F201-5 Orenstein SR, Whitington PF, Orenstein DM. The infant seat as treatment for gastroesophageal reflux. N Engl J Med 1983;309:760-3 Orenstein SR. Prone positioning in infant gastroesophageal reflux: is elevation of the head worth the trouble? J Pediatr 1990;117:184-7 Taminiau JA. Gastro-oesophageal reflux in children. Scand J Gastroenterol 1997;2(Suppl 223):18-20 Tobin JM, McCloud P, Cameron DJ. Posture and gastro-oesophageal reflux: a case for left lateral positioning. Arch Dis Child 1997;76:254-8 Vandenplas Y, Belli DC, Dupont C, et al. The relation between gastro-oesophageal reflux, sleepingposition and sudden infant death and its impact on positional therapy. Europ J Pediatr 1997;156:104-6
Evidence Level: I (Against infant seats and head-elevation); II (for the use of the left lateral position) Are thickened feeds of use for GOR? Two systematic reviews of randomised controlled trials (Huang, 2004; Carroll, 2002;) found no statistically significant reduction in reflux with the use of thickened infant foods compared to placebo. One study in 24 infants (Borrelli, 1997) did, however, detect a significant benefit of formula thickened with carob bean gum compared with rice flour (pH<4 for 5% vs 8% of time). This was replicated by a later study in 14 infants (Wenzl, 2003). Another study in 19 infants (Sutphen, 1989) showed that supplementing with dextrose 5% water was associated with less reflux than dextrose 10% water. US guidelines (Anon, 2001) note that, although thickened feeds do not improve reflux index scores, they do decrease the number of episodes of vomiting. A study in 30 formula-fed babies (Vandenplas, 1987) found little change in the reflux index (17.8% vs 18.4%) after 7-14 days of thickened feeds, but in 24 infants a decrease in the number of reflux episodes in 24 hours (15.1 vs 34.5). The duration of the longest recorded episode did, however, increase from 23.3 min to 56.6 min, leading the authors to warn against possible protracted episodes of occult GOR. Two similar studies, in 20 (Orenstein, 1987) and 52 infants respectively (Bailey, 1987) also came to the same conclusion. A double-blind, randomised trial in 104 infants (Vanderhoof, 2003) found that a pre-thickened formula (Enfamil AR) was associated with greater symptom reduction by one week compared to placebo: % feedings with any regurgitation (p=0.045), total regurgitation volume score (p=0.035), and % feedings with choke/gag/cough (p=0.004). Sleep was improved in those infants with most symptoms at baseline (p=0.030). The ESPGHAN Committee on Nutrition recommends that thickened feeds should be used only in selected infants with failure to thrive caused by excessive nutrient losses associated with GOR, and also points out that antireflux milk products usually do not meet the European Unions compositional standards for infant formula (Aggett, 2002). A randomised controlled trial in 96 formula-fed infants (Xinias, 2005) compared a group given standard formula (n=45) with a group given formula thickened with cornstarch/casein (n=51) for 28 days. In the cornstarch group, the percentage of time with a pH < 4.0, number of reflux episodes > 5 min and duration of the longest reflux episode all decreased significantly whilst remaining unchanged in the standard formula group. A systematic review and meta-analysis of 14 RCTs in 877 infants (Horvath, 2008) found that thickened feeds significantly increased the percentage of infants with no regurgitation (RR: 2.9; 95% CI: 1.7 to 4.9). No one thickening agent demonstrated superiority over another.
Aggett PJ, Agostoni C, Goulet O, et al. Antireflux or antiregurgitation milk products for infants and young children: a commentary by the ESPGHAN Committee on Nutrition. J Pediatr Gastroenterol Nutr 2002;34:496-8 Anon. Guidelines for evaluation and treatment of gastroesophageal reflux in infants and children: recommendations of the North American Society for Pediatric Gastroenterology and Nutrition. J Pediatr Gastroenterol Nutr 2001;32(Suppl 2):S1-S31 Bailey DJ, Andres JM, Danek GD, et al. Lack of efficacy of thickened feeding as treatment for gastroesophageal reflux. J Pediatr 1987;110:187-9 Borrelli O, Salvia G, Campanozzi A, et al. Use of a new thickened formula for treatment of symptomatic gastroesophageal reflux in infants. Dig Liver Dis 1997;29:237-42 Carroll AE, Garrison MM, Christakis DA. A systematic review of nonpharmacological and nonsurgical therapies for gastroesophageal reflux in infants. Arch Pediatr Adolesc Med 2002;156:109 -13 Horvath A, Dziechciarz P, Szajewska H. The effect of thickened-feed interventions on gastroesophageal reflux in infants: systematic review and meta-analysis of randomized, controlled trials. Pediatrics 2008;122:e1268-77 Huang RC, Forbes DA, Davies MW. Feed thickener for newborn infants with gastro-oesophageal reflux (Cochrane Review). In: The Cochrane Library, Issue 1, 2004. Chichester, UK: John Wiley & Sons, Ltd.
Orenstein SR, Magill HL, Brooks P. Thickening of infant feedings for therapy of gastroesophageal reflux. J Pediatr 1987;110:181-6 Sutphen JL, Dillard VL. Dietary caloric density and osmolality influence gastroesophageal reflux in infants. Gastroenterology 1989;97:601-4 Vandenplas Y, Sacre L. Milk-thickening agents as a treatment for gastroesophageal reflux. Clin Pediatr 1987;26:66-8 Vanderhoof JA, Moran JR, Harris CL, et al. Efficacy of a pre-thickened infant formula: a multicenter, double-blind, randomized, placebo-controlled parallel group trial in 104 infants with symptomatic gastroesophageal reflux. Clin Pediatr 2003;42:483-95 Wenzl TG, Schneider S, Scheele F, et al. Effects of thickened feeding on gastroesophageal reflux in infants: a placebo-controlled crossover study using intraluminal impedance. Pediatrics 2003;111:e355-9 Xinias I, Mouane N, Le Luyer B, et al. Cornstarch thickened formula reduces oesophageal acid exposure time in infants. Dig Liver Dis 2005;37:23-7
Evidence Level: I (for reduced vomiting) Is Gaviscon of use in GOR? A randomised study on 20 infants and children (Buts, 1987) compared Gaviscon with placebo (10 patients in each group). After 8 days of treatment with Gaviscon, all pH monitoring variables were significantly reduced between 35% and 61% of initial recorded values. In the placebo group, mean values changed little (-9.5 - +8.2 of initial values). Reported episodes of regurgitation were also reduced in the Gaviscon group compared to no change in the placebo group. A randomised, parallel group study conducted on 50 infants with GOR (Greally, 1992) compared Gaviscon plus Carobel (carob seed flour) with oral cisapride (0.8 mg/kg/day over one month). 14 of 26 in the cisapride group (53%) were considered better by their parents, compared with 19 of 24 (79%) in the Gaviscon/Carobel group. 5 of 17 pH variables improved from baseline in the cisapride group compared to 11 of 17 given Gaviscon plus Carobel. Despite this, unpaired analysis of diary and pH data showed no significant difference between the groups. The authors concluded that cisapride was no more effective than Gaviscon plus Carobel. A third randomised study comparing gaviscon and metoclopramide (Forbes, 1986) found that neither decreased the frequency or duration of GOR. Gaviscon 100mg/20 ml feed is recommended in a review of GOR (Taminiau, 1997). A double-blind, non-randomised study in 20 infants (Del Buono, 2005) compared the effects of 6 random administrations of Gaviscon Infant (625 mg in 225 ml milk) with placebo (mannitol and Solvito N, 625 mg in 225 ml milk). There was no significant difference between Gaviscon and placebo in terms of median number or duration of reflux/acid reflux events per hour, nor in minimum distal/proximal pH or total acid clearance time per hour.
Buts JP, Barudi C, Otte JB. Double-blind controlled study on the efficacy of sodium alginate (Gaviscon) in reducing gastroesophageal reflux assessed by 24 h continuous pH monitoring in infants and children. Europ J Pediatr 1987;146:156-8 Del Buono R, Wenzl TG, Ball G, et al. Effect of Gaviscon Infant on gastro-oesophageal reflux in infants assessed by combined intraluminal impedance/pH. Arch Dis Child 2005;90:460-3 Forbes D, Hodgson M, Hill R. The effects of gaviscon and metoclopramide in gastroesophageal reflux in children. J Pediatr Gastroenterol Nutr 1986;5:556-9 Greally P, Hampton FJ, MacFayden UM, et al. Gaviscon and Carobel compared with cisapride in gastrooesophageal reflux. Arch Dis Child 1992;67:618-21 Taminiau JA. Gastro-oesophageal reflux in children. Scand J Gastroenterol 1997;2(Suppl 223):18-20
Evidence Level: II
Is domperidone of use in GOR? A double-blind, randomised, placebo-controlled study in 80 children with GOR (Carroccio, 1994) found that symptoms resolved completely in 16 of 20 receiving domperidone plus magnesium hydroxide and aluminium hydroxide. This compared with 8 of 20 given domperidone plus alginate, 9 of 20 given domperidone alone, and 7 of 20 given a placebo. Similar results were achieved in an earlier study by the same team (Iacono, 1991) which compared only the first two groups of the later study. Percentage reflux time was significantly reduced only in the first group. A double-blind, placebo-controlled trial of domperidone in 17 children (Bines, 1992) found that treatment was necessary for 8 weeks before significant improvement occurred in measures of reflux other than number of episodes in the 2 hour postprandial period. The efficacy of domperidone in children is currently considered to be unproven (Cezard, 2004; Anon, 2001; Brown 2000). A systematic review of 4 RCTs (Pritchard, 2005) concluded that there was no robust evidence of efficacy for the treatment of GOR with domperidone in young children. Given the usually benign nature of the condition, the widespread use of unlicensed medicines for GOR is not warranted.
Anon. Guidelines for evaluation and treatment of gastroesophageal reflux in infants and children: recommendations of the North American Society for Pediatric Gastroenterology and Nutrition. J Pediatr Gastroenterol Nutr 2001;32(Suppl 2):S1-S31 Bines JE, Quinlan JE, Treves S, et al. Efficacy of domperidone in infants and children with gastroesophageal reflux. J Pediatr Gastroenterol Nutr 1992;14:400-5 Brown P. Medical management of gastroesophageal reflux. Curr Opin Pediatr 2000;12:247-50 Carroccio A, Iacono G, Montalto G, et al. Domperidone plus magnesium hydroxide and aluminium hydroxide: a valid therapy in children with gastroesophageal reflux. A double-blind randomized study versus placebo. Scand J Gastroenterol 1994;29:300-4 Cezard JP. Managing gastro-oesophageal reflux disease in children. Digestion 2004;69(suppl 1):3-8 Iacono G, Carroccio A, Cavataio F, et al. Severe gastroesophageal reflux in children: effectiveness of different combinations of drugs. Curr Ther Res Clin Exp 1991;50:474-81 Pritchard DS, Baber N, Stephenson T. Should domperidone be used for the treatment of gastrooesophageal reflux in children? Systematic review of randomized controlled trials in children aged 1 month to 11 years old. Br J Clin Pharmacol 2005;59:725-9
Evidence Level: II Is ranitidine/cimetidine plus a proton pump inhibitor of use in relieving oesophagitis caused by GOR? A randomised, double-blind trial in 32 children with reflux oesophagitis (Cucchiara, 1989) compared cimetidine 30-40 mg/kg/day (n=17) with placebo (n=15) over a 12 week period. 12 patients in the cimetidine group were healed compared to 3 in the placebo group. Ranitidine is considered as first-line treatment for children with severe oesophagitis and case series have shown successful healing in 75-95% of children aged 3 months to 16 years given 6-8 mg/kg/day for 8 weeks (Kelly, 1994). A dose of 2mg/kg twice daily in infants has been shown to reduce the time that gastric pH was <4 by 44% (Sutphen, 1989). With thrice daily dosing the reduction was 90%. The increased gastric pH effect has been shown to last 9-10 hours in infants (Mallet, 1989). Although there have been no RCTs of ranitidine for oesophagitis in children, expert opinion considers it as effective as cimetidine (Anon, 2001). A study of 15 children with severe reflux given omeprazole when H2 antagonists and prokinetics had failed (Gunasekaran, 1993) found that doses of between 10mg and 60mg daily were needed to normalise oesophageal pH. A similar study in 12 infants (Alliet, 1998) found that omeprazole 0.5mg/kg/day for 6 weeks resulted in complete resolution in 9 and improvement in the other 3. 6 out of 10 patients who responded well to omeprazole in doses of 20-40mg/day relapsed when therapy was discontinued after 3 months, raising the possibility that long-term treatment may be necessary (De Giacomo, 1997).
Although administering H2RAs and PPIs together can inhibit the efficacy of the latter (Anon, 2001), step-up therapy, in which treatment begins with an H2RA at standard dosage, followed by a PPI at standard dosage and then a PPI at higher dosage if necessary, is effective (Gunasekaran, 1993). Step-down therapy, which is the reverse approach, has been recommended for adults (Dent, 1999) but the two approaches have not been compared in children. A randomised controlled trial of famotidine in 35 infants (Orenstein, 2003) found significant improvements in regurgitation frequency (p=0.04) and volume (p=0.01), and crying time (p=0.027) when compared to placebo. Although no randomised controlled trials on the use of PPIs in children have yet been carried out (Rudolph, 2003), a double-blind, placebo-controlled trial in 30 infants (Moore, 2003) that did not use randomisation found that omeprazole resulted in a significant fall in the reflux index (-8.9% +/- 5.6% vs 1.9% +/-2.0, p<.001). A systematic review of 12 studies (van der Pol, 2011) found no evidence that PPIs were of benefit in newborns.
Alliet P, Raes M, Bruneel E, et al. Omeprazole in infants with cimetidine-resistant peptic esophagitis. J Pediatr 1998;132:352-4 Anon. Guidelines for evaluation and treatment of gastroesophageal reflux in infants and children: recommendations of the North American Society for Pediatric Gastroenterology and Nutrition. J Pediatr Gastroenterol Nutr 2001;32(Suppl 2):S1-S31 Cucchiara S, Gobio-Casali L, Balli F, et al. Cimetidine treatment of reflux esophagitis in children: an Italian multicentric study. J Pediatr Gastroenterol Nutr 1989;8:150-6 De Giacomo C, Bawa P, Franceschi M, et al. Omeprazole for severe reflux esophagitis in children. J Pediatr Gastrenterol Nutr 1997;24:528-32 Dent J, Brun J, Fendrick AM, et al. An evidence-based appraisal of reflux disease management: the Genval Workshop report. Gut 1999;44(Suppl 2):S1-S16 Gunasekaran TS, Hassall EG. Efficacy and safety of omeprazole for severe gastroesophageal reflux in children. J Pediatr 1993;123:148-54 Kelly DA. Do H2 receptor antagonists have a therapeutic role in childhood? J Pediatr Gastroenterol Nutr 1994;19:270-6 Mallet E, Mouterde O, Dubois F, et al. Use of ranitidine in young infants with gastro-oesophageal reflux. Eur J Clin Pharmacol 1989;36:641-2 Moore DJ, Tao BS, Lines DR, et al. Double-blind placebo-controlled trial of omeprazole in irritable infants with gastroesophageal reflux. J Pediatr 2003;143:219-23 Orenstein SR, Shalaby TM, Devandry SN, et al. Famotidine for infant gastro-oesophageal reflux: a multi-centre, randomized, placebo-controlled, withdrawal trial. Aliment Pharmacol Ther 2003;17:1097107 Rudolph CD. Are proton pump inhibitors indicated for the treatment of gastroesophageal reflux in infants and children? J Pediatr Gastroenterol Nutr 2003;37(Suppl 1):S60-S64 Sutphen JL, Dillard VL. Effect of ranitidine on twenty-four-hour gastric acidity in infants. J Pediatr 1989;114:472-4 van der Pol RJ, Smits M, van Wijk MP, et al. Efficacy of proton-pump inhibitors in children with gastroesophageal reflux disease: a systematic review. Pediatrics 2011;127:925-35
Gastroschisis 2011-13
GASTROSCHISIS
DEFINITION
Congenital defect of the anterior abdominal wall, resulting in herniation of the abdominal contents. The herniated viscera are not covered by any surrounding membranes and are exposed to amniotic fluid during pregnancy and air following delivery
DIAGNOSIS
Majority of cases diagnosed on antenatal ultrasound scan Refer mothers carrying a baby with suspected gastroschisis to a fetal medicine department for further assessment Paediatric surgeon will discuss antenatal care with parents
PRE-DELIVERY
Babies with a gastroschisis will usually be induced in a tertiary surgical centre (provided a neonatal surgical cot is available) If an unplanned delivery is imminent in another hospital, neonatal SpR or consultant will discuss with on-call paediatric surgical consultant or SpR at planned place of surgery If no surgical cot is available there and delivery cannot be postponed, speak to surgical team at one of the surrounding units e.g. Leicester, Birmingham, Nottingham and Liverpool Once baby is induced or mother in labour, inform transport team
DELIVERY
Neonatal SpR and SHO or ANNP attend delivery Take a gastroschisis bag (large sterile bag which can be closed around babys chest with a draw-string), or transparent cling film and a size 8 FR nasogastric (NG) tube Babies become cold very quickly and experience fluid loss from the exposed bowel. Perform the following, as rapidly a possible: person delivering baby to place a plastic cord clamp (not artery forceps), on umbilical cord approximately 5 cm from babys abdomen, checking cord clamp securely fastened. If in doubt, apply a second plastic cord clamp adjacent to the first dry upper part of baby quickly initiate ABC resuscitation as required but avoid prolonged mask ventilation. If prolonged resuscitation necessary, intubate pass a size 8 FR NG tube and fix securely with tape empty babys stomach by aspirating NG tube with a 10 or 20 mL syringe. If <20 mL of fluid aspirated, check position of tube place tube on free drainage by connecting to a bile bag If stomach protruding through defect (Image 1), ensure it is decompressed
Gastroschisis 2011-13
Image 1
Right Left
Gastroepiploic vessel is a longitudinal vessel running along the greater curvature of the stomach and helps identify the stomach from the bowel
Image 1 If stomach cannot be decompressed, call surgical registrar for further advice. Failure to decompress the stomach can cause pressure on the bowel mesentery resulting in bowel ischaemia Assess colour and alignment of bowel Put on two pairs of sterile gloves and carefully handle the bowel to ensure it is not twisted or kinked and there is no traction on the mesentery (Image 2) Image 2
To head
Gastroschisis 2011-13
Image 2 Place baby onto the side corresponding to the side of the defect (usually right), place a folded nappy just slightly under baby and rest bowel onto this to support it Check perfusion of bowel. If vascular compromise suspected, call consultant neonatologist if vascular compromise persists, inform on-call surgical team at planned place of surgery immediately Place babys legs and trunk into gastroschisis bag, feet first, and pull draw-string under babys arms so both arms are outside top of bag Alternatively, cover and support intestines with cling film from upper chest to lower abdomen, holding intestines in central position ensure intestines are visible do not wrap cling film tightly as this will reduce perfusion Show baby to parents and transfer to Neonatal Unit (NNU) Check perfusion of bowel again immediately before transfer to NNU and at least every 15 min thereafter
IN NNU
Inform transport team that baby is born and will need transfer within 2 hr Monitor perfusion and alignment of bowel at least every 15 min Aspirate NG tube again and record volume Insert an IV cannula, Avoid potential long line veins as baby will require many weeks of parenteral nutrition Avoid umbilical lines Infuse 20 mL/kg sodium chloride 0.9% over 1 hr and start routine IV maintenance fluids see IV fluid therapy guideline Start antibiotics: cefuroxime 25 mg/kg IV 12-hrly metronidazole: loading dose 15 mg/kg IV over 10 min, then 7.5 mg/kg IV 12-hrly Give Vitamin K IM into deltoid muscle according to local policy or Neonatal Formulary Inform staff at the Surgical Unit, that baby is ready for transfer. Have available: name gestational age weight ventilatory and oxygen requirements mothers name and ward (if mother admitted)
Blood samples Baby Blood culture FBC and clotting studies, including fibrinogen U&E Blood glucose Capillary blood gas Check with planned place of surgery if a sample from baby for Group & Save, Coombs or cross-match is required Mother Obtain sample of mothers blood for cross-match handwrite form, completing all relevant sections fully. Indicate this is the mother of baby being transferred and include babys name. This information will be required by Surgical Unit blood bank
Gastroschisis 2011-13
If evidence of a coagulopathy, treat with fresh frozen plasma (FFP) or cryoprecipitate, as appropriate see Coagulopathy guideline Aspirate NG tube hourly and replace aspirate volume, mL-for-mL with IV sodium chloride 0.9% with 10 mmol potassium chloride in each 500 mL bag Leave the NG tube on free drainage
DOCUMENTATION
Take photographs for parents Complete nursing and medical documentation for transfer and obtain copies of X-rays if taken Ensure details of mothers name and ward (including direct dial telephone number) are included If mother discharged, obtain contact telephone number (including mobile). If operation necessary and a parent unable to attend Surgical Unit, surgeon will require verbal telephone consent
During transport Carry out and document usual observations, include bowel perfusion and alter its position if necessary Arrival at Surgical Unit Record bowel perfusion and alignment
GOR 2011-13
INVESTIGATIONS
Litmus test (pH paper on two occasions) for acid in oropharyngeal secretions (OPS), but not after stimulation for apnoea or bradycardia (which can cause a GOR episode) and not immediately after a feed 24 hr pH monitoring is of limited value in preterm babies. Consider in cases where repeated apnoea/bradycardia is resistant to other measures If repeated apnoeas/bradycardia, consider 24 hr pulse oximetry recordings to assess extent of problem and relationship to feeding If apnoeas/bradycardia persist at term-equivalent, perform oral contrast study looking at suck-swallow feeding patterns to exclude aspiration, GOR and anatomical malformations
MANAGEMENT
Position Head upwards, at an angle of 30o Nurse baby prone or in left lateral position if monitored Feeding Frequent low volume feeds Avoid overfeeding Infant Gaviscon (1 dose = half dual sachet) breastfed: give during or after a feed (add 5 mL sterile water/milk to make a paste, then add another 510 mL and give with a spoon) bottle fed: add to at least 115 mL of milk NG fed: make up with 5 mL water and give 1 mL per 25 mL of feed Caution: Gaviscon contains sodium 0.92 mmol/half a dual sachet If symptoms persist, consider change to Instant Carobel (will thicken with cold or handwarm milk). Add 2 scoops to 100 mL shake well and leave for 34 min to thicken. Shake feed again and give immediately. Take care that thickened liquid does not block fine bore nasogastric tube Warning: do not give Gaviscon and Carobel together as this will cause the milk to become too thick
GOR 2011-13 Drugs If above measures fail, add ranitidine 2 mg/kg orally 8 hrly or 500 microgram/kg IV 12-hrly if <32 weeks gestation and in the first week of life, increasing to 68 hrly if >32 weeks and/or >7 days old then titrated to gastric pH Give over 35 min (BNFc) as rapid administration can cause arrhythmias If still no improvement, change ranitidine to omeprazole 700 microgram/kg/day and add domperidone 300 microgram/kg 48 hrly Other measures If symptoms persist, consider other measures after discussion with consultant e.g. erythromycin 6 mg/kg four times daily cows milk protein-free formula (in artificially fed infants) some neonates with suspected CMPI are also allergic to hydrolysate formula and will respond to an amino acid-based formula assessment by speech and language therapy team as poor suck-swallow coordination can result in aspiration during feeds if unable to protect airway; can also occur following an episode of GOR
Jaundice 2011-13
JAUNDICE
(Based on NICE Neonatal jaundice guideline published in 2010)
Jaundice 2011-13
Babys blood group and direct Coombs test (interpret result taking into account strength of reaction and whether mother received prophylactic anti-D immunoglobulin during pregnancy) Mothers blood group and antibody status (should be available from maternal case notes) PCV
Plus (if clinically indicated) Full infection screen (in an ill baby) G6PD concentration (if indicated by ethnic origin: Mediterranean, Middle Eastern, South East Asian) FBC and film Persistent jaundice >14 days term infant; >21 days preterm infant (see Liver dysfunction guideline) Total and conjugated bilirubin Examine stool colour FBC Babys blood group and direct Coombs test (interpret result taking into account strength of reaction and whether mother received prophylactic anti-D immunoglobulin during pregnancy) Ensure routine metabolic screening performed (including screening for hypothyroidism) Urine culture Baby with conjugated bilirubin >25 micromol/L, refer urgently to a specialist centre 2nd line investigations (not in NICE guideline) Liver function test (ALT, Albumin, GGT) Coagulation profile G6PD screen in African, Asian or Mediterranean patients Thyroid function tests: ask for FT4 priority and then TSH Congenital infection screen Urine for CMV PCR, toxoplasma ISAGA-IgM and throat swab for HSV culture/PCR Metabolic investigations, e.g: blood galactose-1-phosphate urine for reducing substances alpha-1-antitrypsin
Jaundice 2011-13
Provided bilirubin not significantly elevated, encourage breaks of up to 30 min for breastfeeding, nappy change and cuddles Do not give additional fluids routinely During multiple phototherapy do not interrupt for feeds monitor hydration by weighing baby daily and assessing wet nappies
Monitoring during phototherapy Repeat serum bilirubin 46 hr after starting treatment Repeat serum bilirubin 612 hrly when bilirubin stable or falling Stop phototherapy once serum bilirubin has fallen to at least 50 micromol/L below threshold Check for rebound jaundice with repeat serum bilirubin 1218 hr after stopping phototherapy
Table: Limits for phototherapy and exchange transfusion for infants >38 weeks gestation Age (hours) Serum bilirubin Serum bilirubin Serum bilirubin Serum bilirubin (micromol/L) (micromol/L) (micromol/L) (micromol/L) 0 >100 >100 6 12 18 24 30 36 42 48 54 60 66 72 78 84 90 96+ ACTION >100 >100 >100 >100 >112 >125 >137 >150 >162 >175 >187 >200 Repeat transcutaneous bilirubin/serum bilirubin (612 hr) >112 >125 >137 >150 >162 >175 >187 >200 >212 >225 >237 >250 >262 >275 >287 >300 >125 >150 >175 >200 >212 >225 >237 >250 >262 >275 >287 >300 >312 >325 >337 >350 >150 >200 >250 >300 >350 >400 >450 >450 >450 >450 >450 >450 >450 >450 >450 >450 Perform exchange transfusion
Consider Start phototherapy phototherapy (repeat transcutaneous bilirubin/serum bilirubin in 6 hr) Source: http://www.nice.org.uk/guidance/CG98
Jaundice 2011-13
Treatment graphs giving the phototherapy and exchange transfusion limits for each gestational age can be printed from http://www.nice.org.uk/guidance/CG98 under the heading Implementing this guideline then CG98 Neonatal Jaundice: treatment threshold graphs
What is the incidence of prolonged neonatal jaundice in term and preterm newborns? Jaundice persisting beyond 14 days of age (prolonged jaundice) can (rarely) be a sign of serious underlying liver disease (Hussein, 1991). Jaundice persists beyond 14 days in 1540% of breastfed infants, depending on the series studied (Hannam, 2000). A prospective study of all 7139 term infants born at Kings College Hospital (London) between January 1997 and June 1998 (Hannam, 2000) found 154 with prolonged jaundice, one of which had conjugated hyperbilirubinaemia (0.14 per 1000 live births). Another study of 3661 babies in Sheffield (Crofts, 1999) found 127 who were jaundiced at 28 days, of which 125 were breastfed (9.2%). Although preterm infants, whose livers are more immature, have prolonged jaundice more commonly than term infants (Fenton, 1998) there appear to be no studies of incidence in this group (Lucas, 1986). The first large, prospective study of severe hyperbilirubinaemia in UK infants in the first month of life (Manning, 2007) found an incidence of 0.7 per 1,000 live births (95% CI 0.5 0.8).
Crofts DJ, Michel VJ, Rigby AS, et al. Assessment of stool colour in community management of prolonged jaundice in infancy. Acta Paediatr 1999;88:969-74 Fenton TR, Gastrointestinal problems and jaundice of the newborn. In: Campbell AG, McIntosh N, (eds). Forfar and Arneils Textbook of pediatrics, 5th ed. New York: Churchill Livingstone, 1998. p219 Hannam S, McDonnell M, Rennie JM. Investigation of prolonged neonatal jaundice. Acta Paediatr 2000;89:694-7 Hussein M, Howard ER, Mieli-Vergani G, et al. Jaundice at 14 days of age: exclude biliary atresia. Arch Dis Child 1991;66:1177-9 Lucas A, Baker BA. Breast milk jaundice in premature infants. Arch Dis Child 1986;61:1063-7 Manning D, Todd P, Maxwell M, et al. Prospective surveillance study of severe hyperbilirubinaemia in the newborn in the UK and Ireland. Arch Dis Child Fetal Neonatal Ed 2007;92:F342-6
Evidence Level: IV When does serum bilirubin level of a neonate fall to adult level? High serum bilirubin levels in the first days of life decline during the next several weeks to the values commonly found in adults (Dennery, 2001). This time period is inexact, although 14 days is commonly accepted as a cut-off point for investigation of sustained jaundice (Fenton, 1998).
Dennery PA, Seidman DS, Stevenson DK. Neonatal hyperbilirubinemia. N Engl J Med 2001;344:581-90 Fenton TR, Gastrointestinal problems and jaundice of the newborn. In: Campbell AG, McIntosh N, (eds). Forfar and Arneils Textbook of pediatrics, 5th ed. New York: Churchill Livingstone, 1998. p214
Evidence Level: V What is the incidence of glucose-6PD deficiency in British white children? Glucose-6PD deficiency is most common amongst Greek, Sardinian, Chinese, Jamaican and South East Asian populations (Beutler, 1994;Valaes, 1994; Singh, 1986; Doxiadis, 1961). There appear to be no epidemiological studies in British white children. The prevalence amongst white northern European populations has been estimated as less than 1 in 1,000 (Beutler, 1995).
Beutler E. Glucose-6-phosphate dehydrogenase deficiency and other enzyme abnormalities. In: Beutler th E, Lichtman MA, Coller BS, et al (eds). Williams Hematology, 5 ed. New York, McGraw-Hill, 1995. p572 Beutler E. G6PD deficiency. Blood 1994;84:3613-36 Doxiadis SA, Fessas P, Valaes T. Glucose-6-phosphate dehydrogenase deficiency: a new aetiological factor of severe neonatal jaundice. Lancet 1961;i:297-301 Singh H. Glucose-6-phosphate dehydrogenase deficiency: a preventable cause of mental retardation. BMJ 1986;292:397-8 Valaes T. Severe neonatal jaundice associated with glucose-6-phosphate dehydrogenase deficiency: pathogenesis and global epidemiology. Acta Paediatr Suppl 1994;394:58-76
Evidence Level: V What is the incidence of hereditary spherocytosis presenting with prolonged neonatal jaundice only? The incidence of hereditary spherocytosis in Northern Europeans has been estimated at 1:5,000 (Morton, 1962), although milder forms may be asymptomatic and therefore the true incidence may be higher. A recent review (Delhommeau, 2000) has taken this into consideration and suggested an incidence of 1:2,000. This condition has received little attention in the neonatal period (Delhommeau, 2000) and consequently no information can be identified concerning prolonged jaundice as the sole presenting symptom.
Delhommeau F, Cynober T, Schischmanoff PO, et al. Natural history of hereditary spherocytosis during the first year of life. Blood 2000;95:393-7 Morton NE, MacKinney AA, Kosower N, et al. Genetics of spherocytosis. Am J Hum Genet 1962;14:170
Evidence Level: V What percentage of urinary tract infection in newborns presents with jaundice only? The association of urinary tract infection with neonatal jaundice has been well-recognised (Anon, 1971; Arthur, 1967), but no percentages can be identified for newborns presenting with jaundice alone. Most infants in published series have anaemia and/or septicaemia in addition to their jaundice (Hannam, 2000). Jaundice as the main presenting symptom of UTI appears to predominate in male infants at a ratio of 3:1 (Seeler, 1969), unlike the female preponderance generally found in paediatric UTI. A study in 102 infants with asymptomatic, unexplained indirect hyperbilirubinaemia in the first two weeks of life (Bilgen, 2006) found UTI in 8 cases (8%). The authors concluded that urine culture should be considered in the bilirubin work-up of infants older than three days of age with an unknown etiology.
Anon. Urinary tract infection presenting as jaundice. BMJ 1971;iii:546-7 Arthur AB, Wilson BD. Urinary infection presenting with jaundice. BMJ 1967;i:539-40 Bilgen H, Ozek E, Unver T et al. Urinary tract infection and hyperbilirubinemia. Turk J Pediatr 2006; 48:515 Hannam S, McDonnell M, Rennie JM. Investigation of prolonged neonatal jaundice. Acta Paediatr 2000;89:694-7 Seeler RA, Hahn K. Jaundice in urinary tract infection in infancy. Am J Dis Child 1969;118:553-8
Evidence Level: V At what level of total serum bilirubin (TSB) does kernicterus occur in a) the term baby b) the preterm baby of 32 weeks? At what level should phototherapy be started in the term baby? The American Academy of Pediatrics (Anon, 2004) states that It is not known at what bilirubin concentrationsignificant risk of brain damage occurs or when the risk of damage
exceeds the risk of treatment. Cases of kernicterus have occurred at TSB levels below 200 micromol/l (Gustafson, 1995). This level of uncertainty persists (Wennberg, 2006): There are insufficient published data to precisely define sensitivity and specificity (of TSB) in determining risk for acute bilirubin neurotoxicity or chronic sequelae (kernicterus). One authority (Ives, 1999) suggests that the threshold lies somewhere between 400 and 650 micromol/l. The AAP (Anon, 1994) recommends exchange transfusion and intensive phototherapy when serum bilirubin is >/= 430 mmol/l if age 25-48 hours or >/= 510 mol/l if >48 hours. Standard phototherapy should begin at 257 micromol/l or 308 microml/l for the same age bands, in the term or near term infant. Data from the Pilot Kernicterus Registry (Johnson, 2002) showed that the median total serum bilirubin (TSB) concentration of infants on readmission to hospital with kernicterus was 600 micromol/l (350 mg/l). The most recent information on this subject (Bhutani, 2004) indicates that TSB concentrations of >342 micromol/l (>200 mg/l) should be a cause for concern and that values >/= 513 micromol/l (>/= 300 mg/l) should be considered dangerous. TSB concentrations are, however, poor predictors of bilirubin toxicity in the sick or preterm infant (Bhutani, 2004). Although free or unbound bilirubin may provide a more accurate measure, no tests for this have been validated to date (Bhutani, 2004). A sliding scale has been suggested, based on infant weight, to indicate when intensive phototherapy should be started, but exchange transfusion is recommended when TSB >190 mL/kg (Bhutani, 2004). NICE guidelines (2010) found that There is a lack of good-quality evidence on the association between hyperbilirubinaemia and kernicterus or other adverse sequelae.
American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 35 or more weeks of gestation. Pediatrics 2004;114:297-316 Bhutani VK, Johnson LH. Urgent clinical need for accurate and precise bilirubin measurements in the United States to prevent kernicterus. Clin Chem 2004;50:477-80 Gustafson PA, Boyle DW. Bilirubin index: a new standard for intervention? Med Hypotheses 1995;45:409-16 Ives NK. Neonatal jaundice. In: Rennie JM, Roberton NR, eds. Textbook of neonatology, 3rd ed. Edinburgh, Churchill Livingstone, 1999. p721 Johnson LH, Bhutani VK, Brown AK. System-based approach to management of neonatal jaundice and prevention of kernicterus. J Pediatr 2002;140:396-403 Wennberg RP, Ahlfors CE, Bhutani VK, et al. Toward understanding kernicterus: a challenge to improve the management of jaundiced newborns. Pediatrics 2006;117:474-85
Evidence Level: V Can gamma-glutamyl transpeptidase (GGT) be useful in distinguishing neonatal hepatitis (NH) from extrahepatic biliary atresia (EHBA)? A study in 132 patients (Arora, 1992) found that serum GGT at a cut-off level maintaining 100% sensitivity for EHBA (< 150 IU L(-1)), used in conjunction with non-excreting 99mTcmebrofenin IDA scans, reduced the false positivity of individual tests. In this series, operative cholangiograms would have been avoided in 21 patients having both tests, vs 9 when only IDA scan was performed. A study in 47 infants with EHBA, 10 with NH and 130 age-matched healthy controls (Yamagiwa, 1996), noted significant differences in GGT levels between the EHBA and NH infants at 6 weeks of age (314 +/- 232 IU/L vs 69 +/- 58 IU/L). A much earlier study in 17 infants aged 5-16 weeks (Wright, 1960) found that the mean maximal GGT level in NH patients (183 +/- 54 IU/L) was significantly lower than that found in EHBA patients (760 +/- 492 IU/L).
Arora NK, Kohli R, Gupta DK, et al. Hepatic technetium-99m-mebrofenin iminodiacetate scans and serum gamma-glutamyl transpeptidase levels interpreted in series to differentiate between extrahepatic biliary atresia and neonatal hepatitis. Acta Paediatr 2001;90:975-81
Wright K, Christie DL. Use of gamma-glutamyl transpeptidase in the diagnosis of biliary atresia. Am J Dis Child 1960;135:134-6 Yamagiwa I, Iwafuchi M, Obata K, et al. Pre-operative time course changes in liver function tests in biliary atresia: its usefulness in the discrimination of biliary atresia in early infancy. Acta Paediatr Jpn 1996;38:506-12
Evidence Level: IV What is the optimal dose and duration of treatment in total parenteral nutrition (TPN) related cholestasis? To date, there is no universally accepted treatment for intractable TPN-associated cholestasis (Al-Hathlol, 2006). BNF for Children advises ursodeoxycholic acid (UDCA), 10 mg/kg 3 times a day. Most studies have included very small numbers of patients. A pilot study in 7 children (Spagnuolo, 1996) found that UDCA took 4-8 weeks to normalise biochemical markers of cholestasis. Another, in 13 infants (Al-Hathlol, 2006) used a dose of 15-20 mg/kg/day and found that although patients responded from the second week of therapy, about four months of treatment were needed before normalisation occurred. An alternative treatment is cholecystokinin, which needs to be administered intravenously for 3-5 days in a dose of 2-4 IDU/kg (Teitelbaum, 1997; Teitelbaum, 1995; Rintala, 1995).
Al-Hathlol K, Al-Madani A, Al-Saif S, et al. Ursodeoxycholic acid therapy for intractable total parenteral nutrition-associated cholestasis in surgical very low birth weight infants. Singapore Med J 2006;47:14751 Rintala RJ, Lindahl H, Pohjavuori M. Total parenteral nutrition-associated cholestasis in surgical neonates may be reversed by intravenous cholecystokinin: a preliminary report. J Pediatr Surg 1995;30:827-30 Spagnuolo MI, Iorio R, Vegnente A, et al. Ursodeoxycholic acid for treatment of cholestasis in children on long-term parenteral nutrition: a pilot study. Gastroenterology 1996;111:716-9 Teitelbaum DH. Parenteral nutrition-associated cholestasis. Curr Opin Pediatr 1997;9:270-5 Teitelbaum DH, Han-Markey T, Schumacher RE. Treatment of parenteral nutrition-associated cholestasis with cholecystokinin-octapeptide. J Pediatr Surg 1995;30:1082-5
Evidence Level: IV What are the most appropriate tests to be ordered for prolonged jaundice? A prospective study in 144 infants (Hannam, 2000) concluded that the number of investigations may safely be reduced to: a total and conjugated bilirubin, packed cell volume, glucose-6-phosphate dehydrogenase level (where appropriate), a urine for culture and inspection of a recent stool sample for bile pigmentation.
Hannam S, McDonnell M, Rennie JM. Investigation of prolonged neonatal jaundice. Acta Paediatr 2000;89:694-7
CAUSES
Not all liver dysfunction in preterm infants is caused by parenteral nutrition. Extra-hepatic biliary atresia does occur and must be diagnosed and managed in a timely fashion Neonatal hepatitis Isolated Associated with: parenteral nutrition maternal diabetes hydrops fetalis trisomy 21 Endocrine Hypopituitarism Hypothyroidism Metabolic 1-antitrypsin deficiency Cystic fibrosis Galactosaemia Dubin-Johnson syndrome Bile acid disorder Haemochromatosis Toxins/injury Parenteral nutrition Multifactorial preterm Haemolytic disease Hypoxia
Biliary tract disorders Extra-hepatic biliary atresia Bile duct stricture Choledochal cyst Alagille syndrome Non-syndromic bile duct paucity Infection Cytomegalovirus Toxoplasmosis Sepsis
INVESTIGATIONS
Aim to diagnose causes of liver dysfunction that will benefit from early diagnosis while avoiding unnecessary transfer and investigation of small sick infants First-line investigations Complete following as soon as possible: coagulation screen transaminases, bilirubin (total and conjugated), albumin, gamma GT, and alkaline phosphatase galactosaemia and tyrosinaemia screen 1-antitrypsin concentration and phenotype serum cortisol, T4 and TSH stool in opaque pot for consultant review urine for M,C&S abdominal ultrasound scan, after 4 hr fast if possible, to include liver and gallbladder examination if clinical suspicion high, toxoplasma serology, CMV IgM and urine PCR for CMV, syphilis serology, viral culture from swabs of vesicles for herpes simplex
Liver dysfunction 2011-13 if metabolic disorder suspected, plasma lactate, plasma and urine amino acids, and urine organic acids As they become available, discuss results of liver function, coagulation, stool colour, weight gain and abdominal ultrasound with liver unit team
FURTHER INVESTIGATIONS
Standard aggressive protocol used to investigate term infants is inappropriate in preterm infants because of: insufficient blood volume for blanket testing poor temperature control when attending for isotope scans limited size increases risk of liver biopsy Transfer to specialist centre often not possible owing to need for ongoing respiratory support and neonatal nursing care Preterm infants with diagnoses requiring surgery (e.g. Kasai procedure for biliary atresia) need to be more than term-corrected age or weigh at least 2 kg before surgery considered Early isotope scanning not widely available and of limited value, many infants can be investigated without this procedure Assessment of stool colour can determine which infants with cholestasis require urgent further investigation, as shown below:
Pigmented stools
Acholic stools
USS (4 hr fast)
Gallbladder normal
Excretion
No excretion
Ongoing medical assessment with liver team, nutritional management, vitamin supplementation and ursodeoxycholic acid
Investigations for ongoing liver dysfunction Preterm infants with persistent liver dysfunction but initially normal gallbladder size or an excreting isotope scan can be further investigated locally, discuss with liver team If indicated by results of first-line investigations or progressive dysfunction, consider: ophthalmic review (other than for retinopathy of prematurity) karyotype for dysmorphism very long-chain fatty acids for neurological abnormality
urinary bile salts isotope scan, liver biopsy or bone marrow aspirate
MANAGEMENT OF CHOLESTASIS
Surgical correction, if appropriate (e.g. Kasai, choledochal cyst), usually when >2 kg or term-corrected age, discuss individual cases with liver team Nutrition to overcome malabsorption of long-chain fat and fat-soluble vitamins if breastfeeding, continue unless weight gain or linear growth inadequate if breastfeeding not available or failing to thrive, provide high-calorie diet aiming for 120 150% of estimated average with increased percentage of fat as medium-chain triglycerides (such as Pepti-Junior) OR supplement breast milk with medium-chain triglyceride fat additives, seek advice from liver unit team if individually prescribed modular feed required: co-ordinated by liver unit dietitians while infant is in-patient on liver unit or attending their out-patient clinic Prescribe vitamins during cholestasis and for 3 months following resolution of jaundice; doses required will need monitoring and adjustment if still required after discharge (coordinated by liver team): vitamin K 1 mg daily: monitor PT and APTT vitamin A 25005000 units daily: monitor serum vitamin A vitamin E 50 mg daily: monitor serum vitamin E alfacalcidol 20 nanogram/kg daily: monitor bone biochemistry
Ursodeoxycholic acid NNF dose 15 mg/kg once/day but liver team will normally recommend 2030 mg/kg/day in divided doses for most preterm infants until jaundice resolves, and to stimulate bile flow in infants and children with cystic fibrosis Parenteral nutrition (PN) Wherever possible, feed enterally, as even small amounts have trophic effects on gut, reduce bacterial colonisation and promote bile flow Discontinue PN as soon as possible in all preterm infants with cholestasis Specific treatments Infants with cystic fibrosis, galactosaemia, tyrosinaemia type 1, hypopituitarism, hypothyroidism or bile acid disorders require additional targeted management and lifelong follow-up shared by local teams and appropriate specialists
FOLLOW-UP
For infants with persistent cholestasis, arrange out-patient follow-up with liver team after discharge from neonatal unit If liver dysfunction has resolved, no follow-up with liver team necessary For all others with a specific diagnosis, follow-up will be directed by liver team, appropriate specialists and local consultant Long term hepatic outcome excellent, majority resolve within first year
NEC 2011-13
NEC 2011-13
associated with early postnatal corticosteroids or indometacin often seen at terminal ileum with normal bowel abdominal X-ray does not show evidence of pneumatosis intestinalis Systemic candidiasis: clinical signs can mimic NEC with abdominal distension, metabolic disturbances, hypotension and thrombocytopenia
INVESTIGATIONS
Abdominal X-ray Supine antero-posterior view If perforation suspected but not clear on supine view, left lateral view Not all infants will have radiological findings associated with NEC Blood tests FBC: anaemia, neutropenia and thrombocytopenia often present; early return to normal carries good prognosis Blood film: evidence of haemolysis and toxic changes (e.g. spherocytes, cell fragments, polychromatic cells) C-reactive protein, but a normal value will not be helpful in initial phase Urea and electrolytes Blood gas: evidence of metabolic acidosis (base excess more negative than 10) Coagulation screen Blood cultures
IMMEDIATE TREATMENT
Always discuss management with senior neonatologist In all stages Transfer baby to neonatal intensive care and nurse in incubator to avoid cross infection If respiratory failure and worsening acidosis, intubate and ventilate Nil-by-mouth Gastric decompression free drainage with large nasogastric tube (size 8) or continuous suction with 10 cm water NEC often associated with significant third spacing of fluid into mesentery Triple antibiotics: penicillin/ampicillin and gentamicin and metronidazole IV fluids/TPN: total volume 150 mL/kg or less Long line when stable Pain relief, consider morphine/diamorphine infusion (see Pain and stress guideline) Stage 2: Proven NEC (confirmed radiologically) If breathing supported by nasal CPAP, elective intubation to provide bowel decompression Give IV fluid resuscitation 10 mL/kg sodium chloride 0.9% for shock and repeat as necessary. Shock is most common cause of hypotension in babies with NEC. See Hypotension guideline If coagulation abnormal, give FFP (see Coagulopathy guideline) If thrombocytopenia and/or anaemia occur, transfuse (see Thrombocytopenia guideline) Discuss with surgical team: may need transfer to surgical centre Stage 3 : Advanced NEC (fulminant NEC with or without intestinal perforation) Treat as for stage 2 and refer to surgical team: may need laparotomy or resection of bowel in surgical centre
NEC 2011-13
SUBSEQUENT MANAGEMENT
In recovery phase In Stage 1, if no progression after 48 hr, consider restarting feeds slowly (see Nutrition guideline) and stopping antibiotics In Stage 2, if abdominal examination normal after 10 days, consider restarting feeds some may need longer period of total gut rest stop antibiotics after 710 days In Stage 3, discuss with surgeon and dietitian before restarting feeds Late complications Recurrence (in about 10%) Strictures (in about 10% non-surgical cases) Short bowel syndrome and problems related to gut resection
MONITORING TREATMENT
Observe general condition closely and review at least 12-hrly Daily acid-base fluid balance (twice daily if condition unstable) electrolytes (twice daily if condition unstable) FBC and coagulation (twice daily if condition unstable) repeat X-ray daily or twice daily until condition stable. Discuss with consultant
LONG-TERM MANAGEMENT
Advise parents about signs of bowel obstruction Medical +/- surgical follow-up after discharge Contrast studies if clinically indicated for stenosis Appropriate developmental follow-up
Nutrition 2011-13
NUTRITION
AIMS
To achieve growth and nutrient accretion similar to intrauterine rates To achieve best possible neurodevelopmental outcome To prevent specific nutritional deficiencies
NUTRITIONAL REQUIREMENTS
Daily recommended intake of nutrients for stable/growing preterm infants <1000 g 10001500 g Fluids (mL/kg) 160220 135190 Energy (kcal/kg) 130150 110130 Protein (gm/kg) 3.84.4 3.44.2 3.3 g/100 kcal 2.8 g/100 kcal Protein energy ratio Sodium (mmol/kg) 35 35 Calcium (mmol/kg) 2.55.5 2.55.5 Phosphorus (mmol/kg) 1.94.5 1.94.5 Vitamin A (IU/kg) 700 500 7001500 Vitamin D (IU/kg) 150400 150400
FEEDING GUIDE
Nutrient composition of commonly used milks per 100 mL Preterm Fortified breast milk breast milk (Nutriprem) 6074 7690 Energy (kcal) 1.41.9 2.22.7 Protein (g) 0.61.7 1.02.1 Sodium (mmol) 0.61 2.22.6 Calcium (mmol) 0.350.6 1.72.0 Phosphorus (mmol) SMA LBW 82 2.2 1.8 2.5 1.9 Nutriprem 1 80 2.4 1.8 2.5 1.6
Route of administration Most infants <34 weeks cannot co-ordinate sucking, swallowing and breathing effectively and must be tube fed use gastric feeding with either nasogastric or orogastric tube Feeding schedules Gastrointestinal tolerance of feeding is very variable and concomitant illness can impose additional constraints, manage feeding on an individual basis Following are general guidelines
Nutrition 2011-13
Day 25 Increase fluid volume to 150 mL/kg/day taking into consideration: clinical condition weight profile urine output serum electrolytes renal function Commence enteral feeds if: clinical condition stable not receiving significant inotropic support (dopamine/dobutamine >5 microgram/kg/min or adrenaline) Presence of umbilical catheters is not a contraindication for feeding. In presence of absent or reversed end-diastolic flow on antenatal scans, be more cautious with enteral feeding (see Special situations) Type of milk Wherever possible, use expressed breast milk for initiation of enteral feed. Breast milk remains the ideal milk for both term and preterm infants and should be strongly recommended. However, support mothers in whatever choice they make in its absence, use preterm milk formula (SMA LBW, Nutriprem 1) Volume Stop feeds if not tolerated Day of feeding 01 2 3 4 5 6 7 8 9 10 * # Volume of milk (mL) Birth weight <750 g Birth weight 750999 g 0.5 mL hourly 0.5 mL hourly 0.5 mL hourly 1 mL hourly 1 mL hourly 1.5 mL hourly 1.5 mL hourly 2 mL hourly * 2 mL hourly * 3 mL hourly 2.5 mL hourly 4 mL hourly 3 mL hourly 5 mL hourly 3.5 mL hourly 6 mL hourly 4 mL hourly 6.5 hourly # 4.5 mL hourly # 6.5 hourly #
<500 g, 0.25 mL/hr At this stage, include enteral feeds in total intake of fluids If required
If receiving expressed breast milk Add fortifier (human breast milk fortifier) when milk tolerated at 150 mL/kg/day for 24 hr Observe for signs of feed intolerance (abdominal distension, vomiting, increased aspirates, change in stool frequency) for next 24 hr Thereafter, increase volume of milk gradually to 180220 mL/kg/day as necessary If receiving preterm milk formula 150 mL/kg/day should suffice If necessary, increase to 180 mL/kg/day on consultant advice Nutrient additives Exclusively breastfed babies <35 weeks gestation, after first week once full enteral feeds established 0.6 mL Abidec (NB contains peanut oil) or Dalavit 0.3 mL Joules phosphate 0.5 mL 12-hrly adjusted according to serum phosphate and alkaline phosphatase levels, and urinary reabsorption of phosphate
Nutrition 2011-13
Formula feed babies <35 weeks gestation, after first week once full enteral feeds established 0.6 mL Abidec (NB contains peanut oil) or Dalavit 0.3 mL unless baby receiving Nutriprem 1 or 2, or SMA 1 or 2
Iron At six weeks old and only for exclusively breastfed babies <2.3 kg at birth, start sodium feredetate (e.g. Sytron) once daily: <3 kg, 1 mL >3 kg, 2.5 mL discontinue Sytron once mixed feeding established
SUCK FEEDS
Around 34 weeks corrected gestational age, introduce such feeds after increasing feed interval to 3-hrly Some babies can be started early if experienced nursing staff deem them interested and capable
MONITORING
Monitoring of gastrointestinal tolerance, growth and biochemical balance is critical in nutritional management of preterm infants Clinical monitoring Daily assessment of gastrointestinal tolerance: gastric residues stool frequency abdominal examination as appropriate Anthropometry Monitor weight daily for first few days to assist with fluid management see Intravenous fluid therapy guideline once clinically stable, measure weight twice weekly weight gain of 1520 g/kg/day is adequate in growing phase Measure head circumference weekly to assess cerebral growth
Nutrition 2011-13
Biochemical monitoring In sick or very premature infants, measure plasma urea, electrolytes, calcium, phosphate and albumin twice daily for initial few days. Reduce frequency depending on clinical stability Monitor glucose closely in initial few days once clinical stability and full enteral feeds achieved, carry out these tests at least once a week in very low birth weight (VLBW) infants Check urine once weekly for excretion of sodium and phosphate
COMMON PROBLEMS
Poor growth Babies with weight gain <15 g/kg/day require further assessment Ensure baby receiving adequate nutrition (energy intake >120 kcal/kg/day; protein 3.3 g/100 kcal). Calculate energy and protein intake per kg/day Check for following factors, that may affect growth: clinical illness (e.g. UTI) medications (diuretics, corticosteroids) increased energy requirement resulting from respiratory/cardiac disorders hyponatraemia anaemia Excessive weight gain Babies with weight gain >25 g/kg/day require further assessment Ensure measurement not spurious and not related to catch-up growth after a period of poor weight gain Evaluate for fluid retention and its causes consider diuretics in presence of oedema If receiving >150 kcal/kg/day, reduce energy intake if applicable, change preterm milk to term formula, or decrease volume of feeds Feeding intolerance Intolerance to feeding is common among small preterm infants and most will have episodes requiring either temporary discontinuation of feeding or delay in advancing feeds Carefully observe for signs of necrotising enterocolitis (NEC) including abdominal distension, discolouration, blood in stools, metabolic acidosis see Necrotising enterocolitis guideline Gastric aspirates If aspirates non-bilious and less than half the volume of previous feed, they can be replaced and feeding continue while observing infant closely If aspirates bilious or more than half previous feed volume, consider withholding feeds on that occasion and assess for any signs of NEC
SPECIAL SITUATIONS
Reversed end diastolic flow Start enteral feeds when clinically stable Birth weight <1000 g, advance feeds using schedule above for babies with birth weight <750 g Birth weight 10001500 g, advance feeds using above schedule for babies with birth weight 750999 g Monitor closely for signs of feed intolerance or NEC Patent ductus arteriosus (PDA) Preterm infants with PDA have decreased blood-flow in descending aorta and increased risk of NEC. Indometacin is also associated with decreased gastrointestinal blood supply
Nutrition 2011-13
by Doppler ultrasound scan measurements. Observe closely for feeding intolerance and signs of NEC As increased IV fluid rates are associated with PDA, avoid any increase >150 mL/kg/day Cautiously increase feeds while receiving indometacin
POST-DISCHARGE NUTRITION
Nutrient supplements Infants <35 weeks gestation, give multivitamins (Abidec) after first week, once full enteral feeds established. Continue for first 12 months (not required if Nutriprem 2 or SMA 2 used as primary nutrition) At 6 weeks, offer iron supplements sodium feredetate (e.g. Sytron) 1 mL once daily increased to 2.5 mL daily at discharge, to breast milk fed babies of birth weight <1800 g, continuing until mixed feeding established Formula Consider post-discharge follow-on preterm milk (e.g. Nutriprem2, SMA 2) in premature babies with: chronic lung disease restricted intake (e.g. congenital heart disease) poor growth For term babies with increased energy demands or reduced intake, liaise with dietitian regarding use of a higher energy formula (e.g. SMA High Energy)
Evidence Level: I
Trophic feeding reduces the risk of necrotising enterocolitis, compared to no feeding? A Cochrane systematic review (Tyson, 2005) that included 10 randomised controlled trials comparing trophic to no feeding failed to demonstrate any difference in the incidence of necrotising enterocolitis between the two groups. Among infants given trophic feedings, however, there was an overall reduction in days to full feeding (WMD -2.6; 95% CI 4.1, -1.0), total days that feedings were held (WMD-3.1; 95% CI -4.6, -1.6), and total hospital stay (WMD -11.4; 95% CI 17.2, -5.7) compared to infants given no enteral feeds. A multicentre case-control study in 53 preterm infants (Henderson, 2009) found that the mean duration of trophic feeding (<1 ml/kg/h) was significantly shorter in the cases (of necrotising enterocolitis) than the controls (3.3 vs 6.2 days).
Henderson G, Craig S, Brocklehurst P, et al. Enteral feeding regimens and necrotising enterocolitis in preterm infants: a multicentre case-control study. Arch Dis Child Fetal Neonatal Ed 2009;94:F120-F123 Tyson JE, Kennedy KA. Trophic feedings for parenterally fed infants. Cochrane Database of Systematic Reviews 2005, Issue 3. Art. No.: CD000504
Evidence Level: I Breast milk is protective against necrotising enterocolitis, compared to formula milk? A Cochrane systematic review of 8 trials comparing breast milk to formula milk (Quigley, 2007) found, after meta-analysis of data from 5 trials, a statistically significantly higher incidence of necrotising enterocolitis in the formula fed group: typical RR 2.5 (95% CI 1.2 - 5.1); NNH 33 (95% CI 17- 100).
Quigley M, Henderson G, Anthony MY, et al. Formula milk versus donor breast milk for feeding preterm or low birth weight infants. Cochrane Database of Systematic Reviews 2007, Issue 4. Art. No.: CD002971
Evidence Level: I
PARENTERAL NUTRITION
DEFINITION
Parenteral nutrition [(PN) or total parenteral nutrition (TPN)] is the intravenous infusion of some or all nutrients for tissue maintenance, metabolic requirements and growth promotion in neonates unable to tolerate full enteral feeds Seek advice from your local TPN pharmacist
INDICATIONS FOR PN
Short-term supply of nutrients: Extremely low birth weight (<1000 g) Very low birth weight (<1500 g) AND clinically unstable, absent/reversed end-diastolic flow or full enteral feeds seem unachievable by day 5 Any baby on inotropic support Necrotising enterocolitis (1014 days) Temporary feeding intolerance Prolonged non-use of gastrointestinal (GI) tract >2 weeks Usually commenced in surgical centre before transfer back to neonatal unit: relapsing or complicated necrotising enterocolitis surgical GI disorders (e.g. gastroschisis, large omphalocoele) short bowel syndrome
by 2 each day
Fat (g/kg/day)
3.5
Day 3 2.5 14
Day 4 2.5 14
Comment
Fat (g/kg/day)
Na (mmol/kg/day) K (mmol/kg/day) Ca (mmol/kg/day) PO4 (mmol/kg/day) Mg (mmol/kg/day) Peditrace (mL/kg/day) Solvito N (mL/kg/day) Vitilipid (infant)
3 (range 24)** 2.5 1 1.5 0.2 0.5 (day 1) 1 (day 2 onwards) 1 4 mL/kg/day
** do not add supplemental sodium on days 12 if <32 weeks before naturesis has occurred (measure urine Na levels daily) Glucose maximum concentration: Peripheral PN 1012% Central PN up to 2025% (may rise occasionally) Volume Maintenance up to 150 mL/kg/day (see Intravenous fluids guideline for fluid requirement) maximal fluid volume varies with individual management Remember to account for volume, electrolyte and glucose content of other infusions (e.g. UAC/UVC fluid, inotropes, drugs) Calories Healthy preterm requires 50 kcal/kg/day for basal energy expenditure (not growth) and 11.5 g protein to preserve endogenous protein stores; more is required for growth, particularly if unwell 60 kcal/kg/day will meet energy requirements during sepsis 90 kcal/kg/day and 2.73.5 g protein will support growth and positive nitrogen balance 120 kcal/kg/day may be required for a rapidly growing preterm infant
NUTRITIONAL SOURCES
Glucose (provides 3.4 kcal/g) Initiated at endogenous hepatic glucose production and utilisation rate of 46 mg/kg/min; [810 mg/kg/min in extremely low birth weight (ELBW) infants]. Osmolality of glucose limits its concentration Protein (provides 4 kcal/g) At least 1 g/kg/d in preterm and 2 g/kg/d in ELBW decrease catabolism 33.5 grams protein/kg/d and adequate non-protein energy meets requirements for anabolism
Fat (provides 2.0 kcal/mL) Fat of 33.5 g/kg/day is usually sufficient >4 g/kg/day only in very preterm with normal triglycerides not septic, not on phototherapy fat should ideally provide 3540% of non-protein nitrogen calories To minimise essential fatty acid deficiency, hyperlipidaemia, bilirubin displacement, and respiratory compromise, lipid infusion rates <0.15 g/kg/h are recommended to run throughout 24 hr in neonates, maximal removal capacity of plasma lipids is 0.3 g/kg/hr delivery of 3 g/kg/d of a 20% lipid emulsion equates to an infusion rate of 0.125 g/kg/hr Energy Carbohydrate (glucose) and fat (lipid emulsions) provide necessary energy to meet the demands and, when provided in adequate amounts, spare protein (amino acids) to support cell maturation, remodelling, growth, activity of enzymes and transport proteins for all body organs PN requirement for growth 90115 kcal/kg/day Electrolytes Sodium, potassium, and chloride dependent on obligatory losses, abnormal losses and amounts necessary for growth, and can be adjusted daily If neonate <32 weeks, do not add sodium until they have started their naturesis, monitored by daily urine Na+ Infants given electrolytes solely as chloride salts can develop hyperchloraemic metabolic acidosis (consider adding acetate to PN, where available) Vitamins Vitamin and mineral added according to best estimates based on limited data
SPECIAL NEEDS
Hyperglycaemia If hyperglycaemia severe or persistent, start insulin infusion: see Administration of actrapid insulin (soluble insulin) in Hyperglycaemia guideline Osteopenia If infant at risk of, or has established, osteopenia, give higher than usual intakes of calcium and phosphorus consult dietitian and/or TPN pharmacist regarding prescribing information permissible concentrations depend on amino acid and glucose concentrations in TPN solution Metabolic acidosis For management of metabolic acidosis, add acetate as Na or K salt if available: ask local TPN pharmacist choice of salt(s) will depend on serum electrolytes Increase calories for: infection chronic lung disease healing growth babies who have experienced intrauterine growth retardation (IUGR) Decrease calories for: sedation mechanical ventilation
MONITORING
Daily Fluid input Fluid output Energy intake Protein Non-protein nitrogen Calories Urine glucose Blood glucose (if urine glucose positive) Urine electrolytes Weight Length Head circumference FBC Na K Glucose Urea Creatinine Albumin Bone Bilirubin ** Blood gas (arterial or venous) Serum TG** Magnesium Zinc**
Weekly
* Initially daily and decrease frequency once stable unless indicated for other birth weight or gestationspecific guidance see Intravenous fluid therapy guideline ** In prolonged TPN more >2 weeks
COMPLICATIONS
Catheter related: (see Long line insertion guideline) Peripheral catheters: extravasations and skin sloughs Septicaemia Electrolyte abnormalities Electrolyte and acid-base disturbances Metabolic Hyper/hypoglycaemia, osmotic diuresis Metabolic bone disease: mineral abnormalities (Ca/PO4/Mg) Hyperlipidaemia and hypercholesterolaemia Conjugated hyperbilirubinaemia PN-associated cholestatic hepatitis Can occur with prolonged TPN (>1014 days) probably due to combination of TPN toxicity and reduced oral feeding often transient usually manifests as rising serum bilirubin (with increased direct component) and mildly elevated transaminases leads to deficiencies of fatty acids and trace minerals even small enteral feeds will limit or prevent this problem add trace minerals to TPN consider other causes direct hyperbilirubinaemia (TPN-induced cholestasis is diagnosis of exclusion) if stools alcoholic (putty grey) or very pale, refer urgently to liver unit
WEANING PN
When advancing enteral feedings, reduce rate of PN administration to achieve desired total fluid volume Assess nutrient intake from both PN and enteral feed in relation to overall nutrition goals
POSTNATAL MONITORING
Newborns at risk Those with mothers with known blood group antibodies including: D (Rhesus), c, C, s, E, Duffy Kell: causes bone marrow suppression in addition to haemolysis Management of newborns at risk of haemolysis Antenatally: prepare a plan based on antibody titres, middle cerebral artery Dopplers and evidence of hydrops in severely affected cases, order blood in advance for exchange transfusion Send cord blood urgently for Hb, blood group, direct Coombs test (DCT) and bilirubin in all newborns who have had an in-utero blood transfusion (IUT), send cord blood also for a Kleihauer test chase results If pale with abnormal cardiorespiratory signs (e.g. tachycardia), admit to Neonatal Unit (NNU) If newborn has positive DCT or had an IUT (regardless of DCT and blood group): discuss with senior doctor if cord bloods not available, check babys blood immediately for bilirubin, Hb and DCT monitor serum bilirubin, usually at 6-hrly intervals until the level is both stable/falling and 2 consecutive values are >50 umol/L below the treatment threshold plot bilirubin values on the NICE gestational age-specific charts: http://www.nice.org.uk/guidance/CG98 keep parents informed discuss progress regularly with middle-grade doctor or consultant Decide whether newborn needs phototherapy or exchange transfusion as determined by the gestational age-specific charts If newborn has negative DCT and had no IUT, no further action required; newborn is not affected Management of newborns with haemolysis diagnosed or suspected postnatally Newborns with: blood group incompatibility with a positive DCT, manage as above red cell enzyme defect, inform on-call consultant
PHOTOTHERAPY
Indications/treatment thresholds Prophylactic phototherapy (e.g. from birth) is not beneficial Term babies 38+ weeks gestation Babies <38 weeks gestation
Management Plot bilirubin values on the appropriate gestation NICE treatment chart Administer phototherapy See Jaundice guideline Check bilirubin 6 hr after onset of phototherapy Monitor serum bilirubin at least 6-hrly until the level is both stable/falling and 2 consecutive values of >50 umol/L below the treatment threshold Encourage mother to continue feeding using her chosen method, but emphasise the need for adequate hydration See Jaundice guideline Continue phototherapy until value falls consistently (e.g 2 consecutive bilirubin values) below phototherapy threshold on chart Once phototherapy has been discontinued, check serum bilirubin within 12 hr Communicate plan of management clearly to parents and document discuss any communication difficulties with on-call consultant
BEFORE DISCHARGE
Check discharge Hb and bilirubin
Indication for top-up transfusion for late anaemia At request of neonatal consultant Symptomatic anaemia Hb <75 g/L Ongoing neuro-developmental follow-up and hearing test Arrange for any baby: with definite red cell anomalies who has undergone an exchange transfusion who has had an IUT who required IVIG with serum bilirubin at or above exchange transfusion threshold
Coagulopathy 201315
COAGULOPATHY
Haemostasis is immature during the neonatal period and does not attain full function until 6 months of age prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT) are associated with intraventricular haemorrhage (IVH) in unstable (e.g. hypotensive or hypoxic) or bruised extremely preterm babies 75% of cases of IVH occur within first 24 hr of life and 90% within first 7 days prophylactic fresh frozen plasma (FFP) does not prevent IVH in preterm baby without evidence of coagulopathy
INVESTIGATIONS
Check clotting in: Any bruised or bleeding neonate (e.g. IVH, pulmonary haemorrhage, gastrointestinal bleeding, suspected haemorrhagic disease of newborn etc) Preterm <30 weeks gestation (due to IVH risk) Moderate-to-severe encephalopathy (e.g. babies who are being cooled) Septicaemia Necrotising enterocolitis (NEC) Sick or unstable baby (e.g. ventilated, inotropic support etc) Metabolic disease: urea cycle disorder, galactosaemia, tyrosinaemia, organic acidaemia Liver dysfunction or conjugated jaundice Neonates undergoing surgery or tissue biopsy who have had previous bleeding problems Family history of inherited bleeding disorder (after discussion with consultant haematologist) Thrombocytopenia see Thrombocytopenia guideline Sampling Ensure sample from a free-flowing vein (peripheral or umbilical) or from an arterial line before heparinising Use appropriate coagulation tubes as per local policy Fill exactly to black mark on tube (usually 1.3 mL) If sample clots (this does not confirm normal coagulation), take another If sampling from arterial line with heparin infusion, take larger volume from dead-space (e.g. 2.5 mL), see Arterial line sampling guideline Request PT APTT Fibrinogen If features of DIC (e.g. bruising, bleeding, sepsis), request: fibrin degradation products and D-dimer (if available) If concerned/unsure about initial results, seek senior advice Acceptable reference values Clotting parameter PT APTT Fibrinogen Gestation Term Preterm (<37 weeks) Term Preterm (<37 weeks) Term Preterm (<37 weeks) Ratio INR 11.6 12 11.6 12 Value (laboratory control PT 1114 sec and APTT 3033 sec) 1016 sec 1122 sec 3155 sec 2870 sec 1.74.0 1.53.7
IMMEDIATE TREATMENT
If INR alone is prolonged, check whether clotting samples were performed before first dose of vitamin K. If so. repeat clotting screen If prolonged INR (see thresholds below) and normal APTT in stable term baby (e.g. clotting screen performed as part of conjugated jaundice screen), give repeat dose of
Coagulopathy 201315 vitamin K 1 mg IV. If repeat INR not improving after 6 hr, discuss with senior/haematologist to explore other causes and consider FFP or regular vitamin K In preterm baby <30 weeks (with risk of IVH) or unwell with prolonged INR, repeat vitamin K 1 mg IV with FFP If APTT beyond upper limit of reference range, give FFP (see below) In case of persistently prolonged INR or liver disorder/conjugated jaundice, give regular doses of vitamin K In persistently prolonged APTT, give further doses of FFP (or cryoprecipitate see below)
Use of FFP and cryoprecipitate Do not use FFP or cryoprecipitate purely for volume replacement or polycythaemia without coagulopathy Treatment thresholds for use of FFP If PT or APTT below treatment thresholds: FFP 1020 mL/kg over 3060 min Clotting parameter Unstable*, significant**, bleeding or invasive procedure*** Ratio (INR) 1.5 or Value 15 sec Ratio (INR) 1.8 or Value 20 sec Ratio (INR) 1.5 or Value 45 sec Ratio (INR) 1.8 or Value 60 sec
Gestation Term
Stable baby Ratio (INR) >1.6 or Value >16 sec Ratio (INR) >2 or Value >22 sec Ratio (INR) >1.6 or Value >55 sec Ratio (INR) >2 or Value >70 sec
*Unstable (e.g. DIC, significant sepsis, NEC, ventilated, hypotensive etc) **Significant bleeding (e.g. significant bruising, IVH, gastrointestinaI bleeding, pulmonary haemorrhage etc) ***Invasive procedures (e.g. lumbar puncture, umbilical lines, long lines, chest drain, exchange transfusion etc) In inherited clotting factor deficiencies, use FFP only when pathogen inactivated factor unavailable. Discuss with consultant haematologist before giving FFP If APTT ratio still >1.8 after giving FFP (especially if fibrinogen <1.2), consider cryoprecipitate (510 mL/kg over 3060 min) after discussion with on-call consultant and haematologist
MONITORING
Repeat coagulation profile 24 hr after FFP/cryoprecipitate or every 1224 hr if abnormality or indication persists for >24 hr in the absence of any precipitating factors, seek advice from paediatric haematologist about factor assays and 50:50 mixture correction test Look for and treat causes of abnormal coagulation: sepsis shock haemorrhage severe hypothermia hypoxia If abnormal coagulation persists for >24 hr in the absence of any precipitating factors, seek advice from paediatric haematologist about factor assays and 50:50 mixture correction test
Evidence Level: V Is there any causative relationship between deranged coagulation and intraventricular haemorrhage (IVH) in the newborn? If so, does treating abnormal coagulation prevent IVH in preterm infants? In a study of 106 preterm infants of 34 weeks gestation (Beverley, 1984), 25 (23.5%) developed intraventricular haemorrhage in the first 48 hours of life. Although coagulation parameters appeared normal at birth, differences had appeared by 48 hours, with the IVH group showing a prolonged activated partial thromboplastin time and reduced factor II, VII, and X activity. The authors postulated that, although the aetiology of IVH is multifactorial, early correction of coagulation anomalies may prevent progression to more severe grades of haemorrhage. Survivors of grade III or IV IVH have a 40% risk of permanent brain damage (Krishnamoorthy, 1979). Another study, in 58 VLBW infants (Setzer, 1982), found that 32 (55%) developed IVH. The affected infants had lower mean platelet count and platelet aggregation response and prolonged mean bleeding time compared to those unaffected. A study of 54 preterm infants <35 weeks gestation (Amato, 1988) found peri-intraventricular haemorrhage (PIVH) in 21 (38%). The affected infants showed lower fibrinogen levels than unaffected infants and significant correlation between platelet count and degree of PIVH. One prospective study in 49 infants (Van de Bor, 1986) found no difference in coagulopathy between 20 who developed PIVH and 29 who did not, apart from lower levels of factor V in the affected group. A prospective, randomised trial in 100 infants <32 weeks gestation and <1500 gm in weight (Morales, 1988) compared those whose mothers had received 10 mg vitamin K1 i.m. 5 days prior to delivery with a similar number who had not received the vitamin. Infants in the intervention group showed significant reduction in the prothrombin time (12.7 vs 15.2 seconds) and partial thromboplastin time (42.6 vs 58.9 seconds). These infants also experienced a lower incidence of total (16% vs 36%) and severe (0% vs 11%) grades of IVH. A single maternal dose of 10 mg vitamin K1 i.m. 4 hours before delivery may be sufficient to provide significant protection against IVH (Pomerance, 1987).
One randomised prospective study in 98 infants (Kazzi, 1989) found no improvement in coagulopathy or incidence of IVH when mothers received 10 mg vitamin K1. See also following question on FFP.
Amato M, Fauchere JC, Hermann U. Coagulation abnormalities in low birth weight infants with periintraventricular hemorrhage. Neuropediatrics 1988;19:154-7 Beverley DW, Chance GW, Inwood MJ, et al. Intraventricular haemorrhage and haemostasis defects. Arch Dis Child 1984;59:444-8 Kazzi NJ, Ilagan NB, Liang KC, et al. Maternal administration of vitamin K does not improve the coagulation profile of preterm infants. Pediatrics 1989;84:1045-50 Krishnamoorthy KS, Shannon DC, DeLong GR, et al. Neurologic sequelae in the survivors of neonatal intraventricular haemorrhage. Pediatrics 197 9;64:233-7 Morales WJ, Angel JL, OBrien WF, et al. The use of antenatal vitamin K in the prevention of early neonatal intraventricular hemorrhage. Am J Obstet Gynecol 1988;159:774-9 Pomerance JJ, Teal JG, Gogolok JF, et al. Maternally administered antenatal vitamin K1: effect on neonatal prothrombin activity, partial thromboplastin time, and intraventricular hemorrhage. Obstet Gynecol 1987;70:235-41 Setzer ES, Webb IB, Wassenaar JW, et al. Platelet dysfunction and coagulopathy in intraventricular hemorrhage in the premature infant. J Pediatr 1982;100:599-605 Van de Bor M, Briet E, Van Bel F, et al. Hemostasis and periventricular-intraventricular hemorrhage of the newborn. Am J Dis Child 1986;140:1131-4
Evidence Level: III What are the indications for giving fresh frozen plasma (FFP)? The main use of FFP in the neonate is to replace single coagulation deficiencies (factors V, VII, X, XI and XIII), where a specific or combined factor concentrate is unavailable (Contreras, 1992). This is essentially a second best solution, as very large volumes are often needed in order to reach sufficiently high plasma levels (Muntean, 2002). A prospective study in 33 neonates (Hyytiainen, 2003) found that FFP had an acute thrombinreducing effect in those infants with the highest pretransfusional thrombin formation. The authors stress that FFP has poorly-defined effects on coagulation and unproven clinical efficacy. One randomised study in 73 preterm infants (Beverley, 1985) found that 15 (41%) of control patients sustained intraventricular haemorrhage compared with 5 (14%) of 36 patients given FFP 10ml/kg on admission and at 24 hours of age. An earlier controlled trial of the same regimen in 66 infants (Hambleton, 1973) had found no evidence of a protective effect for FFP on IVH. Similarly negative results were obtained in a later and larger trial in 776 infants (Anon, 1996). Osborn and Evans (2009) performed a meta-analysis of randomized trials of early volume expansion in neonates using different volume expanders (including FFP). This meta-analysis concluded that there were no benefits associated with the early administration of FFP to preterm neonates, in terms of improving blood pressure, decreasing rates or severity of IVH, decreasing mortality, or improving neurodevelopmental outcomes.
Anon. Randomised trial of prophylactic early fresh-frozen plasma or gelatin or glucose in preterm babies: outcome at 2 years. Northern Neonatal Nursing Initiative Trial Group. Lancet 1996;348:22932 Beverley DW, Pitts TT, Congdon PJ, et al. Prevention of intraventricular haemorrhage by fresh frozen plasma. Arch Dis Child 1985;60:710-3
Contreras M, Ala FA, Greaves M, et al. Guidelines for the use of fresh frozen plasma. British Committee for Standards in Haematology, Working Party of the Blood Transfusion Task Force. Transfus Med 1992;2:57-63 Hambleton G, Appleyard WJ. Controlled trial of fresh frozen plasma in asphyxiated low birthweight infants. Arch Dis Child 1973;48:31-5 Hyytiainen S, Syrjala M, Fellman V, et al. Fresh frozen plasma reduces thrombin formation in newborn infants. J Thromb Haemost 2003;1:1189-94 Muntean W. Fresh frozen plasma in the pediatric age group and in congenital factor deficiency. Thromb Res 2002;107(Suppl 1):S29-32 Osborn DA, Evans NJ. Early volume expansion for prevention of morbidity and mortality in very preterm infants. Cochrane Database of Systematic Reviews 2004, Issue 2.
Evidence Level: I (for no evidence in favour of FFP for IVH) What is the role of Vitamin K in abnormal coagulation in the newborn? Vitamin K is administered to prevent haemorrhagic disease of the newborn (HDN). A Cochrane review of 13 RCTs (Puckett, 2000) concluded that a single dose (1.0 mg) of intramuscular vitamin K after birth is effective in the prevention of classic HDN. Either intramuscular or oral prophylaxis improved biochemical indices of coagulation status at 1-7 days. Neither route of administration has been tested in RCTs for effect on late HDN (week 2-12), and the oral route has also not been tested for effect on classic HDN (day 1-7). The American Academy of Pediatrics has consistently recommended vitamin K in its guidelines since 1961 (Anon, 2003). There is no evidence that vitamin K deficiency bleeding is commoner in preterm babies (Hey, 2003), or that cancer risk is raised in infants given intramuscular vitamin K (Roman, 2002).
Anon. Controversies concerning vitamin K and the newborn. Policy statement. American Academy of Pediatrics. Committee on Fetus and Newborn. Pediatrics 2003;112:191-2 Hey E. Vitamin K what, why, and when. Arch Dis Child Fetal Neonatal Ed 2003;88:F80-3 Puckett RM, Offringa M. Prophylactic vitamin K for vitamin K deficiency bleeding in neonates. The Cochrane Database of Systematic Reviews 2000, Issue 4. Art. No.: CD002776 Roman E, Fear NT, Ansell P, et al. Vitamin K and childhood cancer: analysis of individual patient data from six case-control studies. Br J Cancer 2002;86:63-9
Evidence Level: I When and how should abnormal coagulation be treated in the newborn (especially the premature)? Expectant management is sufficient for infants that appear well, but the sick baby may have disseminated intravascular coagulation (Buchanan, 1986). Treatment of the triggering event, lowdose heparin, antithrombin concentrate and selected components may all be used, but good evidence for efficacy is lacking (Bick, 2002).
Bick RL. Disseminated intravascular coagulation: a review of etiology, Pathophysiology, diagnosis, and management: guidelines for care. Clin Appl Thromb Hemost 2002;8:1-31 Buchanan GR. Coagulation disorders in the neonate. Pediatr Clin N Am 1986;33:203-220
Evidence Level: V
Polycythaemia 201315
POLYCYTHAEMIA
RECOGNITION AND ASSESSMENT
Definition Peripheral venous haematocrit (Hct) >65% Symptoms rarely occur with peripheral Hct of <70% Hct peaks at 2 hr after birth and then decreases with significant changes occurring by 6 hr Clinical consequences Hyperviscosity Decreased blood flow and impaired tissue perfusion Thrombus formation Complications Cerebral micro-infarction and adverse neuro-developmental outcome Renal vein thrombosis Necrotising enterocolitis (NEC) Causes Intra-uterine increased erythropoiesis Placental insufficiency (SGA) Postmaturity Maternal diabetes Maternal smoking Chromosomal abnormalities: trisomy 21, 18, 13 BeckwithWiedemann syndrome Congenital adrenal hyperplasia Neonatal thryotoxicosis Congenital hypothyroidism Symptoms and signs Commonly plethoric but asymptomatic Cardiorespiratory Respiratory distress Persistent pulmonary hypertension of the newborn (PPHN) Congestive cardiac failure Lethargy, hypotonia within 6 hr Difficult arousal, irritability Jittery Easily startled Seizures Poor feeding Vomiting NEC Hypoglycaemia Hypocalcaemia Jaundice Thrombocytopenia Renal vein thrombosis Renal failure Erythrocyte transfusion Maternal-fetal Twin-to-twin transfusion Delayed cord clamping Unattended delivery
CNS
GIT
Metabolic
Haematological Renal
INVESTIGATIONS
In all unwell babies and at-risk babies who look plethoric (as mentioned above) FBC/Hct If Hct >65%, repeat a free-flowing venous sample or obtain arterial Hct If polycythaemic, check blood glucose and serum calcium
Polycythaemia 201315
IMMEDIATE TREATMENT
Ensure babies at risk have liberal fluid intake one day ahead (e.g. 90 mL/kg/day on day 1) Asymptomatic babies with Hct >70% Repeat venous Hct after 6 hr if still high, discuss with consultant (current evidence does not show any benefit in treating asymptomatic babies) Symptomatic babies with Hct >65% Possible symptoms: fits and excessive jitteriness, with neurological signs and refractory hypoglycaemia Treatment Dilutional exchange transfusion. Discuss with consultant explain to parents need for exchange and possible risks before performing dilutional exchange transfusion. Partial exchange transfusion increases risk of NEC use sodium chloride 0.9% see Exchange transfusion guideline Volume to be exchanged = 20 mL/kg Perform exchange via peripheral arterial and IV lines or via umbilical venous catheter (UVC) Take 510 mL aliquots and complete procedure over 1520 min
SUBSEQUENT MANAGEMENT
Babies who required dilutional exchange transfusion require long-term neurodevelopmental follow-up Otherwise, follow-up will be dependent on background problem
Thrombocytopenia Ready
THROMBOCYTOPENIA
DEFINITION
Platelet count <150 x 109/L mild (platelet count 100150 109/L) and moderate (50100 109/L) thrombocytopenia occur frequently in preterm babies who are ill, and in those born to women with pregnancy-induced hypertension (PIH) severe thrombocytopenia (<50 109/L) is uncommon, particularly in apparently healthy term babies and raises the possibility of neonatal allo-immune thrombocytopenia (NAIT; see below)
CAUSES
Common WELL Placental insufficiency Intrauterine growth retardation (IUGR) Maternal diabetes Immune mediated Allo-immune thrombocytopenia (NAIT) Auto-immune (maternal ITP, SLE) Trisomies (13, 18, 21) ILL Infection Necrotising enterocolitis (NEC) Disseminated intravascular coagulation (DIC) Perinatal asphyxia Congenital infections Thrombosis (renal, aortic) Congenital leukaemia or neuroblastoma Metabolic disorders (propionic and methymalonic acidemia)
Rare
Inherited disorders Thrombocytopenia Absent Radii (TAR) syndrome Congenital amegakaryocytic thrombocytopenia (CAMT) Cavernous haemangioma (KasabachMerritt syndrome)
Severe thrombocytopenia in an otherwise healthy term newborn baby is NAIT until proved otherwise
INVESTIGATIONS
Evaluation of early-onset (<72 hr after birth) thrombocytopenia (see Flowchart) in preterm babies with early-onset mild-to-moderate thrombocytopenia in whom there is good evidence of placental insufficiency, further investigations are not warranted unless platelet count does not recover within 1014 days in preterm babies without placental insufficiency, investigate first for sepsis in term babies, investigate for sepsis and NAIT If significant thrombocytopenia, clotting screen Look for presence of active bleeding or visible petechiae If other features suggestive, congenital infection screen Obstetric history, particularly maternal platelet count, drugs. pre-eclampsia. Family history of bleeding disorders Careful examination, include other associated features (e.g. trisomies and inherited syndromes)
Evaluation of late onset thrombocytopenia Thrombocytopenia presenting in baby after first 3 days of life, presume underlying sepsis or NEC until proved otherwise these babies are at significant risk of haemorrhage, though the benefit of platelet transfusion is not clear-cut
Platelet count 9 <50 x 10 /L, or active bleeding with platelet count 50100 10 /L
9
YES
NO
Check coagulation
Infection workup/antibiotics Check coagulation Baby clinically well Baby unwell Exclude sepsis and/or DIC
Monitor platelet count closely Sepsis likely and platelets normalised after treatment Platelet count at 10 days No further action Exclude congenital infections (CMV, toxo, HSV, HIV) Sepsis unlikely and platelets still low Exclude immune thrombocytopenia (NAIT, ITP, SLE)
>150 10 /L
9
<150 10 /L
9
NO Diagnosis made?
No further evaluation
MANAGEMENT
General Avoid Heel prick and IM injections, use venepuncture and IV injections Invasive procedure (central line, lumbar puncture, chest drain etc). If any of above are unavoidable: discuss with on-call consultant platelet transfusion before undertaking unavoidable invasive procedures if platelet count <50 x 109/L give particular attention to haemostasis Platelet transfusion Only available immediate and specific therapy for thrombocytopenia but carries a risk of transfusion-related infections and transfusion reactions and only after discussion with senior Indications for platelet transfusion (term and preterm babies) Main objective is to prevent the consequences of severe thrombocytopenia, significant risk of acute intracerebral haemorrhage and neuromorbidity
Thrombocytopenia Ready Platelet count <30 x 109/L In otherwise well baby, including NAIT, if no evidence of bleeding and no family history of intracranial haemorrhage Platelet count <50 x 109/L In baby with: clinical instability concurrent coagulopathy birth weight <1000 g and age <1 week previous major bleeding e.g. intraventricular haemorrhage (IVH) current minor bleeding (e.g. petechiae, venepuncture oozing) planned surgery, exchange transfusion or invasive procedure (central line insertion, lumbar puncture, chest drain etc) platelet count falling and likely to fall below 30 NAIT if previously affected sibling with intracranial bleed PDA treated with indomethacin or Ibuprofen Platelet count <100 x 109/L If major bleeding and platelet count falling rapidly, platelet transfusion : Type of platelets NAIT: HPA compatible platelets wherever possible All others: blood group-compatible cytomegalovirus (CMV) negative Irradiation of platelets is not routinely required but consider for babies with definite or suspected immunodeficiency or those who have undergone intrauterine transfusions Volume of platelets 10 mL/kg (should raise platelet count by >50 109/L). Babies with suspected NAIT will require higher dose 20 mL/kg
ADMINISTRATION OF PLATELETS
Never administer platelets through an arterial line or UAC Use platelets as soon as they arrive on ward (ensure IV access before requesting platelets from blood bank) Keep platelets at room temperature To minimise loss, draw contents of pack into 50 mL syringe through a special platelet or fresh blood transfusion set with a 170200 micrometre filter and infuse, using a narrow bore extension set linked to IV line, primed with sodium chloride 0.9% Transfuse platelets over 3060 min, mixing syringe from time to time to avoid platelets settling down There is no need for routine use of diuretic after platelet transfusion Check platelet count 1 hour after transfusion
Recognition For HPA-1a antigen-negative women, complete a neonatal alert form Petechiae, purpura, excessive bleeding and severe thrombocytopenia in an otherwise healthy term newborn baby indicate NAIT until proved otherwise NAIT can also present with: fetal intracranial haemorrhage or unexplained hydrocephalus postnatal intracranial haemorrhage in term baby If NAIT suspected, involve consultant neonatologist immediately
Thrombocytopenia Ready Assessment Check babys platelet count daily until >100 109/L Check mothers platelet count (may already be in maternal healthcare record) Obtain blood from mother, baby and father for platelet typing and antibodies. Liaise with haematology department about appropriate samples Arrange cranial ultrasound scan (see Cranial ultrasound scans guideline) Treatment In 30% of cases, maternal antibody may not be found and can be detected later treat babies with suspected NAIT empirically with antigen-negative platelets Transfuse baby with suspected NAIT with accredited HPA-1 antigen-negative platelets if: bleeding or platelet count <30 109/L National Blood Transfusion Service has a pool of suitable donors, and platelets are available at short notice from blood bank if accredited HPA-1a negative platelets not available, administer random donor platelets Inform blood bank and consultant haematologist as soon as NAIT suspected. Do not delay transfusion for investigations If thrombocytopenia severe (<50 109/L), or haemorrhage persists despite transfusion of antigen-negative platelets, administer intravenous human immunoglobulin (IVIG) 400 mg/kg/day for 3 consecutive days (may require additional doses 24 weeks later) Aim to keep platelet count >30 109/L for first week of life, or as long as active bleeding continues Report newly diagnosed babies with NAIT to fetal medicine consultant for counselling for future pregnancies
Transfusion 201315
Baby Ventilated baby or Significant PDA Baby in supplemental oxygen/CPAP or Symptoms of anaemia e.g. significant unexplained apnoea (e.g. >9 episodes in 12 hr, or 2 episodes in 24 hr needing face mask ventilation) or Baby aged <24 hr Poor weight gain (without any other cause) or Asymptomatic baby with poor reticulocyte response (reticulocyte <4% or absolute reticulocyte count <100 x 109/L) Asymptomatic baby with good reticulocyte count (reticulocyte 4% or absolute reticulocyte count 100 x 109/L)
100
80 70
PRE-TRANSFUSION
Communication If clinical condition permits before transfusion, inform parents that baby will receive blood transfusion document discussion If parents refuse transfusion (e.g. Jehovahs witness) follow local policy Crossmatch For top-up transfusions in well baby, arrange with blood bank during normal working hours Crossmatch against maternal serum (or neonatal serum if maternal serum not available) For first transfusion, send samples of babys and mother's blood Direct Coombs testing The laboratory will perform Direct Coombs test (DCT) on maternal serum for any atypical antibodies If maternal DCT negative, blood issued will be crossmatched once against maternal serum. No further blood samples are necessary for repeat top-up transfusions If maternal DCT positive, crossmatching of donor red blood cells against maternal serum is required every time Multiple transfusions In babies <29 weeks who may need multiple transfusions, use paediatric satellite packs (Paedipacks) from one donor (if available) to reduce multiple donor exposure When to use irradiated blood It is preferred practice for all blood given to babies to be irradiated. However, irradiated blood MUST always be given for those: who have received intra-uterine transfusion
Transfusion 201315
with suspected or proven immunodeficiency receiving blood from a first- or second-degree relative, or an HLA-selected donor
When to use CMV-free blood As CMV seronegativity cannot be guaranteed in untested blood, use only CMVseronegative blood for neonatal transfusions Blood products in use in the UK are leuco-depleted to <5 x 106 leucocytes/unit at point of manufacture Special considerations Iron supplements Premature babies receiving breast milk or with Hb <100 g/L, commence oral iron supplementation (sodium feredetate Sytron 1 mL once daily) at 6 weeks of age See Nutrition guideline Withholding feeds during transfusion Some units withhold enteral feeds during the 34 hr duration of transfusion follow local practice Babies with necrotising enterocolitis (NEC) Transfuse using red cells in sodium chloride 0.9%, adenine, glucose and mannitol (SAGM), preferably, as it is relatively plasma-free. This may not be available in all units follow local practice Investigate any unexpected haemolysis associated with transfusion in an baby with NEC for T-cell activation in consultation with local haematology department and with close involvement of consultant neonatologist Exchange transfusion See Exchange transfusion guideline
TRANSFUSION
Volume of transfusion Give 1520 mL/kg of red cell transfusion irrespective of pre-transfusion Hb A paediatric pack contains approximately 50 mL blood. Use one pack if possible Rate of administration Administer blood at 15 mL/kg over 3 hr or 20 mL/kg over 4 hr (5 mL/kg/hr) Increase rate in presence of active haemorrhage with shock Via peripheral venous or umbilical venous line (not via long line or arterial line) Use of furosemide Routine use NOT recommended Consider soon after blood transfusion for babies: with chronic lung disease with haemodynamically significant PDA in heart failure with oedema or fluid overload
Transfusion 201315
Ensure positive identification of baby using accessible identification Appropriate labelling of syringes to ensure compliance with current best practice Unless clinically urgent, avoid transfusion out-of-hours To reduce need for blood transfusion, minimise blood sampling in babies (microtechniques, non-invasive monitoring) and avoid unnecessary testing Ensure donor exposure is minimised by using satellite packs from same donor
Hazards of transfusion Most important are: infections bacterial or viral hypocalcaemia volume overload citrate toxicity rebound hypoglycaemia (following high glucose levels in additive solutions) thrombocytopenia after exchange transfusion
Evidence Level: V Last amended August 2013 Last reviewed August 2013
Vitamin K 201315
VITAMIN K
INDICATIONS
Prophylaxis Babies are relatively deficient in vitamin K (phytomenadione) and those who do not receive supplements are at risk of bleeding (vitamin K deficient bleeding, formerly known as haemorrhagic disease of the newborn) All babies should be given vitamin K with parental consent Therapy After blood has been taken for clotting studies, vitamin K can also be used to treat any baby with active bleeding that might have resulted from vitamin K deficiency a prolonged prothrombin time (INR 3.5) that falls within 1 hr of treatment, with normal platelet count and fibrinogen concentration suggest the diagnosis. However, as INR is a poor indicator of vitamin K deficiency, PIVKAII is a better investigation if available
ADMINISTRATION
Prophylaxis Vitamin K (Konakion MM Paediatric) as a single dose (see Table below for dosage schedule) avoid IV administration for prophylaxis as it does not provide the same sustained protection as IM Give in accordance with manufacturers instructions in order to ensure clinical effectiveness If parents decline IM route, offer oral vitamin K as second line option (safety fears of parenteral vitamin K appear to be unfounded) If parents refuse prophylaxis, ask senior neonatologist to see and record discussion in notes Babies who are exclusively breastfed will require additional oral doses after discharge from hospital (2 mg oral at birth, then 2 more doses of 2 mg within next 68 weeks (Neonatal Formulary, 6th edition) IM use Do not dilute or mix with other parenteral injections Oral use Break open ampoule and withdraw 0.2 mL (2 mg) into the oral dispenser provided. Drop contents directly into baby's mouth by pressing plunger Prophylaxis dosage Konakion MM Paediatric Healthy babies of >36 weeks First line 1 mg IM at birth or soon after Second line 2 mg oral at birth, then 2 mg oral at 47 days, then 2 mg oral at 1 month if exclusively breastfed Term babies at special risk Instrumental delivery, caesarean section Maternal treatment with enzyme-inducing anticonvulsants (carbamazepine, phenobarbital, phenytoin), rifampicin or warfarin Requiring admission to NICU Babies with cholestatic disease where oral 1 mg IM at birth or soon after Do not offer oral vitamin K
Vitamin K 201315
absorption likely to be impaired Preterm babies <36 weeks but 2500 g All babies <2500 g
Babies who have or may have Factor VIII or Factor IX deficiency or other coagulation deficiency
1 mg IM at birth or soon after 0.4 mg/kg (0.04 mL/kg) IM shortly after birth (maximum dose 1 mg) Do not exceed this parenteral dose The frequency of further doses should depend on coagulation status Give orally unless results of Factor assays normal
For babies with birth weight 2500 g Administer Konakion MM Paediatric 1 mg (0.1 mL) intramuscularly this is approximately half of the ampoule volume and should be drawn up using the 0.5 mL Omnican-F syringe with 0.01 mL graduations supplied with the ampoule For babies with birth weight <2500 g Administer 400 microgram/kg (0.04 mL) with a maximum of 1 mg (0.1 mL) of Konakion MM Paediatric intramuscularly round up the dose to nearest hundredth [e.g. 300 microgram (0.03 mL), 500 microgram (0.05 mL) etc] draw up the dose using a 0.5 mL Omnican-F syringe with 0.01 mL graduations supplied with the ampoule Therapy dosage If not already given IM, give vitamin K 100 microgram/kg IV up to 1 mg maximum dose Further doses as required, depending on clinical picture and coagulation status may need to be accompanied by a more immediately effective treatment such as transfusion of fresh frozen plasma IV administration If necessary, dilute with glucose 5% only
BCG 201315
BCG IMMUNISATION
See also BCG and unknown maternal HIV status guideline
INDICATIONS
Born or living in an area where the notification rate of TB is >40/100,000 (including UK) A parent or grandparent born in a country where the notification rate of TB is >40/100,000 Family history of TB in previous 5 yr Local policy to give BCG to all neonates in that area
Djibouti Dominican Republic Ecuador El Salvador Equatorial Guinea Eritrea Ethiopia Gabon Gambia Georgia Ghana Greenland Guatemala Guinea Guinea-Bissau Guyana Haiti Honduras Hong Kong India Indonesia Iraq Kazakhstan Kenya Kiribati Korea DPR Korea (Rep. of) Kyrgyzstan Lao PDR Latvia Lesotho Liberia Lithuania Macao Madagascar Malawi Malaysia Maldives Mali Marshall Islands Mauritania Micronesia Moldova Mongolia Morocco Mozambique Myanmar Namibia Nepal Niger Nigeria Pakistan Palau Panama Papua New Guinea Paraguay Peru Philippines Romania Russia Rwanda Saudi Arabia Senegal Sierra Leone Solomon Islands Somalia South Africa Sri Lanka Sudan South Sudan Suriname Swaziland Tajikistan Tanzania Thailand Timor-Leste Togo Turkmenistan Tuvalu Uganda Ukraine Uzbekistan Vanuatu Venezuela Vietnam Yemen Zambia Zimbabwe
Tuberculin testing not necessary <6 yr old unless baby has been in recent contact with tuberculosis or has resided in high-incidence country for >3 months
CONTRAINDICATIONS
Temperature >38oC Severe eczema (give at suitable lesion-free site) Neonate in household where an active TB case suspected or confirmed Immunodeficient or on high-dose corticosteroids defer BCG until 3 months after stopping corticosteroids if given prednisolone 1 mg/kg/day for >3 weeks, 2 mg/kg/day for 1 week, (or equivalent doses of another corticosteroid, e.g. dexamethasone 0.2 mg = prednisolone 1 mg) HIV positive, living in UK if mother HIV positive and exclusively formula feeding, give vaccine only after baby has had negative test for HIV after 3 months of age if high risk of TB exposure and maternal HIV viral load <50 copies/mL after 36 weeks gestation, BCG can be given at birth
BCG 201315
encourage maternal HIV testing but do not withhold BCG if mother declines testing unless mother from sub-Saharan Africa in which case refer to HIV team for counselling about testing
SPECIAL CASES
No need to delay routine vaccinations BCG can be given simultaneously with other vaccines [including palivizumab (Synagis)] but not in same arm no further immunisation should be given in the arm used for BCG immunisation for at least 3 months due to risk of regional lymphadenitis if not given at same time, leave 4 weeks before giving other live vaccines
EQUIPMENT
Alcohol hand gel Injection tray 1 mL syringe Brown (26 FG 0.45 10 mm) or orange needle (3/8 inch 25 FG 0.5 10 mm) Green needle 21 FG 1 inch Cotton wool balls Foil dish for cotton wool balls Non-woven gauze Sharps container Bags for clinical waste BCG vaccine BCG vials are kept in fridge consist of 2 vials make up brown vial with entire contents of clear vial invert vial 12 times to mix, do not shake available for use for 4 hr after reconstitution dose: 0.05 mL (note: vial contains 20 doses) Document that BCG has been given, site, dose and batch number
PROCEDURE
Consent Midwife to record at booking if risk factor present Postnatal check for risk factor Ensure baby within inclusion group Give mother information on vaccine Give appropriate language leaflet BCG and your baby; protecting babies against TB, available from http://nidirect.gov.uk/bcg_leaflet-2.pdf order line: 0300 123 1002 or email: mailto:dh@prolog.uk.com DH guidelines state written consent is not required but follow local practice Injection Only staff trained to give intradermal injections to give BCG
BCG 201315
Hold arm at 450 to body At insertion of deltoid muscle near middle of left upper arm If skin is clean, no further cleaning is necessary Stretch skin between thumb and forefinger Introduce needle bevel upwards about 3 mm into superficial layers of dermis almost parallel to skin If considerable resistance not felt, remove needle and reinsert before giving more vaccine
DOCUMENTATION
Complete Unscheduled vaccine form or letter with batch number, vaccine name and site of immunisation Send to local TB service/Public Health Department Keep a local record Enter in Red book on relevant page
SEQUELAE
Scar within 26 wk a small papule will appear sometimes, this ulcerates and can ooze site need not be protected from water do not cover with an impervious dressing can take several months to heal occasionally persists as keloid (particularly if given superior to insertion of deltoid) Adenitis: a minor degree of adenitis can occur in the weeks following BCG local abscess no treatment indicated Rare sequelae: chronic suppurative lymphadenopathy disseminated disease, if immunocompromised osteitis, refer to infectious diseases specialist
Babies born to mothers with tuberculosis Discuss with local paediatric TB team Send placental specimen for TB culture if TB isolated, give baby TB treatment for 6 months If mother sputum AFB positive and not completed 2 weeks treatment by birth, give baby isoniazid 5 mg/kg daily Tuberculin test after 3 months if negative, perform interferon gamma test: if negative, stop isoniazid and give BCG if either test positive, assess baby for active TB. If assessment negative, continue isoniazid for 6 months Babies can breastfeed
BCG-HIV 201315
Negative
Unknown
Positive
Explore reasons for absence of maternal HIV screening or maternal refusal Obtain consent for postnatal maternal testing
Follow local HIV protocol for prophylaxis, and follow up of HIV exposure in baby Do not give neonatal BCG before discharge
Positive
Negative
Inform consultant Follow local HIV protocol Do not give neonatal BCG before discharge
Identify maternal high risk factors for HIV (i.e. originates from endemic area >1% incidence; partner with HIV or immunodeficiency; suspected HIV/AIDS in mother) If mother not considered high risk for HIV infection, record in notes; give BCG If mother is considered high risk for HIV, baby* requires testing for HIV antibodies, and, if positive, offer HIV prophylaxis as per protocol. BCG withheld until 3 months as per protocol for HIV When seeking consent for HIV testing in baby where mother has refused testing on herself and her baby, discuss with consultant/legal advisor
BCG-HIV 201315
* HIV antibody test carried out on baby in first instance to assess whether there has been exposure to HIV (i.e. mother infected). If mother HIV positive, baby will have transplacental acquisition of mothers antibodies regardless of babys infection status. Carrying out HIV antibody test on baby at birth where mother had refused consent for testing herself will provide useful information on babys exposure status, and whether mother is infected A positive HIV antibody test at birth does not provide an indication of babys infection status. This requires regular screening for HIV DNA PCR and 18 months HIV antibody testing
Note: It is possible for mother to be HIV antibody negative and baby to be HIV antibody negative when tested, if mother is in window period of HIV infection and blood is taken before she mounts an antibody response, which can be transplacentally passed to baby. If concerned that mother poses a high risk, repeat test on mother 6 weeks later, but be aware this does not help the baby in terms of prophylaxis with ARV Prevalence (%) of HIV in adults by region in 2008 Sub-Saharan Africa 5.2 Middle East and North Africa 0.2 South and South East Asia 0.3 East Asia <0.1 Oceania 0.3 Latin America 0.6 Caribbean 1.0 Eastern Europe and Central Asia 0.7 Western and Central Europe 0.3 North America 0.6 Further information 1. http://www.cdc.gov/hiv/topics/basic 2. http://www.who.int/wer/2004/en/wer7904.pdf 3. WHO/UNAIDS AIDS global epidemic update 2009 at: http://data.unaids.org/pub/Report/2009/JC1700_Epi_Update_2009_en.pdf 4. British HIV association at: www.bhiva.org
CMV 201315
CMV
Transmission can occur following primary or recurrent maternal infection Maternal tests CMV IgM A negative result makes infection unlikely A positive result is suggestive of recent infection only if there is a rise during pregnancy; compare IgG avidity (titre) from two specimens >10 days apart Antenatal ultrasound Features include: IUGR Hydrocephalus (ventricular dilatation), intracranial calcification, microcephaly Ascites, hydrops fetalis Pleural or pericardial effusions Oligo- or polyhydramnios Hepatomegaly Abdominal calcification Pseudomeconium ileus Thickened placenta Main clinical signs Small for gestational age Petechiae/purpura Hepatosplenomegaly Jaundice Pneumonia Investigation results CMV IgM positive CMV PCR urine positive Haemolytic anaemia Thrombocytopenia Conjugated hyperbilirubinaemia Raised liver enzymes HIV antibody test If CMV positive, continue with further investigations
FURTHER INVESTIGATIONS
CSF: raised CSF protein Ophthalmology: chorioretinitis Audiology: sensorineural hearing loss Head ultrasound: hydrocephalus, cysts
TREATMENT
Asymptomatic (CMV IgM or PCR positive) or symptoms other than neurological Seek expert advice from paediatric infectious disease specialist regarding offering valganciclovir (currently only as part of trial www.ecci.ac.uk) Neurologically symptomatic Ganciclovir [prepared by pharmacy (cytotoxic)] 6 mg/kg IV over 1 hour 12-hrly for 6 weeks Discuss side effects -vs- benefits with parents:
CMV 201315
advantages: potential reduced risk of deafness and developmental delay disadvantages: during treatment reversible blood dyscrasia; long-term unknown risk to fertility and malignancy Monitor for neutropenia, thrombocytopenia, hepatic and renal function throughout: may need dose reduction discuss with specialist in paediatric infectious diseases Start treatment as soon as possible; if diagnosis delayed, treatment can be started up to 1 month of age
FEEDING
Do not discourage infected women from breastfeeding their own uninfected, term babies (CMV can be transmitted via breastfeeding, but benefits of feeding outweigh risks posed by breastfeeding as a source of transmission) Avoid breastfeeding of premature neonates if mother is positive and baby asymptomatic
FOLLOW-UP
Annual hearing and ophthalmology assessment for both asymptomatic and symptomatic congenitally infected babies
Conjunctivitis 201315
CONJUNCTIVITIS
Conjunctivitis is a potentially blinding condition with associated systemic manifestations
Differential diagnosis Sticky eye with blocked tear duct in which there is no inflammation of conjunctiva Congenital glaucoma in which there is corneal opacity
AETIOLOGY
Bacterial Staphylococcus aureus and epidemidis Haemophilus influenzae Streptococcus pneumoniae Serratia spp, E Coli, Pseudomonas spp Neisseria gonorrhoeae typical onset 05 days of age mild inflammation with serosanguineous discharge to thick, purulent discharge with tense oedema of eyelids Chlamydia trachomatis typical onset 514 days of age: mild-to-severe swelling with purulent discharge (may be blood-stained)
MANAGEMENT
Sticky eye/blocked tear duct 46 hrly eye toilet using sodium chloride 0.9% Conjunctivitis (see signs above) Swab all for: Gram stain and bacterial culture and sensitivities if other suspicions of HSV (e.g. vesicles etc.), viral swab for HSV PCR Chlamydia swab (specific for Chlamydia PCR) Treat with: frequent eye toilet as necessary chloramphenicol 0.5% eye drops fusidic acid 1% eye drops for staphylococcus Presentation within first 24 hr suggests gonococcal infection inform senior paediatrician
SUBSEQUENT MANAGEMENT
In severe non-resolving cases Take throat and eye swabs for viral PCR If herpes suspected, look for other signs of herpetic infection Treat suspected herpes with IV and topical aciclovir for 14 days Refer to ophthalmology Neisseria gonorrhoeae suspected Request urgent Gram stain and culture Assess neonate for septicaemia
Conjunctivitis 201315
Neisseria gonorrhoeae confirmed Give single dose cefotaxime 100 mg/kg IM or, if signs of systemic infection, give at least 7 days IV cefotaxime (or 5 days after clinical response) 50 mg/kg 12-hrly for baby aged <7 days, then 8-hrly Refer to ophthalmology Chlamydia result positive Treat with erythromycin 12.5 mg/kg 6-hrly for 14 days Gonococcal or chlamydia infection detected Refer mother and partner to genito-urinary medicine for immediate treatment
Ganciclovir/valganciclovir is of use in the treatment of congenital CMV infection? Ganciclovir and valganciclovir are two of a number of agents (including cidofovir and foscarnet) having documented in vitro activity against CMV. Ganciclovir has, to date, been more rigorously evaluated for safety and efficacy in infants with congenital CMV infection (Jones, 2003). Valganciclovir has only been the subject of two small randomised studies (Kimberlin 2008, Lombardi 2009). The pharmacokinetic parameters were found to be similar to Ganciclovir. A placebo-controlled, double blind, randomised study comparing 6 weeks versus 6 months with Valganciclovir is currently being carried out by the CASG. (Kadambari 2011) Ganciclovir therapy has been associated with a high rate of complications. An open label, phase II trial in 47 symptomatic infants (Whitley, 1997) administered daily doses of 8 or 12 mg/kg in divided doses, 12 hrly for 6 weeks. Thrombocytopaenia occurred in 37 babies (78%) and neutropaenia in 29 (61%). Although levels of CMV in the urine decreased during the treatment period, they returned to near pretreatment levels when therapy was discontinued. Hearing improvement or stabilization occurred in 5 (16%) of 30 babies at 6 months or later. A randomised controlled trial in 100 symptomatic infants (Kimberlin, 2003) administered 6mg/kg i.v. 12 hrly for 6 weeks vs no treatment. A large number of patients in this study were nonevaluable at follow-up, leaving 42 patients (25 in the treatment group and 17 controls). Twenty one (84%) of the treatment group had improved or maintained normal hearing at 6 months, vs 10 (59%) of the controls. Twenty nine (63%) of 46 patients in the treatment group had grade 3 or 4 neutropaenia during treatment vs 9 (21%) of 43 controls (P < .01). At present, there is no evidence-based guidance available on the selection of suitable patients for treatment with ganciclovir, or for appropriate dosing or whether oral valganciclovir is useful for longer-term treatment (Smets, 2006). These questions were to be addressed by a Cochrane Review (Jones, 2003) but as yet it has not been completed. A controlled Phase III study of symptomatic congenital CMV involving the CNS (Oliver, 2009) randomised 100 neonates to either 6 weeks of intravenous ganciclovir or no treatment. Denver developmental tests were performed at 6 weeks, 6 months, and 12 months. For each age, developmental milestones that > or =90% of normal children would be expected to have achieved were identified. The numbers of milestones not met ("delays") were determined for each subject. The average number of delays per subject was compared for each treatment group. At 6 months, the average number of delays was 4.46 and 7.51, respectively, for ganciclovir recipients and "no treatment" subjects (p=0.02). At 12 months, the average number of delays was 10.06 and 17.14, respectively (p=0.007). In a multivariate regression model, the effect of ganciclovir therapy remained statistically significant at 12 months (p=0.007).
Jones CA. Congenital cytomegalovirus infection. Curr Prob Pediatr Adolesc Health Care 2003;33:65-100 Jones CA, Walker KS, Henderson-Smart DJ. Antiviral therapy for symptomatic congenital cytomegalovirus infection in neonates and infants up to 3 months of age (Protocol). Cochrane Database of Systematic Reviews 2003, Issue 3. Art. No.: CD004340
Kimberlin DW, Lin CY, Sanchez PJ, et al. Effect of ganciclovir therapy on hearing in symptomatic congenital cytomegalovirus disease involving the central nervous system: a randomized, controlled trial. J Pediatr 2003;143:16-25 Kimberlin DW, Acosta, EP, Sanchez, PJ, et al. Pharmacokinetic and Pharmacodynamic Assessment of Oral Valganciclovir in the Treatment of Symptomatic Congenital Cytomegalovirus Disease. G. Lombardi, F. Garofili, P. Vilani et al. Oral valganciclovir treatment in newborns with symptomatic congenital cytomegalovirus infection Eur J Clin Microbiol Dis, 12 (28) (2009), pp. 14651470. Oliver SE, Cloud GA, Sanchez PJ, et al. Neurodevelopmental outcomes following ganciclovir therapy in symptomatic congenital cytomegalovirus infections involving the central nervous system. J Clin Virol 2009;46 Suppl 4:S22-6 Smets K, De Coen K, Dhooge I, et al. Selecting neonates with congenital cytomegalovirus infection for ganciclovir therapy. Eur J Pediatr 2006;165:885-90 Whitley R, Cloud G, Gruber W, et al. Ganciclovir treatment of symptomatic congenital cytomegalovirus infection: results of a Phase II study. National Institutes of Allergy and Infectious Diseases Collaborative Antiviral Study Group. J Infect Dis 1997;175:1080-6
Evidence Level: II Last amended July 2013 Last reviewed July 2013
Group B 201315
Indications for intrapartum antibiotic prophylaxis Previous baby with invasive GBS disease GBS bacteriuria this pregnancy Maternal pyrexia (>38OC during labour) GBS on vaginal swab in this pregnancy (particularly in third trimester)
Edwards MS, Jackson CV, Baker CJ. Increased risk of group B streptococcal disease in twins. JAMA 1981;245:2044-6 Heath PT, Schuchat A. Perinatal group B streptococcal disease. Best Pract Res Clin Obstet Gynaecol 2007;21:411-24 Honig E, Mouton JW, Van der Meijden WI. Can group B streptococci cause symptomatic vaginitis? Infect Dis Obstet Gynecol 1999;7:206-9 Oddie S, Embleton ND. Risk factors for early onset neonatal group B streptococcal sepsis: case-control study. BMJ 2002;325:308-11 Pass MA, Khare S, Dillon HC. Twin pregnancies: incidence of group B streptococcal colonization and disease. J Pediatr 1980;97:635-7 Schuchat A, Zywicki SS, Dinsmoor MJ, et al. Risk factors and opportunities for prevention of early-onset neonatal sepsis: a multicenter case-control study. Pediatrics 2000;105:21-6 Schuchat A, Wenger JD. Epidemiology of group B streptococcal disease: risk factors, prevention strategies, and vaccine development. Epidemiol Rev 1994;16:374-402 Towers CV, Rumney PJ, Minkiewicz SF, et al. Incidence of intrapartum maternal risk factors for identifying neonates at risk for early-onset group B streptococcal sepsis: a prospective study. Am J Obstet Gynecol 1999;181:1197-202 Wood EG, Dillon HC. A prospective study of group B streptococcal bacteriuria in pregnancy. Am J Obstet Gynecol 1981;140:515-20
Evidence Level: III Intrapartum antibiotics (IAP) reduce the incidence of early onset group B streptococcus disease (EOGBSD)? A Cochrane review of 3 trials in 852 women (Ohlsson, 2009) found that The use of IAP did not significantly reduce the incidence of all cause mortality, mortality from GBS infection or from infections caused by bacteria other than GBS. The incidence of early GBS infection was reduced with IAP compared to no treatment (risk ratio 0.17, 95% confidence interval (CI) 0.04 to 0.74, three trials, 488 infants; risk difference -0.04, 95% CI -0.07 to -0.01; number needed to treat to benefit 25, 95% CI 14 to 100, I2 0%). The incidence of LOD or sepsis from organisms other than GBS and puerperal infection was not significantly different between groups. The reviewers also stated that the risk of bias was high in the trials included in the review.
Ohlsson A, Shah VS. Intrapartum antibiotics for known maternal Group B streptococcal colonization. Cochrane Database of Systematic Reviews 2009, Issue 3. Art. No.: CD007467
HEPATITIS B and C
HEPATITIS B
Check mothers hepatitis B status before birth Antenatal Midwife to inform obstetrician, neonatologist, public health team and GP of plan to immunise Hepatitis B immunoglobulin (HBIG) issued by Health Protection Agency (HPA) via local consultant microbiologist. Order well in advance of birth Labour When an HBsAg +ve mother arrives in labour or for caesarean section, labour ward must inform on-call neonatal team Postnatal For all newborns, check screening results of mothers antenatal tests If antenatal testing not done, request urgent maternal HBsAg test
COMMUNICATION
Give mother information about hepatitis B, modes of transmission and prevention of spread Mother may breastfeed
Give low-birth-weight and premature babies full neonatal dose hepatitis B vaccine Give HBIG and hepatitis B vaccine to babies with birth weight <1.5 kg born to mother with hepatitis B, regardless of mothers HBeAg status Give hepatitis B vaccine to HIV exposed/infected babies
When Give within 24 hr of birth, ideally as soon as possible after delivery What Give hepatitis B vaccine 0.5 mL IM. Caution: brands have different doses [e.g. EngerixB 10 microgram (recommended), HBVaxPro Paediatric 5 microgram] HBIG 2 mL (whole ampoule) 200 units additionally given to babies of highly infectious mothers (see Table above)
How Use two separate injection sites for hepatitis B vaccine and HBIG, in anterolateral thighs (not buttocks) Give hepatitis B vaccine IM, except in bleeding disorder where it may be given deep subcutaneously Relationship to other immunisations No need to delay BCG following HBIG Hepatitis B vaccine may be given with other vaccines, but use separate site. If same limb used, give vaccines >2.5 cm apart
SUBSEQUENT MANAGEMENT
Further doses 2nd dose at 1 month 3rd dose at 2 months 4th dose at 12 months Give appointment for next dose or ensure agreement to give vaccine at GP practice 1 year follow-up Check childs HBsAg status at one year old (to assess if chronic carrier, or vaccine was effective). This testing can be carried out at same time as 4th dose if a carrier, refer to liver team for further management
HEPATITIS C
ANTENATAL
High-risk groups: intravenous drug users (or women with partners who are IVDU) from a country of high prevalence [e.g. North Africa (particularly Egypt), Middle East] Discuss baby testing with mothers who had hepatitis C during antenatal period If maternal HCV RNA negative, baby not at risk
POSTNATAL
Hepatitis C antibody testing after 18 months (serum, clotted specimen) If antibody positive or if HIV co-infected, test for HCV RNA (EDTA) If RNA positive, check ALT and refer to regional hepatitis unit
DOCUMENTATION
Document hepatitis C follow-up visits in Red book to ensure health visitor aware and baby followed up
BREASTFEEDING
Mother may breastfeed
Evidence Levels: I; II Last amended July 2013 Last reviewed July 2013
HSV 201315
HERPES SIMPLEX
ASYMPTOMATIC BABY, MOTHER WITH HSV INFECTION
Indications for prophylactic aciclovir Primary maternal HSV (definition: no history of genital herpes before 3rd trimester) and vaginal delivery Primary maternal HSV delivering by Caesarean section with additional risk factor Mother with visible lesions of genital herpes at time of delivery AND any of the following: rupture of membranes >6 hr invasive procedure (e.g. scalp electrode, fetal blood sampling: avoid if HSV known) cervicitis or chorioamnioitis Management of baby Prophylactic aciclovir 20 mg/kg IV 8-hrly for baby Take swab from nasopharynx, eye and any skin lesions 24 hr after delivery put in viral transport medium send blood for HSV DNA PCR if HSV PCR negative, and no symptoms suggestive of HSV disease, stop aciclovir Babies born to mothers with history of recurrent HSV but no active lesions: take swabs as above but, if baby well, no need for prophylaxis or admission if swabs positive for HSV, isolate and treat with IV aciclovir for 10 days if baby well
FOLLOW-UP
If persistent positive PCR or relapse, discuss prophylactic aciclovir 300 mg/m2 8-hrly oral for 6 months with infectious diseases/microbiology review monthly to monitor neutrophil count and adjust dose according to body surface area
Mofenson LM, Lambert JS, Stiehm ER, et al. Risk factors for perinatal transmission of human immunodeficiency virus type 1 in women treated with zidovudine. N Engl J Med 1999;341:385-93 Siegfried N, van der Merwe L, Brocklehurst P, et al. Antiretrovirals for reducing the risk of mother-tochild transmission of HIV infection. Cochrane Database of Systematic Reviews 2011, Issue 7. Art. No.: CD003510 Thea DM, Steketee RW, Pliner V, et al. The effect of maternal viral load on the risk of perinatal transmission of HIV-1. New York City Perinatal HIV Transmission Collaborative Study Group. AIDS 1997;11:437-44
Evidence Level: I Should delivery be by elective caesarean section? A recent review (Mitchla, 2000) states that There is still no information as to whether (caesarean section) provides any added benefit for women on highly active antiviral therapy with an undetectable HIV viral load. The American College of Obstetricians and Gynecologists originally recommended, in 1999, that caesarean section should be offered to all HIV-seropositive pregnant women. A survey of 2,000 randomly-selected obstetricians and gynaecologists in the U.S. (Rowland, 2001) found, however, that 47% of respondents disagreed with this recommendation, and 72% did not advise caesarean delivery in women with undetectable viral loads. Current recommendations (Anon, 2001) are that there is no evidence of benefit in women with viral loads < 1000 copies/ml, but that the individuals wishes regarding mode of delivery should be respected. The European Collaborative Study (Boer, 2010), a cohort study on 5238 mother-child pairs (MCPs), found that, amongst MCPs with maternal HIV RNA<400 HIV-1 RNA copies/mL (n=960), elective caesarean section (CS) was associated with 80% decreased transfer risk (AOR 0.20; 95% CI 0.05-0.65). Two infants born to 559 women with viral loads <50 copies/mL were infected, one of whom was delivered by elective CS (transmission rate 0.4%; 95% CI 0.04-1.29).
Anon. Scheduled cesarean delivery and the prevention of vertical transmission of HIV infection. ACOG committee opinion Number 234, May 2000 (Replaces Number 219, August 1999). Committee on Obstetric Practice. Int J Gynecol Obstet 2001;73:279-81 Boer K, England K, Godfried MH, et al. Mode of delivery in HIV-infected pregnant women and prevention of mother-to-child transmission: changing practices in Western Europe. HIV Medicine 2010;11:368-78 Mitchla Z, Sharland M. Current treatment options to prevent perinatal transmission of HIV. Expert Opin Pharmacother 2000;1:239-48 Rowland BL, Vermillion ST, Soper DE. Scheduled cesarean delivery and the prevention of human immunodeficiency virus transmission: a survey of practicing obstetricians. Am J Obstet Gynecol 2001;185:327-31
Evidence Level: V Should breast-feeding be avoided? In a small study involving 17 samples of breast milk from 4 HIV-positive mothers (Chantry, 2000) 15 (88%) showed measurable HIV-1 proviral DNA, despite all mothers having had low or undetectable viral loads. Advice from BHIVA (2012) and the U.S. Public Health Service Task Force (Anon, 2005) is that all HIV-seropositive mothers should avoid breast-feeding. To prevent the transmission of HIV infection during the postpartum period, the British HIV Association and Children's HIV Association (BHIVA/CHIVA) continue to recommend the complete avoidance of breast feeding for infants born to HIV-infected mothers, regardless of maternal disease status, viral load or treatment. (see top of page)
Anon. Recommendations for use of antiretroviral drugs in pregnant HIV-1-infected women for maternal health and interventions to reduce perinatal HIV-1 transmission in the United States. Public Health Service Task Force, 2005
http://aidsinfo.nih.gov/guidelines/perinatal/PER_022405.pdf British HIV Association (BHIVA). Guidelines for the management of HIV infection in pregnant women 2012 http://www.bhiva.org/documents/Guidelines/Treatment/2012/120430PregnancyGuidelines.pdf Chantry CJ, Morrison P, Panchula J, et al. Effects of lipolysis or heat treatment on HIV-1 provirus in breast milk. J Acquir Immune Defic Syndr 2000;24:325-9
Evidence Level: IV Should the infant be tested with pro-viral DNA/RNA PCR? A prospective study compared DNA-PCR and viral RNA amplification and detection in 44 HIV-infected infants and 9 uninfected infants (Brown, 1996). Specimens were tested at 3 stages between birth and around 35 days of age, and in each case, viral RNA was found to be more sensitive than DNA-PCR. After the first month of life, the sensitivity of the DNA-PCR increases from 50% to 96% (Cervia, 2003). As viral RNA levels increase rapidly from birth and reach a peak at 1-2 months of age (Shearer, 1997), testing during this period should be conclusive on the question of whether or not transmission has occurred. The available evidence, however, is at present inconclusive as to the value of testing or treating infants of mothers with undetectable viral load (see 1st question).
Brown TM, Steketee RW, Abrams EJ, et al. Early diagnosis of perinatal HIV infection comparing DNApolymerase chain reaction and plasma viral RNA amplification. Int Conf AIDS 1996 Jul 7-12 (abstract no. Tu.B.2374) Cervia J, Kaplan B, Schuval S, et al. Virologic testing in the management of perinatal HIV exposure. AIDS Read 2003;13:39-46 Shearer WT, Quinn TC, LaRussa P. Viral load and disease progression in infants infected with human immunodeficiency virus type 1. N Engl J Med 1997;336:1337-42
Evidence Level: V Last amended August 2013 Last reviewed August 2013
ACTIONS
Any red flags or no red flags but 2 risk factors or clinical indicators perform investigations, including blood cultures, and start antibiotics No red flag or clinical indicators but one risk factor or no red flag or risk factors but 1 clinical indicator use clinical judgement and consider withholding antibiotics monitor baby for clinical indicators of possible infection, including vital signs monitor for at least 12 hr from birth (at 1 hr, 2 hr and then 2-hrly for 10 hr) If further clinical concerns, perform investigations including blood cultures and start antibiotics if decision made to give antibiotics, aim to start within 30 min and always within 1 hr of decision
Choice of antibiotics Use benzylpenicillin and gentamicin as first choice for empirical treatment Benzylpenicillin 25 mg/kg 12-hrly If baby appears very ill, give 25 mg 8-hrly Gentamicin Follow local guideline or: 5 mg/kg if a second dose to be given (see below), give 36 hr after first dose interval may be shortened based on clinical judgement e.g. for Gram-negative infection or if baby appears very ill Monitoring of gentamicin see below
Stop at 36 hr if: initial clinical suspicion of infection was not strong and CRP <10 mg/L on both tests and negative blood culture and baby is well with no clinical indicators of possible infection Treat for 7 days if: strong clinical suggestion of infection continued clinical concerns about infection at 36 hr CRP >10 mg/L on either measurement positive blood culture If baby not fully recovered at 7 days, continue antibiotics this is advisable based on blood culture result and expert microbiological advice if necessary Meningitis If meningitis suspected but Gram stain is uninformative, use amoxicillin and cefotaxime Review treatment decisions taking CSF results into account
If CSF Gram stain suggests GBS, give benzylpenicillin 50 mg/kg 12-hrly and gentamicin 4.5 mg/kg every 36 hr If CSF culture confirms GBS, continue benzylpenicillin for at least 14 days and gentamicin for 5 days If CSF culture or Gram stain confirms Gram-negative infection, stop amoxicillin and treat with cefotaxime alone If blood culture or CSF culture is positive for listeria, consider stopping cefotaxime and treating with amoxicillin and gentamicin If CSF Gram stain or culture suggests any organism other than GBS, use an antibiotic regimen based on local expert microbiological advice
Therapeutic monitoring of gentamicin Follow local guidelines or: Trough concentrations: if second dose to be given, measure before administering review level before giving third dose monitor before every third dose, or more frequently if necessary (e.g. concern about previous level or renal impairment) adjust dose interval aiming to achieve level of <2 mg/L if course lasts >3 doses, level of <1 mg/L is advisable if a trough level is not available, do not withhold next dose of gentamicin Peak concentrations: measure in selected babies e.g. - with oedema - with macrosomia (birth weight >4.5 kg) - who do not respond to treatment Measure 1 hr after starting gentamicin infusion If peak is <8 mg/L, increase dose
Infection 201315
DEFINITION
Infection after first 72 hr of life Late onset Group B streptococcus (GBS) infection: after first 6 days of life When acquired in hospital most commonly Gram-positive organisms. Coagulasenegative staphylococci account for approximately 50% of all late onset infections Gram-negative bacteria accounts for 2040% and these are increasingly resistant to gentamicin (Klebsiella>Serratia>Enterobacter>Pseudomonas>E.coli and Acinetobacter)
Risk factors Risk of infection is inversely related to gestational age and birth weight and directly related to severity of illness at birth, reflecting need for invasive interventions e.g. prolonged ventilation, central venous access and parenteral nutrition Delayed introduction of enteral feeds is associated with higher infection rates Increased risk of sepsis after gut surgery especially if enteral feeds slow to establish e.g. post-gastroschisis or necrotising enterocolitis (NEC) with stoma
PREVENTION
Strict hand washing and alcohol hand rubs: to the elbow with particular attention between digits on entering the unit and between each patient Unless absolutely essential, avoid entering incubators or touching any part of cots Do not lean on incubators or other patient equipment Wear apron and gloves when carrying out any procedure e.g. heel prick, resiting IV cannula Meticulous regimen for changing drips and 3-way taps Initiate enteral feeds with maternal breast milk within 24 hr of birth
PRESENTATION
Can be vague and non-specific Symptoms Respiratory distress increase in oxygen requirement/respiratory support Apnoea/bradycardia Cyanosis or poor colour Poor perfusion (CRT >3 sec; toe-core temperature gap >2C; mottling) Hypotension Tachycardia Temperature instability (high or low) Glucose instability Hypotonia Irritability Lethargy/inactivity Poor feeding and poor suck Jaundice Seizures Vomiting Abdominal distension Nursing staff may describe babies with a mixture of these symptoms as having gone off Signs Look for Systemic signs of sepsis such as tachycardia, poor perfusion, reduced tone, quiet, lethargy Tachypnoea and intercostal and/or subcostal recession Bulging of the fontanelle suggesting raised intracranial pressure not always detectable in babies with neonatal meningitis
Infection 201315
Abdominal distension and tenderness auscultate for bowel sounds; reduced or absent with infection (as a result of septic ileus) or NEC inspect stool for visible blood petechiae, bleeding diathesis Septic spots in eyes, umbilicus, nails and skin Reluctance to move or tenderness in joints and limbs suggestive of osteomyelitis or septic arthritis
INVESTIGATIONS (perform before starting antibiotics) Swabs for culture Swab any suspicious lesion (e.g. skin, umbilicus or nails) Routine rectal swabs may detect resistant Gram-negative bacteria that require treatment with an alternative antibiotic e.g. meropenem, or MRSA which requires treatment with vancomycin. Otherwise swabs are not diagnostically useful
Blood cultures From a peripheral vein, using a closed system, non-touch, aseptic technique Full blood count A neutrophil count <2 or >15 109/L (supportive but not diagnostic, and marginally more sensitive than a total white cell count) Platelet count of <100 109/L Toxic granulation in neutrophils [or if measured: an immature:total (I:T) neutrophil ratio >0.2] Clotting profile If evidence of bleeding diathesis or in severe infection/septicaemia CRP Acute phase protein synthesised in the liver in response to inflammatory cytokines Generally a delay of 24 hr between onset of symptoms and rise in serum CRP Take sample at presentation and further sample 1824 hr after first CRP sample a rise may support diagnosis of infection but failure to rise does not exclude it where other findings are supportive if blood culture negative and clinical condition satisfactory, failure of CRP to rise during first 48 hr is a useful indicator that antibiotics may be safely stopped Urine microscopy, culture and sensitivity Clean-catch or supra-pubic aspiration (SPA). Use ultrasound scan to check urine in bladder before SPA do not send urine collected in a bag for culture Lumbar puncture (LP) If baby unstable, deranged clotting or thrombocytopenia, discuss advisability with consultant May be performed later but cultures often negative Send CSF for urgent Gram-stain and culture (MC&S), protein and glucose In critically ill baby, consider PCR for HSV, especially term babies Others Chest X-ray If abdominal distension noted, abdominal X-ray Documentation Always contemporaneously document symptoms and signs of infection at the time of taking blood culture and all blood and CSF cultures (and abdominal radiographs) on BadgerNet ad-hoc reporting field
Infection 201315
EMPIRICAL TREATMENT
Do not use oral antibiotics to treat infection in babies Late onset sepsis Antibiotics First line: empirical flucloxacillin and gentamicin unless microbiology isolates dictate otherwise (see Neonatal Formulary for dose intervals) Second line: vancomycin plus gentamicin or tazocin Third line or if cultures dictate: meropenem plus vancomycin When course of antibiotic prolonged >1 week, babies are very preterm and post-gut surgery, consider commencing fungal prophylaxis with either oral and topical nystatin or IV/oral fluconazole. Steroid therapy also associated with increased risk of fungal infection Do not use vancomycin routinely: for babies with indwelling catheters and on parenteral nutrition, unless they are very unwell to treat endotracheal secretion colonisation with coagulase-negative staphylococci (CONS) Maintain vancomycin trough levels between 1015 g/mL, as bactericidal activity is related to trough concentration When culture results available, always change to narrowest spectrum antibiotic, or stop antibiotics if negative cultures, inflammatory markers not raised and no clinical signs of infection Remove indwelling catheters for all infections except CONS If line precious and baby responding to treatment, consider infusing vancomycin down long line and leaving it to dwell for 1 hour before flushing. Ensure therapeutic trough levels If meningitis diagnosed or strongly suspected clinically, treat with high dose cefotaxime 50 mg/kg/dose give nystatin (oral/nasogastric) or fluconazole IV/oral prophylaxis to all babies treated with broad spectrum antibiotics if policy in your unit If baby has improved clinically and bacteriological cultures are negative so far, stop antibiotics after 48 hr treat for at least 7 days, or for 5 days after clinical response
SPECIFIC INFECTIONS
Discharging eyes See Conjunctivitis guideline Umbilicus sepsis (omphalitis) Systemic antibiotics required only if local induration or surrounding reddening of the skin Meningitis For all babies with a positive blood culture, other than CONS, consider LP.. This must be discussed with an experienced clinician. Organisms such as Group B streptococcus and E. coli penetrate the CSF readily Empirical treatment whilst CSF results pending CSF visually clear, give first line antibiotics as per guidance for late-onset sepsis CSF cloudy or high clinical suspicion of meningitis, give high-dose cefotaxime Subsequent management Meningitis confirmed when organisms seen on urgent Gram stain and/or grown from subsequent culture Cultures often negative, especially if CSF taken after baby given antibiotics or if mother given intrapartum antibiotics No other single CSF value can reliably diagnose meningitis. However, meningitis is suggested by: >10 WBC in CSF (especially if lymphocyte predominance) in a bloody tap: ratio of >1 WBC: 500 RBCs
Infection 201315
low CSF glucose: <2/3 simultaneous blood glucose high CSF protein: >1 g/L Send CSF for herpes PCR and start empiric treatment with IV aciclovir until results available if: neonatal herpes encephalitis suggested by symptoms and signs of infection, which may or may not include seizures and CSF showing monocytosis or lymphocytosis, increased protein and decreased glucose If bacterial meningitis confirmed on culture or high clinical suspicion of meningitis, treat with high-dose cefotaxime for 1421 days, depending on organism. Seek advice from microbiologist If low clinical suspicion of meningitis, stop antibiotics after 48 hr if: CSF glucose > 2/3 simultaneous blood glucose and CSF protein <1 g/L cultures negative and baby remains well
Urinary tract infection (UTI) Usually occurs as late-onset infection Start IV empiric antibiotic treatment, as above, immediately after appropriate urine collection (not bag urine) Continue IV empiric antibiotics until culture results available once stable, treat with oral antibiotics according to sensitivities Exclude obstruction by renal ultrasound scan as soon as available Subsequent management Prophylaxis: single night-time dose of oral trimethoprim 2 mg/kg/dose for all babies with confirmed UTI, while completing investigations to identify predisposing factors For further information on management of UTI in babies See Urinary tract infection guideline in Partners in Paediatrics guidelines Necrotising enterocolitis See Necrotising enterocolitis guideline Fungal infection Mostly late onset Incidence in UK up to 1.2% in very-low-birth-weight (VLBW) babies and 2.6% in extremely-low-birth-weight (ELBW) babies (versus up to 28% in the USA), hence no routine prophylaxis in the UK Risk factors <1500 g Parenteral nutrition Indwelling catheter No enteral feeds Ventilation H2 antagonists Exposure to broad spectrum antibiotics, especially cephalosporins Abdominal surgery Peritoneal dialysis. Symptoms and signs Non-specific as for late onset infection Additional investigations If fungal infection suspected or diagnosed, end-organ evaluation to include: abdominal ultrasound cerebral ultrasound lumbar puncture fundoscopy
Infection 201315
echocardiogram blood cultures 2448 hrly to confirm clearance suprapubic or catheter specimen of urine
Treatment First choice Liposomal amphotericin start at 12 mg/kg. Can increase dose as tolerated to maximum of 6 mg/kg (see Neonatal Formulary for doses and intervals)
ADJUNCTIVE THERAPY
No substantive trials to date show benefit of IV immunoglobulin, recombinant cytokines etc
Puri J, Revathi G, Faridi MM, et al. Role of body surface cultures in prediction of sepsis in a neonatal intensive care unit. Ann Trop Paediatr 1995;15:307-11 Van den Bruel A, Thompson MJ, Haj-Hassan T, et al. Diagnostic value of laboratory tests in identifying serious infections in febrile children: systematic review. BMJ 2011; 342:d3082
Evidence Level: I A study in 6,207 infants (Bonsu, 2003) found that no threshold of the total peripheral white blood cell (WBC) count had both good sensitivity and specificity. At a count cutoff of 5,000 cells/mm3, sensitivity and specificity were 79% and 5%; at a cutoff of 15,000 cells/mm3, 45% and 78%. The authors concluded that the test was relatively inaccurate and that decisions to obtain blood cultures should not rely on it alone. A practice guideline (Baraff, 1993) had previously suggested that a WBC count threshold of 15,000/mm3, having a negative predictive value of 97.6%, but a positive predictive value of only 13%, could be used to avoid unnecessary requests for blood cultures. Another study, comparing WBC with absolute neutrophil count (ANC) in 170 infants (Gombos, 1998), concluded that both tests were fair indicators for occult bacteremia. WBC had a sensitivity of 61% and a sensitivity of 59%, with 61% and 68% for ANC. A prospective study of 1920 patients (Purcell, 2007) found that The probability of an abnormal WBC count <5000 and 15,000-30,000 being associated with a concurrent serious bacterial infection was very low and no different from that of a normal WBC count in febrile patients admitted with respiratory syncytial virus lower respiratory tract infection.
Baraff LJ, Bass JW, Fleischer GR, et al. Practice guideline for the management of infants and children 0 to 36 months of age with fever without source. Pediatrics 1993;92:1-12 Bonsu BK, Harper MB. Identifying febrile young infants with bacteremia: is the peripheral white blood cell count an accurate screen? Ann Emerg Med 2003;42:216-25 Gombos MM, Bienkowski RS, Gochman RF, et al. The absolute neutrophil count: is it the best indicator for occult bacteremia in infants? Am J Clin Pathol 1998;109:221-5 Purcell K, Fergie J. Lack of usefulness of an abnormal white blood cell count for predicting a concurrent serious bacterial infection in infants and young children hospitalized with respiratory syncytial virus lower respiratory tract infection. Pediatr Infect Dis J 2007;26:311-5
Evidence Level: III A prospective study of 301 screening episodes for neonatal sepsis (Garland, 2003) found that no single test alone was sufficiently reliable to accurately predict early onset sepsis. Creactive protein (CRP) had a sensitivity of 67% and a negative predictive value of 86%. This compared to 63% and 80% for full blood examination and 57% and 83% for gastric aspirate. Another prospective study in 1,186 infants (Benitz, 1998) concurred with this view, but concluded that two CRP measurements <1 mg/dl obtained 24 hours apart, 8-48 hours after presentation, indicate that bacterial infection is unlikely and thus that antibiotics are not needed. Alternatively, many studies have observed that CRP in combination with other tests (such as WBC count) results in improved sensitivity (Arnon, 2004; Hengst, 2003; Laborada, 2003; Manucha, 2002). A prospective study off 711 patients with pneumonia (Clark, 2007) found that C-reactive protein was not associated with the degree of severity of the illness. A systematic review (Sanders, 2008) concluded that poor sensitivity associated with CRP meant that it should not be used as a single test for excluding bacterial infection.
Arnon S, Litmanovitz I, Regev R, et al. The prognostic virtue of inflammatory markers during late-onset sepsis in preterm infants. J Perinat Med 2004;32:176-80 Benitz WE, Han MY, Madan A, et al. Serial serum C-reactive protein levels in the diagnosis of neonatal infection. Pediatrics 1998;102:E41 Clark JE, Hammal D, Spencer D, et al. Children with pneumonia: how do they present and how are they managed? Arch Dis Child 2007;92:394-8
Garland SM, Bowman ED. Reappraisal of C-reactive protein as a screening tool for neonatal sepsis. Pathology 003;35:240-3 Hengst JM. The role of C-reactive protein in the evaluation and management of infants with suspected sepsis. Adv Neonatal Care 2003;3:3-13 Laborada G, Rego M, Jain A, et al. Diagnostic value of cytokines and C-reactive protein in the first 24 hours of neonatal sepsis. Am J Perinatol 2003;20:491-501 Manucha V, Rusia U, Sikka M, et al. Utility of haematological parameters and C-reactive protein in the detection of neonatal sepsis. J Paediatr Child Health 2002;38:459-64 Sanders S, Barnett A, Correa-Velez I, et al. Systematic review of the diagnostic accuracy of C-reactive protein to detect bacterial infection in nonhospitalized infants and children with fever. J Pediatr 2008;153:570-4
Evidence Level: III A study in 3,339 neonates (Galanakis, 2002) found that respiratory distress syndrome was the main risk factor for late-onset sepsis (RR 5.70). A prospective study in 145 infants referred because of respiratory distress (Dorond, 1979) found a 4.8% incidence of bacteremia, with confirmed septicaemia in 3.5%. The authors concluded that antibiotics should not be given routinely in such cases, in view of the low incidence of confirmed septicaemia. In a prospective study of 116 infants with respiratory distress (Boyle, 1978), 9 (8%) were septic. WBC count would have provided early identification of 8 of these, as well as false positive results for 14% (15/105) of the remainder, which, in the authors estimation, would have justified antibiotic treatment for those with a cutoff of <10,000/mm3.
Boyle RJ, Chandler BD, Stonestreet BS, et al. Early identification of sepsis in infants with respiratory distress. Pediatrics 1978;62:744-50 Dorond RD, Cook LN, Andrews BF. Incidence of sepsis in neonates with clinical respiratory distress. South Med J 1979;72:1262-4 Galanakis E, Krallis N, Levidiotou S, et al. Neonatal bacteraemia: a population-based study. Scand J Infect Dis 2002;34:598-601
Evidence Level: III A retrospective study of 117 women with PROM (Chua, 1995) found that prolongation of PROM to delivery interval for >48 hours increased the incidence of infection in their infants (33% vs 8.8% and 8.9% for intervals of <12 hours and 12-24 hours respectively. In a secondary analysis of data from 5,041 women in the International Multicenter Term PROM Study (Seaward, 1998), the following were identified as independent predictors of neonatal infection: Clinical chorioamnionitis (OR 5.89, P<.0001) Positive maternal group B streptococcal status (vs negative or unknown, OR 3.08, P<.0001) 7-8 vaginal digital examinations (vs 0-2, OR 2.37, P=.04) 24-<48 hours from membrane rupture to active labour (vs <12 hours, OR 1.97, P=.02) >/= 48 hours from membrane rupture to active labour (vs <12 hours, OR 2.25, P=.02) Maternal antibiotics before delivery (OR 1.63, P=.05)
Chua S, Arulkumaran S, Sailesh KS, et al. Prelabour rupture of membranes to delivery interval related to the incidence of maternal and neonatal infection. J Obstet Gynaecol 1995;21:367-72 Seaward PG, Hannah ME, Myhr TL, et al. International Multicenter Term PROM Study: evaluation of predictors of neonatal infection in infants born to patients with premature rupture of membranes at term. Am J Obstet Gynecol 1998;179:635-9
Evidence Level: III Discharging eyes in neonates are commonly due to vertical transmission of a sexually transmitted disease (chlamydia or gonorrhoea) from the mother (Winceslaus, 1987). Group B streptococcus may, however, also be a causative organism (Poschl, 2002).
Poschl JM, Hellstern G, Ruef P, et al. Ophthalmia neonatorum caused by group B streptococcus. Scand J Infect Dis 2002;34:921-2 Winceslaus J, Goh BT, Dunlop EM, et al. Diagnosis of ophthalmia neonatorum. BMJ 1987;295:1377-9
Evidence Level: V Acute inflammation of the umbilical cord (funisitis) was associated with a significantly higher rate of congenital sepsis in a study of 315 consecutive singleton preterm births (Yoon, 2000): 12% (8/66) vs 1% (3/216).
Yoon BH, Romero R, Park JS, et al. The relationship among inflammatory lesions of the umbilical cord (funisitis), umbilical cord plasma interleukin 6 concentration, amniotic fluid infection, and neonatal sepsis. Am J Obstet Gynecol 2000;183:1124-9
Evidence Level: IV Last amended August 2013 Last reviewed August 2013
MRSA 201315
SCREENING
Babies transferred from other hospitals Screen on arrival. Include babies who attend other hospitals for invasive day case procedures (e.g. PDA ligation) Obtain: swabs: nose, perineal, umbilicus if still moist and any skin lesion urine if long-term urinary catheter present rectal swab for ESBL (multi-resistant gram-negative bacteria) Take standard precautions until results available
MANAGEMENT
Incidental finding of MRSA Mother Screen mother with nasal, perineal, wound and skin lesion swabs, if: delivery by caesarean section mother had recent admission to hospital before delivery mother has chronic health problem (e.g. DM, asthma) mother has other risk factor: high BMI or is a healthcare worker with patient contact mother or household member has a history of skin/soft tissue infection abscess or recurrent skin infections in the last 12 months If none of these risk factors present, screening is not necessary Contacts on NICU Screen babies who have been in NICU >2 weeks Those who have been in close proximity of the index case (i.e. in the same room) Others (potentially all) following a risk assessment and discussion with consultant of the week, co-ordinator and consultant microbiologist Healthy babies about to be discharged do not require screening MRSA outbreak Two or more babies with same strain of MRSA constitutes an outbreak MRSA from different babies are considered the same if they have been sent by microbiology to a reference lab for typing and have been reported by reference lab as indistinguishable Action Screen all babies in neonatal unit (swabs as above) Optimise infection control measures: see local infection control policy If further cases of the same strain occur: nose and throat swab and swabs of any skin lesions from all staff screening is co-ordinated by infection control team (ICT) in collaboration with occupational health (OH) department at an outbreak meeting results are sent to OH and ICT but not to the unit
MRSA 201315
Repeat screening swabs only when all antibiotic treatment has finished and if baby not about to be discharged Decolonisation can be assumed if 3 consecutive swabs taken at 37 day intervals are negative. Do not attempt to decolonise more than twice during any one admission
Palivizumab 201315
PALIVIZUMAB
INDICATIONS
Chronic lung disease (CLD) defined as requiring oxygen for 28 days from birth and in shaded area below (age on 1st October) Gestational age at birth (whole weeks) Chronological age (months) 1.0 to <1.5 1.53 36 69 >9
24 >24 to 26 >26 to 28 >28 to 30 >30 to 32 >32 to 34 35
Haemodynamically significant acyanotic CHD including pulmonary hypertension and in shaded area below (age on 1st October)
Gestational age at birth (whole weeks)
24
>24 to 26
>26 to 28
>28 to 30
>30 to 32
>32 to34
35
DH Excel toolkit for calculating eligibility: http://webarchive.nationalarchives.gov.uk/+/www.dh.gov.uk/en/Publichealth/Immunisation /Keyvaccineinformation/DH_104070 Children <24 months with severe combined immunodeficiency syndrome (SCID) Long-term ventilated (LTV) children <12 months or <24 months if on oxygen Babies aged <12 months (corrected gestational age, if preterm) and have 1 of the following high risk categories: preterm babies in oxygen at 36 weeks corrected age term/preterm babies on home oxygen haemodynamically significant acyanotic CHD at any gestation including pulmonary hypertension cyanotic or acyanotic CHD plus significant co-morbidities particularly if multiple organ systems are involved
PROCEDURE
Consultant neonatologist will identify patient and sign accompanying letter to GP 5 doses monthly in RSV season at the beginning of October, November, December, January and February give appointment for subsequent doses at palivizumab clinic (if held) where possible, administer 1st dose before start of RSV season 15 mg/kg by IM injection into antero-lateral aspect of thigh Order palivizumab injection from local community or hospital pharmacy (this can take some days) Palivizumab must be stored at 28C. Full reconstitution and administration instructions are provided in the Summary of product characteristics (SPC). It is important to: avoid shaking vial stand at room temperature for minimum of 20 min until solution clarifies administer within 3 hr of preparation (palivizumab contains no preservative) ensure final concentration when reconstituted 100 mg/mL split between 2 sites if >1 mL (final concentration when reconstituted 100 mg/mL)
Palivizumab 201315
DOCUMENTATION
After immunisation, document the following in case notes as well as in Child Health Record (Red book): consent gained from parents vaccine given and reasons for any omissions site of injection(s) in case of any reactions batch number of product(s) expiry date of product(s) legible signature of doctor administering immunisations adverse reactions Sign treatment sheet Update problem sheet with date and immunisations given Document all information on discharge summary and medical case notes including recommendations for future immunisations and need for any special vaccinations, such as influenza, palivizumab, etc
Clarify maternal treatment and post-treatment titres if possible Discuss management plan with parents before birth if possible Follow Management Flowchart
CLINICAL FEATURES
Clinical evidence of early congenital syphilis Rash Infectious snuffles (copious nasal secretions) Haemorrhagic rhinitis Osteochondritis Periostitis Pseudo-paralysis Mucocutaneous patches Peri-oral fissures Hepatosplenomegaly Lymphadenopthy Oedema Glomerulonephritis Ocular or neurological involvement Haemolysis Thrombocytopenia
Maternal treatment is adequate if: treated with full course of penicillin: 3 injections over 3 wk >4 wk before delivery AND there is a documented four-fold decrease in VDRL titres
INVESTIGATIONS
Diagnostic serology Baby may have positive serology depending on timing of maternal infection, therefore mother must be screened simultaneously for titre comparison. DO NOT USE CORD BLOOD Non-treponemal tests Venereal disease research laboratory (VDRL) test: 4 x decrease in titre = effective treatment 4 x increase after treatment = relapse or re-infection May be false negative in babies who acquire congenital syphilis in late pregnancy or have extremely high antibody titres before dilution (prozone phenomenon) May be false positive in viral infections (Epstein Barr, varicella zoster, hepatitis, measles), tuberculosis, endocarditis, malaria, lymphoma, connective tissue disease, pregnancy, intravenous drug use, and wharton jelly contamination of cord blood samples
Syphilis author 201315 Treponemal tests IgM Treponema. pallidum particle agglutination test (TPPA) Treponema. pallidum haemagglutination assay (TPHA) Fluorescent treponemal antibody absorption test (FTA-ABS) Tests may also be positive in other spirochetal disease e.g. yaws, pinta, leptospirosis, and Lyme disease. There is poor correlation of titres with disease activity Interpretation of syphilis serology of baby Syphilis serology is positive in baby if: anti-treponemal antibody IgM positive babys TPPA is four-times greater than repeated maternal TPPA titre at delivery Example of positive TPPA Maternal titres 1:1040 Baby serology 1:4160 (i.e. baby four-times greater than mother) Babys VDRL titre is four-times greater than repeated maternal VDRL titre at delivery Example of positive VDRL Maternal titres 1:64 Baby serology 1:256 (i.e. baby four-times greater than mother) CSF CSF investigations require at least 0.5 mL of CSF. A CSF is classed as positive if: increased WCC and protein reactive TPPA and VDRL (a negative VDRL does not exclude neurosyphillis) Remember to suspect other causes of elevated values when evaluating baby for congenital syphilis
TREATMENT
Possible congenital syphilis: benzylpenicillin 50 mg/kg IV 12-hrly for 7 days and 8-hrly for next 3 days
If delay in results, offer single dose IM benzathine penicillin while awaiting results
50,000 units/kg as a single dose by IM injection within 24 hr of decision to treat. Reconstitute vial with the solvent provided (WFI) to produce a solution containing 300,000 units/mL Example: 2 kg baby: Dose = 50,000 units x 2 = 100,000 units volume to inject = 100,000/300,000 = 0.3 mL. If >24 hr of therapy is missed, restart entire course
FOLLOW-UP
If IgM test is negative, other tests are reactive with titres <four-fold higher than mothers with no signs of congenital syphilis, repeat reactive tests at 3, 6 and 12 months or until all tests (VDRL, TPPA and IgM) become negative (usually by 6 months) If babys serum negative on screening, and no signs of congenital infection, no further testing is necessary If any doubt regarding test interpretation/follow-up, discuss with local expert in neonatal infection/microbiology If Neurosyphilis, CSF at 6 months
Syphilis author 201315 Management flowchart: Baby born to mother with positive syphilis serology (IgM/VDRL or TPPA
reactive)
Clarify maternal treatment and post-treatment titres if possible Discuss management plan with parents before birth if possible
Clinical examination of baby Assess adequacy of maternal treatment (see Assessment of maternal treatment) Baby syphilis serology IgM, VDRL+TPPA (paired sample with mother must be taken and
sent at same time)
Physical examination abnormal OR Inadequate maternal treatment (including non-penicillin regimen) OR Results from paired samples show positive baby serology (see associated text)
If examination is normal, mother has had adequate treatment but no documented reduction in titres post treatment
Physical examination normal AND Negative baby serology (VDRL, TPPA and IgM negative) AND Adequate maternal treatment (including follow-up testing to confirm successful treatment)
If results expected quickly, send child home and chase results of paired serology Warn parents baby may have to return for treatment if serology positive If delay in results, offer single dose IM benzathine penicillin* while awaiting results
+ Serology (see
associated text)
Possible congenital syphilis Evaluate: FBC and differential CSF cell count, protein, VDRL and TPPA Other tests as clinically indicated: chest X-ray, X-ray femur LFT cranial ultrasound ophthalmology review auditory brain stem responses Treat Benzylpenicillin 50 mg/kg IV 12-hrly for 7 days and 8-hrly for next 3 days
Follow-up Clinic review at 3, 6 and 12 months Repeat serology at 3, 6 and 12 months or until VDRL, TPPA and IgM all non-reactive If Neurosyphilis, CSF at 6 months
Discharge If any uncertainty about completeness of antenatal follow-up or neonatal serology, further review with serology recommended at 3 and 6 months
*Benzathine penicillin: Dose 50,000 units/kg as a single dose by IM injection within 24 hr of decision to treat Reconstitution: reconstitute vial with the solvent provided (WFI) to produce a solution containing 300,000 units/mL Example: 2 kg baby: Dose = 50,000 units x 2 = 100,000 units volume to inject = 100,000/300,000 = 0.3 mL
Maternal TB 201315
BCG
Investigate baby Three consecutive early morning pre-feed gastric washings, collected in alkali medium (arrange with microbiologist) for TB culture and AFB Chest X-ray +/- CSF Additional investigation on mother: 3 +/- maternal endometrial sample for TB culture and AFB
Asymptomatic
Symptomatic
Mantoux TB Elispot Negative (<6 mm) Continue INH. Repeat Mantoux +/- TB Elispot (inferon gamma release assay) 4 6 weeks later Positive7
Give further 3 months INH Full TB treatment. Refer to TB team or infectious diseases (ID) physician
Consultant (ID physician or paediatrician with infectious disease interest) follow-up for all these processes
Maternal TB 201315 Notes 1. As clearance of mycobacteria from pregnant mothers sputum is not clearly defined, treat newborns of any incompletely treated mother as at risk 2. Baby may acquire mycobacteria from an incompletely treated mother either in-utero, intrapartum or postpartum. Gastric washing samples taken pre-feed (usually early morning) are useful, as any potential mycobacteria caught by babys innate mucociliary escalator will be washed into trachea, bronchi and upwards, swallowed and present in the relatively less acidic neonatal stomach. Alkali solution keeps the mycobacteria alive until plated in the laboratory 3. Endometrial swab or placental sample for acid fast bacilli detection on microscopy and tuberculosis culture taken by obstetrician at delivery, especially where there has been active tuberculosis during current pregnancy. 4. If TB Elispot not available, Mantoux skin test is sufficient; provided baby has not had BCG. PS TB Elispot (T spot) takes 72 hr and cannot be carried out at weekend. Arrange with microbiology laboratory. Repeat Mantoux + TB Elispot test (see NICE guidance 2011 CG 117 page 31) 5. Symptomatic baby may include pneumonia, hepatosplenomegaly, lymphadenopathy etc 6. BCG is a live attenuated vaccine which can be affected by isoniazid (INH) 7. TB Elispot and Mantoux skin tests define infection but cannot distinguish between infection and disease
Varicella 201315
VARICELLA
RECOGNITION AND ASSESSMENT
Definition There are two separate presentations depending on timing of infection: fetal varicella syndrome: maternal chickenpox infection before 20 weeks gestation neonatal varicella: maternal infection in perinatal period or close contact with chickenpox or shingles in first 7 days after birth
Varicella 201315
Give VZIG in the following cases of postnatal exposure to varicella: varicella antibody-negative babies (this can be determined by testing mother for varicella antibodies) exposed to chickenpox or herpes zoster from any other contact other than mother, in first 7 days of life (see Decision pathway) VZ antibody-negative babies of any age, exposed to chickenpox or herpes zoster while still requiring intensive or prolonged special care nursing for babies exposed postnatally, regardless of maternal chickenpox history, who: weighed <1 kg at birth, or were 28 weeks gestation at birth, or are >60 days old, or have had repeated blood sampling with replacement by packed cell infusions perform VZ IgG assay and, if negative, give VZIG (because they are at risk of not having received or retained sufficient maternal VZ IgG)
Exposure
Yes No VZIG
Seropositive No VZIG
Symptoms and signs of neonatal varicella Mild: vesicular rash Severe: pneumonitis, pulmonary necrosis, fulminant hepatitis mortality 30% without varicella-zoster immunoglobulin (VZIG)
TREATMENT
Aciclovir Indications Babies with signs and symptoms of neonatal varicella Babies with postnatal exposure for whom VZIG was indicated (as above) but not given within 24 hr of exposure Chickenpox in baby currently treated with corticosteroids or born prematurely or immunocompromised Dosage 20 mg/kg IV (over 1 hr) 8-hrly, diluted to 5 mg/mL For renal impairment, refer to Neonatal Formulary Treat for at least 7 days, up to 21 days if severe
Varicella 201315
SUBSEQUENT MANAGEMENT
Where On postnatal ward, unless baby requires neonatal intensive care support: isolate mother and baby together in separate room until 5 days after onset of rash and all lesions crusted over if baby already exposed, breastfeeding can continue but explain to mother possible risk of transmission Staff Exposed staff with no history of chickenpox, VZ vaccination or of unknown VZ IgG status should have VZ IgG measured by Occupational Health if VZ IgG negative, immunise with varicella vaccine remove from clinical duties during days 721 following exposure if in high risk group for complications (immunocompromised), offer VZIG
MONITORING TREATMENT
Aciclovir ensure good hydration stop once clinical improvement occurs or when all lesions crusted
Evidence Level: V Last amended August 2013 Last reviewed August 2013
One or more of the following features may be present: abnormal looking perineum delayed or no passage of meconium abdominal distension bilious vomiting drooling of saliva (if coexistent oesophageal atresia)
Examination Full physical examination. Look for: dysmorphic features cardiac anomalies limb anomalies abdominal distension absence of anus (Figure 1) abnormal position of bowel opening (Figure 2). In girls, an abnormal opening may be seen at the vestibule rarely (in girls): cloaca [1 opening in the perineum instead of 3 (urethra, vagina and anus)] Caution The presence of meconium in the nappy does not exclude an ARM, as a neonate may still pass meconium through a fistula Always clean the perineum and establish that a normally sited anus is present
MANAGEMENT
Keep nil-by-mouth Insert a size 8 Fr nasogastric tube (NGT) and fix securely (see Nasogastric tube insertion guideline). Successful passage of an NGT excludes diagnosis of oesophageal atresia Empty stomach by aspirating NGT 4-hrly with a 5 mL syringe. Place NGT on free drainage by connecting to a bile bag Insert an IV cannula and obtain blood for FBC, U&E, glucose and blood cultures. Group and Coombs and crossmatch will be done at planned place of surgery Start maintenance IV fluids (see IV fluid therapy guideline) Give broad spectrum antibiotics Give vitamin K IM (see Vitamin K guideline) Collect pre-transfusion bloodspot and send with baby to surgical centre Replace nasogastric losses mL-for-mL with 10 mmol potassium chloride in 500 mL sodium chloride 0.9% IV Chest X-ray to confirm position of NGT and: vertebral anomalies abnormal cardiac outline Supine abdominal X-ray looking for: dilated bowel vertebral anomalies A combined chest and abdominal X-ray is suitable as an alternative Take photographs for parents
Referral Refer to paediatric surgical team e.g. Birmingham Childrens Hospital as an emergency via KIDS single point referral line 0300 200 1100 Obtain a sample of mothers blood for crossmatch. Handwrite form, completing all relevant sections and indicating this is the mother of the baby being transferred. Include babys name Complete nursing and medical documentation for transfer and arrange electronic transfer of any Xrays taken. Ensure you have mothers name and telephone contact details (Including ward details if she is still an in-patient). Surgeon will require verbal telephone consent if operation is required and a parent is not able to attend surgical unit at appropriate time Inform surgical unit staff when baby is ready for transfer. Have available: name, gestational age, weight, ventilatory and oxygen requirements (if applicable) and mothers name and ward (if admitted) Useful Information http://www.bch.nhs.uk/content/neonatal-surgery http://www.bch.nhs.uk/find-us/maps-directions
Evidence Level II
CONTRAINDICATIONS
Pyrexial baby. Remove any other lines e.g. PICC and administer antibiotics until apyrexial for at least 48 hr before insertion of Broviac line
Consent and communication with parents Before transferring to surgical centre, explain procedure to parents and discuss risks including: infection bleeding/bruising line dislodgement/break/blockage wound problems (especially vascuports) pneumothorax (uncommon) haemothorax (uncommon) pericardial effusion (uncommon) cardiac arrhythmias (uncommon) Inform parents the surgeon inserting the line will meet with them before the procedure to discuss their concerns and complete formal consent form if parents not be able to attend surgical centre on day of procedure, formal delegated consent must be gained by local neonatal team and completed consent form must accompany baby to surgical centre. File a copy babys healthcare record
Documentalldiscussionswithparentsinbabieshealthcarerecord
Problems in established lines Infection line cuff skin endocarditis Causes of line blockage Difficult to aspirate and flush Tip of line inserted in wrong place Lumen blocked blood clot or PN/drug concretion Fibrin sheath over end of line Thrombus at the line of line blood clot or vegetations Line tip pressed against vessel wall heart valve atrial wall Line partially pulled out tip no longer in vessel Tip eroded through vessel wall and lying outside lumen Damage to line or lumen
Complications of insertion
Pneumothorax
Malposition
Venous occlusion
INSERTION
Inserted using an ultrasound guided percutaneous approach under general anaesthetic at a paediatric surgical centre Blood transfusions due to bleeding as a complication of surgery are very rarely required and usually occur due to an underlying condition
Referral Refer urgently to on-call paediatric surgeon at planned place of surgery. Arrangements will be made on an individual basis depending on degree of urgency and clinical need Once procedure date set, liaise with the transport team Ensure a transfer letter is ready to accompany baby, together with recent FBC, clotting screen and U&E results Prepare baby for transfer. Follow pre-operative fasting instructions from the surgical team Post-operative care All lines will be imaged in theatre unless otherwise specified Line will be looped on the chest under a 3000 dressing +/- a biopatch biopatch used for babies >26 weeks and >7 days old avoid excessive pressure over the patch (risk of skin necrosis) Change dressing weekly for 3 weeks 2.7 Fr line: continuous infusion at 2 mL/hr to prevent blockage 4.2 Fr line: when not in use: heplock twice weekly with prescribed heparin 0.4 mL (100 units/mL) use aseptic technique Clamp catheter immediately following instillation of heparin To use a heplocked line, aspirate the lumen until blood is withdrawn and discard the aspirated solution
REMOVAL
Neonatal consultant will decide when line to be removed, often following discussion with surgeons Indications Line no longer needed Line blocked or damaged Cuff dislodged so that it is visible outside the skin Central line infection, not controlled by antibiotics Evidence of sepsis with no obvious cause, not controlled by antibiotics Repeated (>2) episodes of Broviac line related sepsis Preparation for removal Discuss procedure, benefits and risks with parents and document discussion in babys healthcare record Most Broviac line removals are performed on an elective basis according to the degree of urgency and other clinical needs Once a date is agreed, inform transport team Ensure transfer letter is ready to accompany baby, together with results of recent FBC, clotting screen and U&E Prepare baby for transfer. Follow pre-operative fasting instructions from the surgical team Equipment Surgical consent form Trolley Sterile dressings pack Cut-down pack (e.g. insertion of a UVC or a chest drain) Local anaesthetic (lidocaine 1% or marcaine 0.25%)
Sterile pot to send tip to microbiology Sterile gauze Cleaning fluid i.e. chlorhexidine etc Steristrips Mepore dressing
Procedure Line removal under local anaesthetic by surgical team (may be done on NNU) Monitor baby during procedure Remove dressings and clean area Inject local anaesthetic around cuff site Dissect cuff out and remove together with line tip Apply pressure to neck to prevent haematoma Close wound with Steristrips and a mepore dressing Send tip to microbiology for culture Complications Bleeding usually oozes from exit site that will settle with pressure Infection Line breaking during removal (embolisation) very rare but line tip may require removal Wound problems Embolised line Very rare but occasionally line will break causing the tip to embolise into the right atrium or pulmonary artery If line stops working, perform chest X-ray Requiring retrieval by interventional cardiologist at paediatric surgical centre. Liaise with either on-call paediatric surgeon or vascular access team at planned place of surgery Useful Information http://www.bch.nhs.uk/content/neonatal-surgery http://www.bch.nhs.uk/find-us/maps-directions
Exomphalos 201315
Umbilical cord
Liver
Diagnosis and antenatal care Majority diagnosed antenatally Often associated with chromosomal and other abnormalities Multi-professional discussions needed to carefully plan antenatal and postnatal care If suspected antenatally, refer to a fetal medicine department for further assessment Paediatric surgeon will discuss care plan with parents in the antenatal period Give parents information leaflet Aim to deliver in appropriate neonatal unit (NNU) with postnatal transfer to paediatric surgical unit Pre-delivery Liaise with on-call team at paediatric surgical centre e.g. Birmingham Childrens Hospital surgical team via KIDS 0300 200 1100 before making arrangements for elective delivery Delivery Experienced paediatrician/ANNP to attend delivery Clamp umbilical cord only after careful assessment of the umbilical defect (to avoid any bowel present at base of cord) Use plastic cord clamp (not artery forceps) on umbilical cord at least 10 cm away from where normal umbilical cord starts to avoid bowel injury Dry baby Initiate ABC resuscitation, as required. Avoid prolonged mask ventilation Nurse in supine position Pass a size 8 Fr nasogastric tube (NGT) and fix securely with tape (see Nasogastric tube insertion guideline)
Exomphalos 201315
Empty stomach by aspirating NGT with 1020 mL syringe. If <20 mL fluid aspirated, check position of tube. Place tube on free drainage by connecting to a bile bag Put on a nappy, taking care to fold it down under the defect Place babys legs and trunk, feet first, into a sterile plastic bag, to protect the defect and reduce fluid loss. Pull the draw-string under the arms, so that both arms are outside the top of the bag Show baby to parents and transfer to NNU
In NNU Careful physical examination by experienced neonatal practitioner. If baby has a major lethal congenital abnormality, local consultant to decide whether referral for management is appropriate. May require discussion with on-call consultant surgeon. If the decision is not to transfer, inform surgical unit Nurse in supine position Insert IV cannula. Avoid vein which could be used for long line e.g. antecubital fossa, long saphenous or scalp Avoid umbilical lines Take blood for: culture FBC, CRP and clotting screen, including fibrinogen U&E blood glucose and venous blood gas Crossmatch sample will be taken at surgical centre Send 1 bloodspot on neonatal screening card marked as 'pre-transfusion' (for sickle cell screening) with baby to surgical centre Administer fluid boluses as indicated by babys condition Start maintenance IV fluids (see IV fluid therapy guideline) Give vitamin K (see Vitamin K guideline) Insert NGT, leave on free drainage and aspirate NGT 4-hrly with a 20 mL enteral syringe Replace nasogastric losses mL-for-mL with potassium chloride 10 mmol in 500 mL bag sodium chloride 0.9% IV Start broad spectrum antibiotics including metronidazole Monitor blood glucose 46 hrly For exomphalos minor, remove bowel bag and dress defect with jelonet and gauze Discuss babys condition and treatment plan with parents and ensure they have seen the baby before transfer. Take photographs for parents Referral Refer baby to planned paediatric surgical unit e.g. BCH. This may require a conference call with the on-call surgeon to discuss urgency of transfer; an emergency surgical procedure is not indicated ensure you have basic demographic and clinical details and can discuss the babys condition and treatment Obtain a sample of mothers blood for crossmatch. Handwrite form, completing all relevant sections and indicating this is the mother of the baby being transferred. Include babys name Complete nursing and medical documentation for transfer and obtain copies of X-rays if taken. Ensure you have mothers contact details (ward telephone number or home/mobile number if she has been discharged). Surgeon will obtain verbal telephone consent if operation is required and a parent is not able to attend surgical unit at appropriate time Inform surgical unit staff when baby is ready for transfer. Have available: name, gestational age, weight, ventilatory and oxygen requirements (if applicable)
Exomphalos 201315
While awaiting transfer to surgical unit Reassess hourly for further fluid boluses and, if necessary, give 10 mL/kg of 4.5% human albumin solution (HAS) If evidence of a coagulopathy, treat appropriately (see Coagulopathy guidelines) Aspirate NGT 4-hrly Replace nasogastric losses mL-for-mL with potassium chloride 10 mmol in 500 mL bag sodium chloride 0.9%. Leave NGT on free drainage Transfer to surgical unit Place baby in transport incubator Take baby to parents (if not yet seen) in the transport incubator, en-route to the ambulance Ensure mothers blood, babys pre-transfusion bloodspots, letters for surgical team and all documentation accompanies baby Ensure documentation includes whether vitamin K given, the referring consultant, whether parents had antenatal counselling, mothers contact details Make and document all the usual observations during transport and on arrival at the surgical unit Useful Information http://www.bch.nhs.uk/content/neonatal-surgery http://www.bch.nhs.uk/find-us/maps-directions Parent information/support organisation (GEEPS exomphalos page) http://www.networks.nhs.uk/nhs-networks/southern-west-midlands-newbornnetwork/guidelines-1/surgical-guidelines http://www.networks.nhs.uk/nhs-networks/staffordshire-shropshire-and-black-countrynewborn/documents/Exomphalos.pdf
GASTROINTESTINAL STOMATA
TYPES OF STOMATA
Split stoma and mucus fistula Bowel is divided and both ends brought out through abdominal wall separately Proximal end is the functioning stoma and the distal end is the mucus fistula Operation note should make it clear where the stoma and mucus fistula are situated on the abdomen Stoma and mucus fistula may sometimes be fashioned sideby-side without a skin bridge. The wound is closed with dissolvable sutures Fig. 1: Split stoma and mucus fistula
Fig. 2: End stoma without mucus fistula End stoma without mucus fistula Proximal bowel end is brought out through abdominal wall as stoma and distal end is closed and left within the abdominal cavity
Fig. 3: Loop stoma (slightly prolapsed) Loop stoma Formed by suturing a loop of bowel to the abdominal wall and making an opening into bowel, which remains in continuity
MANAGEMENT
Application of stoma bag Once stoma working, fit an appropriately-sized stoma bag and empty 46 hrly In a split stoma and mucus fistula, fit the stoma bag on the stoma only where possible and leave mucus fistula exposed and dressed with a paraffin gauze dressing (e.g. Jelonet) or Vaseline and non-sterile gauze dressing Change bag every 3 days (maximum) or if it leaks Remove using a stoma adhesive remover wipe Clean stoma with warm tap water and dry with non-sterile gauze Check stoma bag on alternate days initially and weekly thereafter
Monitoring Examine babys abdomen and stoma daily Look for: dehydration abdominal distension wound infection or breakdown peri-stomal skin excoriation granulation tissue formation stomal bleeding discolouration of stoma or mucus fistula stomal prolapse or retraction stoma bag leakage rectal discharge If stoma is dusky or black, call the surgical team If skin surrounding the stoma is excoriated, identify cause and treat Weight Measure and record weight daily. Inadequate weight gain or weight loss may be secondary to: insufficient calorie intake malabsorption dehydration electrolyte abnormalities Stoma effluent Maintain a regularly updated fluid balance chart and record: fluid intake and stoma losses colour and consistency of stoma effluent Serum electrolytes Measure at least every 2 days in the first 7 post-operative days Urinary sodium and potassium Monitoring is extremely important for nutrition and growth Measure twice weekly Babies with stomata (especially small bowel stomata) are at risk of losing a significant amount of sodium into the effluent. They will often fail to gain weight if total body sodium is depleted. Serum sodium is an unreliable indicator of total body sodium. Urinary sodium and Na:K ratio are better indicators Sodium supplements usually required in babies with a small bowel stoma until the stoma closed If urinary sodium is <20 mmol/L or ratio of concentration of urinary sodium to potassium is <3:1, increase sodium intake
NUTRITION
On total parenteral nutrition and no enteral feeds Check surgical discharge letter and operation notes for instructions on starting enteral feeds Introduce enteral feeds slowly and increase gradually in accordance with local unit feeding regimen Useful indicators of potential feed intolerance are: vomiting and abdominal distension bile in nasogastric aspirates large nasogastric losses
low stoma losses indicating dysmotility/obstruction high stoma losses indicating malabsorption reducing substances or fat globules in the stool/stoma effluent
On combination parenteral nutrition and enteral feeds Increase enteral feeds gradually in accordance with local units feeding regimen It is not possible to predict how much enteral feed baby will be able to tolerate. As a general rule, the more distal the stoma, the better the tolerance of feeds The amount of stoma effluent and presence/absence of reducing substances in the stoma effluent should guide the advancement of enteral feeds On full enteral feeds Tolerance of enteral feeds can fluctuate with time and babies with stomata are at high risk of life-threatening dehydration and electrolyte abnormalities as a result of gastroenteritis. There should be a low threshold for readmission to hospital and appropriate resuscitation
COMPLICATIONS
High stoma output Daily output >20 mL/kg/day in premature or low-birth-weight babies and 30 mL/kg/day in term babies Measure serum and urinary electrolytes Replace stoma losses mL-for-mL with 10 mmol potassium chloride in 500 mL sodium chloride 0.9% IV Test stoma effluent for reducing substances and fat globules If reducing substances are positive or fat globules present, consider reduction of enteral feed or changing type of enteral feed after consultation with a surgeon, specialist surgical outreach nurse or dietitian Perform blood gas; (stoma effluent may be rich in bicarbonate and metabolic acidosis may be present; consider sodium bicarbonate supplementation) Mucus fistula If present, consider recycling of stoma effluent (see Recycling stoma losses via a mucus fistula guideline). Before recycling, consult surgical team to decide whether a contrast study through the mucus fistula is required If a contrast study advised, make arrangements with BCH and inform surgical team when the study will take place Surgical team will review and advise if recycling may start If baby not thriving, consider parenteral nutrition (see Parenteral nutrition guideline) Increasing enteral feeds in a baby with poor weight gain and a high output stoma, may worsen the situation If none of the above measures are effective, stop enteral feeds, start parenteral nutrition and consult surgical team to discuss surgical options
Stomal stenosis May be present if: stomal output reduces or stoma stops functioning stoma effluent becomes watery Call surgical team for advice Prolapse Call surgical team for advice. If stoma is discoloured, emergency action is required
STOMA CLOSURE
Often aimed to be performed when baby is well and thriving, which may be after discharge from hospital Indications for early closure are: failure to achieve full enteral feeds. recurrent stomal prolapse with or without stomal discolouration stomal stenosis high stoma output not responding to measures outlined above
Who to call when you need help? Surgical team Call team of consultant surgeon who performed the surgery In an emergency out-of-hours, on-call surgical registrar Stoma care specialist e.g. Gail Fitzpatrick at BCH (mobile 07557 001653) for management for stoma-related complications and parent and staff training Bernadette Reda, surgical outreach liaison nurse, will visit neonatal units and provide advice, support and training on surgical management Useful Information http://www.bch.nhs.uk/content/neonatal-surgery http://www.bch.nhs.uk/find-us/maps-directions
INGUINAL HERNIA
INTRODUCTION
Incidence: 0.51% in term babies and 510% in premature babies Right-sided in 50% of cases, left-sided in 10% and both sides in 40% Most cases can be managed with elective surgery at time of discharge from Neonatal Unit (NNU) Manage incarcerated hernia as a surgical emergency
CLINICAL FEATURES
Visible swelling or bulge in inguino-scrotal region in boys, inguino-labial region in girls. May be constant or intermittent, becoming more prominent with crying or straining Simple inguinal hernia: usually painless Silk sign: hernial sac palpated over cord structures (sensation may be similar to rubbing two layers of silk together). Highly suggestive of an inguinal hernia Incarcerated inguinal hernia: generally presents with a tender firm mass in the inguinal canal or scrotum baby may be fussy, unwilling to feed and crying inconsolably overlying skin may be oedematous, erythematous and discoloured may be associated abdominal distension with or without bilious vomiting arrange emergency surgical referral
INTRA-ABDOMINAL CYSTS
INTRODUCTION
This guideline does not apply to cystic structures which may be arising from the urinary tract Antenatally detected intra-abdominal cysts include: ovarian intestinal duplication mesenteric vitello-intestinal
MANAGEMENT
Antenatal Refer to/discuss appropriate place for delivery with a fetal medicine unit Send fetal ultrasound report to the neonatal surgical ward at planned place of surgery Antenatal counselling by a surgeon is not routinely offered but can be arranged at fetal medicine or parental request Delivery In the majority of cases, obstetric management will not alter Postnatal Resuscitate baby as normal Once stable, perform full postnatal physical examination see Examination of the newborn guideline Meconium pseudocyst If suspected antenatally, do not feed baby at birth Insert a size 8 Fr nasogastric tube (NGT) immediately after birth and fix securely with tape see Nasogastric tube insertion guideline Empty stomach by aspirating NGT with a 10 or 20 mL syringe if <20 mL aspirated, check position of tube Once stabilised, admit baby to Neonatal Unit (NNU) Place NGT on free drainage by connecting to a bile bag Commence intravenous fluids see IV fluid therapy guideline On day of birth, refer to on-call surgical team at planned place of surgery Other types of intra-abdominal cysts Unless significant abdominal distension present following birth, allow baby to feed normally and observe in the postnatal ward for at least 48 hr If baby well after 48 hr with no abdominal symptoms, discharge Arrange an out-patient abdominal ultrasound scan within 1 week of birth
SURGICAL REFERRAL
Urgency will depend on clinical situation Meconium pseudocyst: manage as above and refer to surgeon on day of birth Symptomatic cyst: stabilise on NNU and refer to on-call surgical team on day of presentation Asymptomatic cyst: abdominal ultrasound within 1 week of birth when result known, written out-patient referral to consultant paediatric surgeon Resolved cyst: ultrasound within 1 week of birth, even if cyst appears to have resolved during pregnancy. Arrange out-patient surgical referral
Evidence Level: IV
Created 2013
OESOPHAGEAL ATRESIA
DEFINITION
Congenital anomaly with blind ending oesophagus which may be associated with a fistula between the abnormal oesophagus and the trachea
DIAGNOSIS
Suspect antenatally if scans show polyhydramnios +/- absent stomach bubble refer to fetal medicine specialist plan appropriate place of delivery parents should meet paediatric surgeon antenatally Most cases present shortly after birth. Suspect if: history of polyhydramnios +/- absent stomach bubble frothing at mouth respiratory symptoms on feeding difficulty in passing NG tube (NGT) imperforate anus
DELIVERY
If diagnosis suspected antenatally, avoid: mask ventilation: pouch distension may lead to respiratory compromise and/or aspiration via a distal pouch fistula intubation If intubation indicated, site endotracheal tube (ETT) tip as close to carina as possible to minimise gas flow through a fistula. Ventilatory pressures should be as low as possible
Confirmation of diagnosis Experienced operator to place radio-opaque 8 Fr NGT. Typically resistance is felt 1012 cm from nostril in term baby do not use force (may lead to oesophageal perforation) AP X-ray of whole chest and abdomen diagnosis confirmed if NGT curled in upper oesophagus gastric air bubble confirms presence of fistula between trachea and distal oesophagus Do not attempt a contrast oesophagogram
Replogle tube
Duoderm
Elastoplast
Place mittens on baby to prevent tube being pulled out attach tapered end of tube to continuous suction. Start pressure at 5 kPa aiming for continuous flow of secretions from upper oesophagus. Maximum pressure 10 kPa do not share suction with other drains e.g. chest drain Baby should be relaxed and pink with no respiratory distress or secretions in the mouth Keep nil-by-mouth Flush Replogle tube with 0.5 mL sodium chloride 0.9% via the sidearm every 15 min. More frequently if visible oral secretions If using an enteral tube to drain saliva, aspirate every 15 min, more frequently if visible oral secretions or respiratory difficulty evident If no movement of secretions in Replogle tube after flushing with 0.5 mL sodium chloride 0.9% via the sidearm, change tube Do not leave syringe attached to sidearm as this will prevent the tube working effectively change tube every 10 days or daily if viscous secretions
Samples Obtain IV access Take blood for FBC, clotting, U&E, blood glucose and blood culture Birmingham Childrens hospital do not require a baby crossmatch sample before transfer (check if different surgical unit) Send 1 bloodspot on neonatal screening card to surgical unit with baby for sickle cell screening (mark card pre-transfusion) Fluids and medication Commence maintenance IV fluids (see Intravenous fluid therapy guideline) Give vitamin K IM (see Vitamin K guideline) Start broad spectrum antibiotics Referral Examine baby for other associated abnormalities (e.g. cardiac murmur, ano-rectal abnormalities). If major congenital abnormality detected, discuss with consultant before arranging transfer for management of oesophageal atresia as this may not be appropriate Discuss babys condition and treatment plan with parents and ensure they have seen baby before transfer. Take photographs for parents Contact surgical centre to arrange transfer as soon as possible Obtain sample of mothers blood for crossmatch. Handwrite form, completing all relevant sections and indicating this is the mother of the baby being transferred. Include babys name Complete nursing and medical documentation for transfer and obtain copies of X-rays if taken. Ensure you have mothers contact details (ward telephone number or home/mobile number if she has been discharged). Surgeon will obtain verbal telephone consent if operation is required and a parent is not able to attend surgical unit at appropriate time Inform surgical unit staff when baby is ready for transfer. Have available: name, gestational age, weight, ventilatory and oxygen requirements (if applicable) and mothers name and ward (if admitted) Useful Information http://www.bch.nhs.uk/content/neonatal-surgery http://www.bch.nhs.uk/find-us/maps-directions http://www.tofs.org.uk http://www.networks.nhs.uk/nhs-networks/staffordshire-shropshire-and-black-countrynewborn/documents/
Flush tube to prevent blocking of lumen: instil 0.5 mL sodium chloride 0.9% into blue sidearm and remove syringe immediately observe flow of sodium chloride 0.9% along tube
Pass Replogle tube, ideally nasally, into upper pouch until resistance felt, (approximately 10 12 cm from nostril in a term baby), withdraw slightly and fix with Elastoplast
Aim To prevent aspiration of secretions by continuous drainage of upper oesophageal pouch Equipment Replogle tube size 10 Fr + 1 spare to keep at bedside Low-flow suction Regular suction 2 mL IV syringe Sodium chloride 0.9% Duoderm dressing and Elastoplast Lubricant Monitoring Check Replogle tube several times an hour and flush to prevent blocking of lumen by instilling 0.5 mL sodium chloride 0.9% into blue sidearm, removing syringe immediately and observing the flow of sodium chloride 0.9% along the tube. Monitor oxygen saturation, respiratory status and heart rate continuously For long-term Replogle use, monitor electrolytes and consider replacement therapy Blocked tube Suspect if: no continuous flow of secretions along tube visible oral secretions baby in distress Clear airway with high-flow oropharyngeal suction Increase low-flow suction and flush Replogle tube with air, observing flow of saliva along tube If patency not restored, replace with new Replogle tube and return low-flow suction to previous level If blocked, alternate nostrils
BENEFITS
Bowel decompression Establishment of feeding Weight gain Reduced risk of colitis
CONTRAINDICATIONS
Rectal biopsies taken in preceding 24 hr Rectal bleeding (relative contraindication) Severe anal stenosis Anus not clearly identified Known surgical patient (without discussion with surgical team)
ADVERSE REACTIONS
Bleeding from anus or rectum Perforation of bowel; this is very rare Electrolyte imbalance if inappropriate fluid used or retained Vomiting Hypothermia Distress to baby and parent
Consent Explain procedure to parents/carer and obtain verbal consent Equipment Tube size 610 Fr (recommended: Conveen easicath pre-lubricated catheter) Lubricating gel (if catheter not lubricated) Bladder tip syringe no smaller than 60 mL Rectal washout solution (sodium chloride 0.9%) warmed to room temperature Plastic apron Gloves Protective sheet Receptacle to collect effluent Container for clean rectal washout solution Blanket to wrap baby Preparation Place all equipment at cot side Sedation is not necessary Wash hands, put on gloves and apron Position baby supine with legs raised Keep baby warm
PROCEDURE
Inspect and palpate abdomen note distension or presence of lumps Draw up 60 mL solution into syringe and keep on one side Insert lubricated catheter into rectum (up to approximately 10 cm or until resistance felt) noting any flatus or faecal fluid drained Massage abdomen in a clockwise direction to release flatus
Attach syringe containing solution to tube in rectum and gently instil fluid: Weight <2 kg Weight >2 kg 510 mL 20 mL
Disconnect syringe from tube and drain effluent into receptacle Repeat procedure until drained solution becomes clearer, up to a maximum of 3 times If solution does not drain out, manipulate tube in and out and massage abdomen If no faeces are passed or all the solution is retained, seek medical help Re-examine abdomen and note any differences Wash, dress and comfort baby
Preparation for discharge For discharge, baby should require no more than 2 rectal washouts a day Order equipment via paediatric community nurse Ward will supply 5 days equipment Discharge letter for GP detailing equipment required Arrange home visit with clinical nurse specialist in stoma care if available locally Ensure parents competent to perform rectal washout and can describe signs of colitis complete rectal washout parent competency sheet if available locally
INDICATIONS
Stoma output >30 mL/kg/day term baby (>20 mL/kg/day for preterm baby) Discrepancy in proximal and distal bowel calibre Poor nutrition Developing cholestasis Difficult venous access
BENEFITS
Maximise nutrition for sustained weight gain and decrease in parenteral nutrition Stimulation of gut hormones and enzymes Increases absorption of water, electrolytes and nutrients by utilising distal bowel Digestive tract matures and increases in length and diameter with use Adaptation is driven by enteral feed in distal bowel Preparation of distal bowel for closure Baby can, in some circumstances, be managed at home
CONTRAINDICATIONS
Integrity of distal bowel compromised Rectal bleeding (not absolute but discuss with surgical team) Anal stenosis or imperforate anus Signs of systemic infection Effluent too thick to infuse
ADVERSE REACTIONS
Bleeding from distal stoma Perforation of bowel by catheter (rare) Leakage of stoma effluent onto peristomal skin may result in excoriation of the skin Distress to baby and parent
Before commencing Discuss with surgical team and confirm they agree with procedure Confirm with surgical team whether a distal contrast study is required Consent Explain procedure to parents/family and obtain verbal consent Equipment Tube (enteral or Foley catheter) size 6 or 8 Fr Lubricating gel (if catheter not lubricated) Enteral syringe (60 mL) Stoma pot to collect stoma effluent Extension tubing Syringe pump (enteral pump if available) Plastic apron and gloves Tape and dressing Documentation Record name of surgeon requesting procedure in babys notes Record condition of peri-stomal skin pre-procedure Preparation Place all necessary equipment at cot side Wash hands and put on gloves and apron Position baby in supine position and keep warm
PROCEDURE
Pass lubricated catheter into mucus fistula up to 2 cm past end holes If using a Foley catheter put only 0.5 mL water into balloon Secure catheter to the abdomen with duoderm, tape and leave in situ Cover mucus fistula with paraffin gauze dressing (e.g. Jelonet) Collect stoma fluid from acting stoma into enteral syringe, connect to catheter via extension tube and start re-cycling using syringe pump Aim to infuse stoma loss over a few hours, but no more than 4 hr. Discard any effluent older than 4 hr If stoma loss <5 mL, re-cycle by syringe as a slow bolus over a few minutes Re-cycling should result in bowel actions per rectum of a consistency thicker than the stoma loss If bowel actions watery and/or frequent, send samples for culture and sensitivity, virology and detection of fat globules and reducing substances. Discuss with surgical team If baby develops signs suggestive of sepsis, stop procedure and perform septic screen as per unit policy. Discuss with surgical team When catheter is not in use, flush twice daily with 5 mL sodium chloride 0.9%
Preparation for home Liaise with neonatal surgical nurse Order equipment via paediatric community nurse Ward will supply 5 days equipment Discharge letter for GP detailing equipment required Arrange home visit with clinical nurse specialist in stoma care Inform surgical team before discharge
CONTRAINDICATIONS
Bleeding disorder Inadequate patency of ulnar artery on transillumination (if cannulating radial artery) or viceversa Pre-existing evidence of circulatory insufficiency in limb Local skin infection Malformation of upper extremity for radial arterial cannulation
Possible sites of arterial entry Radial (most commonly used): the only procedure discussed in this guideline Posterior tibial Dorsalis pedis Ulnar (usually only if ipsilateral radial artery cannulation has not been attempted)
EQUIPMENT
Gloves Cleaning solution as per unit policy 24-gauge cannula T-connector with Luer lock Adhesive tape Splint Sodium chloride 0.45% flush in 2 mL syringe, primed through T-connector Transillumination fibre-optic light source Three-way tap
Connect T-connector to infusion line (sodium chloride 0.45% with heparin 1 unit/mL), with three-way tap in situ for blood sampling
Documentation Document clearly in notes all attempts at cannulation, including those that are unsuccessful
AFTERCARE
Monitor Inspect distal digits regularly for circulatory status: if blanching does not recover after 5 min, remove line Avoid excessive hyperextension of wrist, as this can result in occlusion of artery Ensure a continuous pressure waveform tracing is displayed on monitor screen at all times: if flushing line does not restore lost tracing, change position of limb/dressing Usage Do not administer rapid boluses of fluid as this can lead to retrograde embolisation of clot or air: use minimal volume when flushing after sampling and inject slowly Use cannula only for sampling, and infuse only sodium chloride 0.45% with heparin 1 unit/mL Remove cannula as soon as no longer required Removal Aseptic removal of arterial line:, apply pressure for at least 5 min (longer if coagulopathy/low platelets, until no bleeding or bruising) dressings do not prevent bleeding or bruising do not send tip for culture routinely
COMPLICATIONS
Thromboembolism/vasospasm/thrombosis Blanching and partial loss of digits (radial artery) Necrosis Skin ulceration Reversible occlusion of artery Extravasation of sodium chloride infusate Infection (rarely associated with line infection) Haematoma Haemorrhage Air embolism
CONTRAINDICATIONS
Blood drawn from an arterial line may not be suitable for clotting studies (see Coagulopathy guideline and Bloodspot screening guideline)
COMPLICATIONS
Haemorrhage Ensure all connections are secure, Luer locks tight and 3-way taps appropriately adjusted Infection Maintain sterile technique during sampling to reduce risk of infection Artery spasm Limb appears blanched. Stop procedure and allow time for recovery. Warming of opposite limb can elicit reflex vasodilatation Thromboembolism Flush catheter with 0.5 mL sodium chloride 0.45% each time sample taken. If catheter not sampling, clot formation may be in progress. Request urgent registrar review of arterial line for a prompt decision about removal Inaccuracy of blood gas results Analyse sample immediately. After blood is withdrawn from an artery, it continues to consume oxygen Excess heparin in syringe can result in a falsely low pH and PaCO2. Remove excess heparin from syringe before obtaining sample Do not use if air bubbles in sample: take fresh specimen
EQUIPMENT
Gloves Paper towel Alcohol swabs x 2 Syringes 2 mL syringe (A) for clearing line 2 mL syringe (B) for other blood samples as necessary 1 mL syringe (C) pre-heparinised for blood gas analysis 2 mL syringe (D) containing 0.51 mL heparinised sodium chloride 0.45% Appropriate blood sample bottles and request forms
PROCEDURE
Preparation Record SpO2 and TcCO2 at time of taking blood to allow comparison with blood gas Wash hands and put on gloves Place paper towel beneath 3-way tap collection port (maintain asepsis by non-touch technique rather than sterile gloves and towel) Ensure 3-way tap closed to port hole
Procedure Remove Luer lock cap, clean with alcohol swab and allow to dry, or prepare bioconnector Connect 2 mL syringe (A) Turn 3-way tap so it is closed to infusion and open to syringe and arterial catheter Withdraw 2 mL blood slowly. It must clear the dead space If bioconnector not being used, turn 3-way tap so it is closed to arterial catheter to prevent blood loss from baby if bioconnector used, do not turn 3-way tap until end of procedure Attach appropriate syringe (B/C) needed for required blood sample If bioconnector not being used, turn 3-way tap to open to syringe and arterial catheter and withdraw required amount of blood for blood samples. Do not withdraw more than required amount If bioconnector not being used, turn 3-way tap off to arterial catheter in between syringes B and C if both required, after taking required samples with syringes Reattach syringe (A) Clear the connection of air Slowly return to baby any blood in line not required for samples If bioconnector not being used, turn 3-way tap off to arterial catheter Attach syringe (D) of heparinised sodium chloride 0.45% If bioconnector not being used, turn 3-way tap so it is open to syringe and arterial line, clear line of air and slowly flush line to clear it of blood Turn 3-way tap so it is closed to syringe, remove syringe (D), swab port hole with alcohol wipe and cover with Luer lock cap Record amount of blood removed and volume of flush on babysdaily fluid record
AFTERCARE
Ensure all connections tight and 3-way tap turned off to syringe port to prevent haemorrhage If sampling from umbilical arterial catheter (UAC), ensure lower limbs are pink and well perfused on completion of procedure If sampling from peripheral arterial line, check colour and perfusion of line site and limb housing arterial line Ensure line patency by recommencing infusion pump Before leaving baby, ensure arterial wave form present and all alarms set
Cannulation 201315
CANNULATION
PERIPHERAL VENOUS CANNULATION INDICATIONS
Access for intravenous infusion and medications
CONTRAINDICATIONS
Sore or broken skin
EQUIPMENT
Cleaning solution (see your Trusts policy) Appropriately labelled blood bottles and request cards Non-sterile disposable gloves 24 gauge cannula T-piece connected to a syringe of sodium chloride 0.9%, flushed and ready Tape and splint to secure cannula 3-way tap if necessary EMLA cream is not used in neonates
PROCEDURE
Preparation Identify suitable site: prefer back of hand or foot save long saphenous and antecubital fossa veins for long line insertion scalp: shave area if using scalp vein (do not use as first priority site) inform parents before procedure if possible Identify suitable vein, which should be clearly visible. Unlike in adults, neonatal veins are rarely palpable When baby likely to need numerous cannulations, avoid using potential long line veins It can be helpful to flush cannula with sodium chloride 0.9% to assist in identification of point at which cannula enters vein. If blood samples taken at time of cannula insertion, do not flush cannula as this will contaminate sample for analysis Wash hands and put on gloves
Insertion Apply hand pressure around limb to distend vein Place thumb on skin slightly distal to proposed puncture site Hold cannula at 1020O angle and puncture skin Advance cannula toward vein resistance may diminish slightly as it enters vein and a speck of blood may be seen in hub of needle (this is easier to see if cannula has been flushed with sodium chloride 0.9%). Do not advance needle further as it can pierce back wall of vein When this occurs, hold needle steady and advance cannula a short distance within vein Withdraw needle from cannula Connect T-piece and flush cannula gently with 0.5 mL sodium chloride 0.9% to confirm it is in the vein Secure cannula with clear dressing (e.g. Tegaderm/Opsite) to ensure IV site visible at all times, and connect to infusion Documentation Record date and time of cannula insertion in notes with identification and signature of person carrying out procedure If multiple cannulations, record date and time of removal of cannula Use visual phlebitis scoring for ongoing monitoring of cannula, according to local Trust policy
EQUIPMENT
Sterile dressing pack Cleaning solution as per unit policy suggest aqueous chlorhexidine 2%/isopropyl alcohol 70% (Chloraprep) and wash off with sodium chloride 0.9% once dried for babies <26 weeks gestation Lidocaine 1%, with syringe and needle for preparation and injection Chest drains size FG 8,10,12 (use largest possible depending on size of baby) Low pressure suction unit Scalpel and fine straight blade (size 11) Fine blunt forceps Underwater seal chest drainage bottle and tubing or flutter (Heimlich) valve Steristrips and transparent dressing (e.g. Opsite/Tegaderm)
SITES
Site of insertion depends on position of pneumothorax preferred site is in anterior axillary line, between fourth and sixth intercostal space, to conceal subsequent scarring and avoid interference with breast development alternative site is just lateral to midclavicular line, in second or third intercostal space if pneumothorax does not drain satisfactorily, it may be necessary to insert more than one drain for pleural effusion, use midaxillary line between fourth and fifth intercostal spaces, and direct drain posteriorly
PROCEDURE
Preparation and position of baby Inform parents and obtain verbal consent as recommended by BAPM (unless emergency procedure) Use 1012 FG pleural catheter (small babies may need 8 FG) Position baby supine and flat with affected side slightly tilted up (for example, by using a folded blanket) Prepare skin with full aseptic technique Infiltrate with lidocaine 1%, even in babies being given systemic analgesia Insertion of tube Make small incision in skin with scalpel at lower edge of intercostal space to avoid injury to intercostal vessels Dissect bluntly with fine forceps through intercostal muscle and pleura Use fine forceps to gently advance tip of catheter Push and twist tube gently through incision into pleural space Insert chest drain 23 cm for small preterm and 3 cm for term babies Use of trocar not generally recommended. If used (in bigger baby), protect lung by clamping artery forceps onto trocar 1 cm from the tip Connect tube to prepared underwater seal or flutter (Heimlich) valve (according to local practice) Manipulate tube gently so that tip lies anteriorly in thoracic cavity for pneumothorax, and posteriorly for effusion
Secure tube with Steristrips, and cover with gauze dressing. A suture may be required; do not use purse-string suture Secure tube to chest wall using suitable tape (Opsite or Tegaderm)
AFTERCARE
Check bubbling or oscillation of water column seen with every inspiration Check tube position with chest X-ray (consider lateral x-ray to confirm position)
Suction If bubbling poor and X-ray confirms drain is in correct position but pneumothorax not fully draining on X-ray or cold light, apply continuous suction of 510 cm of water. Thoracic suction is better suited for this purpose than routine wall suction. Occasionally, a second drain may be necessary Flutter valve As an alternative to underwater chest drain system, especially during transport, a flutter valve can be used Document Record presence of bubbling (continuous/intermittent/none) in nursing care chart Record with nursing observations, bubbling and/or oscillation of water column, or fluttering of valve seen with every inspiration
Consent 201315
CONSENT
FOR COMMON NEONATAL INVESTIGATIONS, INTERVENTIONS AND TREATMENTS
The following guidance is taken from 'Good practice framework for consent in neonatal clinical care' produced by the British Association of Perinatal Medicine (BAPM) It is a legal and ethical requirement to gain valid consent before examining and initiating any investigation or treatment for any patient Consent is obtained from someone with parental responsibilities: if married, parents if not married, mother but not father, unless father has acquired parental responsibility via a court order, being registered on birth certificate or parental responsibility agreement a legally appointed guardian a local authority designated in a care order or holding an emergency protection order Consent is valid only when information has been understood by the parent(s) and explains why the intervention is recommended, its risks and implications, and other options should consent be withheld Documentation of information given and parent(s) understanding and agreement to proceed is the most important validation of consent. A signature does not in itself confirm informed consent Witness consent wherever possible, and record name of witness In neonatal practice, there are frequent occasions when no one is available to provide valid consent and treatment is initiated in its absence (e.g. emergency ABC resuscitation, stabilisation, chest drainage or exchange transfusion when delayed treatment would not be in the babys best interests, or following maternal general anaesthetic when mother is unmarried to babys father). It should always be possible later to justify the action to the parents and to reassure them that it was in the babys best interests
GOOD PRACTICE
Give parents of babies admitted to neonatal unit written information (BLISS booklet http://www.bliss.org.uk/help-for-families/in-hospital/common-tests-procedures/) describing low-risk procedures such as venesection, for which explicit consent is not normally sought Give parents information leaflet for data collection, allowing them to opt out
Written explicit consent Purpose and risks of an intervention are formally explained and consent obtained and recorded before the intervention Table 1: Explicit consent (recorded in patient notes, and supported by a signature) is required for: Investigation/intervention Clinical photographs and videorecordings Any biopsy or aspiration Exchange transfusion Treatment for retinopathy Surgical procedures Post-mortem
Use consent form specific for this purpose For example: skin, liver, bone marrow Obtained by ophthalmologist Consent taken by surgical team. If telephone consent required and mother still an in-patient, midwife on postnatal ward or neonatal team to act as witness See Death guideline and use specific form. Usually obtained through consultant or senior middle grade staff
Consent 201315
Table 2: Explicit oral consent Explicit consent as defined above, documented, but not supported by a signature, required for the following: Explicit oral consent Investigations Screening baby and/or mother in high-risk situations with no knowledge of maternal status (e.g. HIV, substance misuse) Genetic testing Gut imaging involving contrast MR/CT imaging Practical procedures Therapeutic lumbar puncture (LP) or ventricular tap in absence of reservoir* Peripherally-placed long lines* Brachial or femoral arterial line Chest drain insertion/replacement* Abdominal drainage for perforation or ascites* Irrigation following extravasation* Hearing screening Immunisation See Immunisations guideline Treatments Vitamin K for normal term babies Nitric oxide Postnatal steroids for chronic lung disease Use of donor breast milk Transport Emergency transfers Routine transfers for out-patients or back-transfers NB: Initiation of cooling for neuroprotection does not require explicit consent, but transfer to another unit for formal cooling does
* It is accepted that, in some circumstances, these procedures are performed in an emergency in babys best interests and may be performed without oral consent; owing to risks associated with procedures or conditions in which they are necessary, it is considered best practice to inform parents as soon as possible and to document this in babys notes Others: Implicit consent Where the nature and risk of the procedure is such that a less formal transfer of information is considered sufficient, and is often retrospective List of investigations, procedures and treatments is long, see Table 3 If unsure, seek senior advice Explain all investigations, procedures and treatments to parents at earliest opportunity
Consent 201315
Table 3: Implicit consent Implicit consent Examination and investigations Practical procedures Examining and assessing baby Routine blood sampling Septic screen Diagnostic LP (possible infectious or metabolic illness) Suprapubic aspiration of urine Screening for infection in response to positive results of maternal screening (e.g. known maternal HIV or substance abuse) CMV, toxoplasmosis, rubella and herpes screening X-ray and ultrasound ECG Retinopathy of prematurity (ROP) screening Umbilical line insertion Percutaneous arterial lines (radial, posterior tibial only) Peripheral venous lines Nasogastric tube insertion Tracheal intubation Ventilation/CPAP Urethral catheterisation Blood transfusion Use of pooled blood products e.g. FFP Partial exchange transfusion Antibiotics Vitamins/minerals Surfactant Anticonvulsants Sedation for intubation and ventilation Inotropes Indomethacin or ibuprofen for patent ductus arteriosus (PDA) Prophylactic indomethacin Postnatal dexamethasone for laryngeal oedema
Treatments: drugs
Nutrition/fluids
DOCUMENTATION
Documentation, supported by a signature for written explicit consent Documentation of oral explicit consent Provide parents with information sheets
EXCHANGE TRANSFUSION
Exchange transfusion replaces withdrawn baby blood with an equal volume of donor blood Discuss all cases with neonatal consultant
INDICATIONS
Anaemia A newborn who has not had an in-utero transfusion (IUT) with a cord Hb <120 g/L and is haemolysing, may require urgent exchange transfusion to remove antibodies and correct anaemia: if Hb <100 g/L, discuss urgently with consultant and proceed to exchange transfusion; avoid simple packed cell transfusions if Hb 100120 g/L, obtain 6-hrly bilirubin values and, if rapidly rising or close to exchange transfusion level, see Table in Jaundice guideline, use intravenous immunoglobin (IVIG) A newborn who has had IUTs and whose Kleihauer test (this test may not be available in your hospital) demonstrates a predominance of adult Hb, anaemia can be managed using a top-up transfusion of irradiated, CMV-negative blood Hyperbilirubinaemia Discuss promptly with consultant. If bilirubin values approaching guidance below; senior decision is required: guidance as determined by exchange transfusion line on gestation-specific NICE jaundice chart See Table in Jaundice guideline if bilirubin rises faster than 8.5 micromol/L/hr despite phototherapy, anticipate need for exchange transfusion Chronic feto-maternal transfusion Disseminated intravascular coagulation (DIC) NB: cord bilirubin acts as a reference to compare with 6 hr bilirubin. There is no current cord bilirubin concentration that would indicate immediate need for exchange transfusion
COMPLICATIONS
Cardiac arrhythmias Air embolism Necrotising enterocolitis Coagulopathy Apnoeas and bradycardia Sepsis Electrolyte disturbances Acidosis owing to non-fresh blood Thrombocytopenia Late hyporegenerative anaemia
PROCEDURE
Prepare Ensure full intensive care space and equipment available and ready Allocate one doctor/practitioner and one other member of nursing staff, both experienced in exchange transfusion, to care for each baby during procedure; document their names in babys notes Obtain written consent when possible, and document in babys notes Phototherapy can usually be interrupted during exchange
Calculate volume of blood to be exchanged: 160 mL/kg (double blood volume) removes 90% of baby red cells and 50% of available intravascular bilirubin Order appropriate volume (usually 2 units) of blood from blood bank, stipulating that it must be: crossmatched against mothers blood group and antibody status, and (if requested by your blood bank) babys blood group CMV-negative irradiated (shelf-life 24 hr) for any baby who has had an in-utero blood transfusion as fresh as possible, and certainly no more than 4 days old plasma reduced red cells for exchange transfusion (haematocrit 0.50.6), not SAG-M blood and not packed cells
Prepare baby Empty stomach using nasogastric tube (see Nasogastric tube insertion guideline) Start intravenous infusion and allow nil-by-mouth Pay attention to thermoregulation, particularly if procedure to be performed under radiant heater Commence continuous cardiac, temperature and saturation monitoring Monitor and document Blood pressure and heart rate every 15 min throughout exchange If any change in babys cardiorespiratory status, pause exchange by priming catheter with donor blood that will not clot. Discuss with consultant Prepare blood Set up blood warmer early (aim for 37OC) Check blood units as per hospital policy Connect donor blood to filter and prime blood giving set Connect to 4-way (if using UVC) or 3-way tap (outside the warmer) as indicated Ensure donor blood well mixed before and throughout exchange Technique Ensure working area sterile Either Insert UVC (see Umbilical venous catheterisation guideline) and confirm position. Use UVC push-pull technique below for exchange Or Insert peripheral venous (in route) and arterial (out route) catheters. Use peripheral venous and arterial catheters continuous technique below for exchange Or Insert UVC (in route) and UAC (out route) and confirm position. Use umbilical venous and arterial continuous technique below for exchange UVC push-pull technique Connect catheter bag (using Vygon connector) and donor blood to 4-way tap and 4-way tap to UVC Remove 10 mL baby blood from UVC using syringe Send first sample for serum bilirubin, full blood count, blood culture, blood glucose, calcium, electrolytes, coagulation and liver function tests when exchange performed for reasons other than known blood group antibodies, send blood for G6PD screening and viral serology Replace precise volume removed with donor blood, slowly using a syringe
Each out-in cycle should replace no more than 8.5 mL/kg and take at least 5 min; start with smaller aliquots (10 mL) and increase to 20 mL (if baby stable and weight allows) only after 30 min. As a guide: birth weight <1000 g: use 5 mL aliquots birth weight 10002000 g: use 10 mL aliquots birth weight >2000 g: use 20 mL aliquots Discard out baby blood into catheter bag Continue out-in cycles every 5 min (maximum aliquot with each cycle) until complete Send last out baby blood sample for serum bilirubin, full blood count, blood culture, blood glucose, calcium and electrolytes
Peripheral venous and arterial catheters continuous technique Connect catheter bag, using Vygon connector, to 3-way tap attached to arterial line extension Never leave arterial line open to catheter bag Connect donor blood to venous catheter Remove 10 mL of babys blood from arterial line and send for tests as listed above under UVC push-pull technique Start venous infusion at rate to match withdrawal rate e.g. 120 mL/hr for a 10 mL volume withdrawal every 5 min Remove out aliquots of babys blood from arterial line every 5 min to match volume of donor blood being infused into venous line Observe limb distal to arterial line at all times and document appearance. If concerned, pause exchange and discuss with consultant Continue steps as above but note that continuous in cycle requires removal of out aliquots only every 5 min If exchange stopped for >23 min, discontinue procedure and ensure all lines are flushed
UVC and UAC continuous technique Use UVC as in line and UAC as out line and proceed as with Peripheral venous and arterial continuous technique
AFTERCARE
Immediate When Hb and bilirubin in final out sample known, check with consultant before removing all lines Complete documentation (volumes in/out, and all observations) Recommence phototherapy Recommence feeds 46 hr after completion Monitor blood sugar 4-hrly until acceptable on 2 consecutive occasions Update parents Intermediate In babies receiving antibiotics, a repeat dose may be required: discuss with consultant Delayed Guthrie spot collection will be indicated, as directed by regional centre Follow-up Neuro-developmental follow-up in all babies who have undergone exchange transfusion Repeat full blood count at intervals (likely 12 weekly but to be determined individually) for up to at least 6 weeks, to detect anaemia secondary to ongoing haemolysis
Extravasation 2013-15
EXTRAVASATION INJURIES
BACKGROUND
Approximately 4% of babies develop skin necrosis as a result of extravasation of an IV infusion A small proportion of these babies develop long-term cosmetic or functional compromise Extravasation may be due to: cannula piercing the vessel wall or from distal venous occlusion causing backpressure within the vessel Peripheral and central venous catheters are both capable of causing extravasation A Cochrane review shows that centrally placed catheters undergo extravasation as frequently as peripheral Use this guideline to define the grading and management of extravasation injuries for babies Limiting the IV pump cycle to 1 hr may minimise the extent of tissue damage from extravasation providing the entry site is observed concurrently The degree of tissue damage due to extravasation is dependent upon: volume of infusate, its pH and osmolality the dissociation constant and pharmacological action of any drug(s) being infused For examples of commonly used solutions with the potential to cause skin necrosis, see Table 1
Table 1 Alkali solutions Phenytoin pH 12 Amphotericin pH 8 Tham Bicarbonate Acidic solutions TPN solutions N1, N2 and N3 pH 6 Sodium chloride 0.9% pH 5 Dopamine pH 2.54.5 Hyperosmolar solutions Potassium chloride Calcium chloride TPN (N2 and N3) X-ray contrast (e.g. Ultravist)
Wound dressings When choosing wound dressing, consider the need to prevent: further trauma epidermal water loss contractures by allowing a full range of limb movements Dressings must be: easy to apply to small body surface areas sterile suitable for use in humidified/incubator environments Most commonly used dressings Hydrocolloid 9 (e.g. Duoderm) or Hydrogel (e.g. Intrasite gel, Intrasite conformable) if in doubt, seek advice from tissue viability nurse
ASSESSMENT
Table 2: Grading of extravasation injuries Grade 1 Pain at infusion site Grade 2 Pain at infusion site Swelling No skin blanching Normal capillary refill and peripheral pulsation Grade 3 Pain at infusion site Swelling Skin blanching Cool blanched area Normal capillary refill and peripheral pulsation Grade 4 Pain at infusion site Swelling Skin blanching Cool blanched area Reduced capillary refill +/- arterial occlusion +/- blistering
Extravasation 2013-15 Investigations No specific investigations required. However, if wound appears infected: wound swab FBC CRP blood culture start appropriate antibiotics follow local practice
ACUTE MANAGEMENT
Table 3 Grade 1 and Grade 2 Stop infusion Remove cannula and splints/tapes Elevate limb Stop infusion Remove cannula and splints/tapes Elevate limb Grade 3 Stop infusion Leave cannula in situ until review by doctor/ANNP Consider irrigation of affected area Remove constricting tapes Elevate limb Inform tissue viability nurse Grade 4 Stop infusion Leave cannula in situ until review by doctor/ANNP Photograph lesion providing no delay in further treatment Irrigate affected area Elevate limb Inform tissue viability nurse/registrar/ consultant +/- plastic surgery team
Most extravasation injuries are of Grades 1 and 2 and do not require extensive intervention Grade 3 and 4 injuries have a greater potential for skin necrosis, compartment syndrome and need for future plastic surgery, depending on type of solution extravasated
FURTHER ASSESSMENT
Following irrigation treatment, review all injuries within 24 hr of extravasation occurring Irrigation of major grades of extravasation has been used to prevent extensive skin loss and need for plastic surgery and skin grafting. However, the evidence for the use of irrigation in preventing long-term injury is limited
Other considerations Family-centred care Inform parents of extravasation injury and management plan Special considerations Infection control observe standard infection control procedures Complete an incident report for Grade 3 and 4 extravasations
Extravasation 2013-15 Massage out any excess fluid using gentle manipulation Cover with paraffin gauze for 2448 hr
When irrigating with sodium chloride 0.9%, use discretion depending on babys weight Documentation Person performing procedure must document in babys medical record
INDICATIONS
Total/partial parenteral nutrition Concentrated (>10%) glucose infusions Infusions of glucose >5% + calcium gluconate Inotrope infusions Prolonged drug or fluid administration where peripheral access difficult
CONTRAINDICATIONS
Infection at proposed insertion site Systemic sepsis: defer until sepsis treatment commenced and blood cultures negative Tissue perfusion concerns
EQUIPMENT
Sterile gown and sterile gloves Cleaning solution as per unit policy Sodium chloride 0.9% for injection Tape measure Overhead light Neonatal long line appropriate for size of baby and expected rate of infusion Long line insertion pack or, if not available, individual items to include: dressing pack with swabs and plastic dish sterile towels/sheets non-toothed forceps 510 mL syringe Steristrips sterile scissors clear dressing (e.g. Tegaderm/Opsite)
PROCEDURE
Must be performed or directly supervised by an individual competent in the insertion of these devices Consent and preparation Inform parents and obtain verbal consent as recommended by BAPM Discuss timing of procedure with nurses Keep baby warm. Work through portholes Identify site of insertion typically long saphenous at ankle or medial/lateral antecubital vein at elbow where access difficult, other large peripheral veins or scalp veins anterior to ear may be used Measure distance, aiming to insert tip of catheter into superior or inferior vena cava (to xiphisternum for lower limb insertion, to upper sternum for upper limb insertion) Analgesia Unless contraindicated, give sucrose Second person to provide containment holding see Pain assessment and management guideline
Aseptic insertion Maintain strict asepsis throughout Prime catheter and cut small piece of gauze for under hub Clean site and allow to dry. Ensure that cleaning fluid does not pool beneath baby Puncture site with needle from pack and follow instructions for that catheter Avoid use of cannulae for long line insertion When blood flows back through the needle, insert line using non-toothed forceps If appropriately placed, the line will pass easily beyond the tip of the needle There may be some resistance when the line passes joints, such as knee, and gentle repositioning of babys limb may help When in place, withdraw needle as stated in catheter instructions Securing catheter in correct position When haemostasis achieved, fix with Steristrips. Place small piece of gauze under hub, and cover with Tegaderm/Opsite, making sure that all dressing and site is covered, but not encircling the limb tightly. Ensure line insertion site is visible through clear dressing Connect a sterile 5 mL syringe containing sodium chloride 0.9% and infuse at 0.5 mL/hr, while awaiting X-ray, to ensure that the line does not clot off X-ray to determine position Small gauge neonatal long lines can be difficult to see on plain X-ray use X-ray magnification, contrast adjustment and inversion to aid process use of a catheter with integral wire stylet or use of contrast medium can help if using contrast medium, refer to local policy o If inserted in upper limb, ensure arm is at 90 angle to thorax during X-ray Determine satisfactory position Catheter tip should preferably be in superior vena cava (SVC) or inferior vena cava (IVC) outside heart. Other large veins e.g. innominate, subclavian, iliac are acceptable Catheter tips in axillary, cephalic and femoral veins are acceptable if the benefit outweighs increased risks of reinsertion Monitor site closely If catheter tip beyond desired location, using aseptic technique, remove dressing and, withdraw catheter the measured distance. Redress with new sterile dressing and confirm new position by X-ray If present, remove wire stylet Catheter tip must not lie within heart (risk of perforation and tamponade) Failure of insertion If second operator is required following an unsuccessful attempt at placement, use fresh equipment
DOCUMENTATION
Record in case notes: date and time of insertion success of insertion and number of attempts type and gauge of catheter site and length of insertion X-ray position and alterations Insert tracking stickers from all packs
AFTERCARE
Dressings and site care Routine dressing changes are unnecessary
Long lines 201315 Replace aseptically only if dressings lift or catheter visibly kinked or becomes insecure Observe site every shift for bleeding, leaking of infusate and signs of infection (redness, swelling)
Line management and medication Minimise number of line breaks Intermittent medications only given via this route in extreme circumstances. (This is a senior medical decision). Plan timing to match infusion changes When breaking into line, observe hand hygiene, wear sterile gloves and clean connection as per local infection control policy Change tubing used to give blood products immediately after transfusion (use to give blood product only if it is difficult to insert alternative IV line) Position maintenance Repeat X-ray weekly to detect line migration Never routinely resite a line Review continued need on daily ward rounds and remove as soon as possible
COMPLICATIONS
Prevention Do not give blood products and medications routinely through long line Never use 1 or 2 mL syringes as catheter can rupture Limit line breaks as above Catheter-related sepsis Commonest complication Most common organism is coagulase-negative staphylococcus (CoNS) Signs: temperature instability bradycardia desaturation apnoea hypotension raised WCC and CRP low platelets coagulopathy If baby stable, leave line in situ, take blood culture, give gram negative and CoNS antibiotic cover while awaiting results. Consider administration of antibiotics via the long line If blood culture confirms CoNS and baby stable, discuss long line removal with senior colleague Continue antibiotics If baby unstable, remove long line If blood cultures grow candida, Staphylococcus aureus or Gram negative organisms, remove line immediately Suspected/proven pericardial tamponade Suspect if any of the following symptoms: acute or refractory hypotension acute respiratory deterioration arrhythmias tachycardia unexplained metabolic acidosis Confirm by X-ray (widened mediastinum, enlarged cardiac shadow) or by presence of pericardial fluid on echocardiogram
Extravasation of fluids Into pleural, peritoneal, pericardial (above) and subcutaneous compartments Seek immediate advice from senior colleagues and follow Extravasation injuries guideline Embolisation of catheter fragments Lines can snap if anchored within a thrombus If undue resistance encountered during removal, do not force Inform your consultant: if accessible it may need surgical removal
REMOVAL
Indications Clinical use is no longer justified Remove 24 hr after stopping total parenteral nutrition (TPN) to ensure tolerance to full enteral feeds, running glucose 10% through line at 0.5 mL/hr to maintain patency Complications see Complications Technique Using aseptic technique: remove adhesive dressing very carefully pull line out slowly, using gentle traction in the direction of the vein, grasping line not hub ensure catheter complete if clinical suspicion of line infection, send tip for culture and sensitivity apply pressure to achieve haemostasis document removal in notes
Evidence Level: V What is the optimum position and the best way of determining the position once placed? The tip of silastic catheters should be placed just behind the confluence of the superior-inferior vena cava and the right atrium (Hausdorf, 1987), or alternatively in the superior vena cava (Anon, 1991). There is an increased risk of systemic air and fat embolism if the tip is in or close to a patent foramen ovale (Hausdorf, 1987). Ultrasonography accurately confirms positioning of even the thinnest catheters, and reduces the need for radiography (Soong, 1991; Hausdorf, 1987, De Carvalho 2012).
Anon. Central venous access in children. Lancet 1991;338:1301-2 Hausdorf G, Bitzan M, Commentz J, et al. Intra-atrial malpositions of silastic catheters in newborns. Crit Care Med 1987;15:308-9 Soong WJ, Hsieh KS, Tiu CM, et al. Central venous silastic catheters in newborns and children: localization by sonography and radiology. Zhonghua Yi Xue Za Zhi (Taipei) 1991;48:97-102 De Carvalho, OPS, Da Luz, GPM, Peterlini, MA. Placement of peripherally inserted central catheters in children guided by ultrasound: a prospective randomized, and controlled trial. 2012; 13:282-7.
Evidence Level: V How should any infections be managed? When should the lines be removed? Catheter infection is more than twice as common in neonates than in older children (Mulloy, 1991), and infection rates as high as 45% have been recorded (Puntis, 1990; Grisoni, 1986). Exitsite infections can be treated with antibiotics, but tunnel infections usually require the catheter to be removed (Anon, 1991). A prospective study of 35 patients (Klein, 1992) included 4 with bacteraemia. Two of these needed catheter removal to clear their infections, but the other 2 were cured by the administration of antibiotics through the catheter. Prophylactic vancomycin or teicoplanin reduces the incidence of catheter-related infection in neonates (Moller, 1995). However, a Cochrane review (Jardine, 2008) found that antibiotic prophylaxis had no effect on overall mortality (RR 0.68, 95% CI 0.31 1.51).
Anon. Central venous access in children. Lancet 1991;338:1301-2
Grisoni ER, Mehta SK, Connors AF. Thrombosis and infection complicating central venous catheterization in neonates. J Pediatr Surg 1986;21:772-6 Jardine LA, Inglis GD, Davies MW. Prophylactic systemic antibiotics to reduce morbidity and mortality in neonates with central venous catheters. The Cochrane Database of Systematic Reviews 2008, Issue 1. CD006179 Klein JF, Shahrivar F. Use of percutaneous silastic central venous catheters in neonates and the management of infectious complications. Am J Perinatol 1992;9:261-4 Moller JC, Reiss I, Schaible T. Vascular access in neonates and infants: indications, routes, techniques and devices, complications. Intensive Care World 1995;12:48-53 Mulloy RH, Jadavji T, Russell ML. Tunneled central venous catheter sepsis: risk factors in a pediatric hospital. JPEN J Parenteral Enteral Nutr 1991;15:460-3 Puntis JW, Holden CE, Smallman S, et al. Staff training: a key factor in reducing intravascular catheter sepsis. Arch Dis Child 1990;65:335-7
Evidence Level: V What are the most common complications and how can they be avoided? The most common serious complications of vascular access are infection (Moller, 1995) and catheter occlusion secondary to thrombus formation at the catheter tip (Sherman, 1983). The first prospective study of silastic catheters in neonates, using only peripheral veins of the scalp and extremities (Durand, 1986), found an overall incidence of mechanical complications of 26.4%. These consisted of blockage of the catheter or accidental displacement. Of 53 catheterisations, 4 (7.5%) were complicated by infections. A study of 535 catheterisations with an average indwelling time of 23 days (Neubauer, 1995) noted one complication for every 153 indwelling catheter days. The most common complication was sepsis, on 22 occasions (4.1%). A large Chinese study of 1,318 catheterisations (Soong, 1995) found a lower rate of sepsis (2.7%), which was still the most common complication. The jugular vein route is particularly associated with thrombosis, which was detected in 8 of 24 patients receiving a silastic catheter in a study of 40 neonates (Rand, 1994). Lines in infants needing total parenteral nutrition or multiple intravenous infusions are particularly susceptible to infection and their use should be avoided if possible (Mulloy, 1991). Broviac catheters may be more suitable in these patients (Anon, 1991). A prospective study of catheter sepsis (Puntis, 1991) found that education of staff in appropriate practice and the utilisation of specialist nurses reduced the rate of infection from 45% to 8% over a 12 month period. A randomised, controlled, double-blind, single-centre trial in 210 infants (Birch, 2010) compared TPN with heparin (n=102) to TPN without heparin (n=108). There was a statistically significant reduction in all episodes of culture-positive, catheter-related sepsis in those infants with heparin added to the TPN, compared with those without heparin (p=0.04; RR 0.57, 95% CI 0.32 to 0.98; NNT 9, 95% CI 4.6 to 212.4). A Cochrane review of 2 trials in 267 neonates (Shah, 2008) found some evidence for the prophylactic use of heparin in prevention of thrombotic complications; treatment was associated with a reduced risk of catheter occlusion (RR 0.28, 95% CI 0.15 0.53; NNT 5, 95% CI 3 8). The only previous systematic review (Randolph, 1998) was not confined to infants.
Anon. Central venous access in children. Lancet 1991;338:1301-2 Birch P; Ogden S; Hewson M. A randomised, controlled trial of heparin in total parenteral nutrition to prevent sepsis associated with neonatal long lines: the Heparin in Long Line Total Parenteral Nutrition (HILLTOP) trial. Arch Dis Child Fetal Neonat Ed 2010;95:F252-7 Durand M, Ramanathan R, Martinelli B, et al. Prospective evaluation of percutaneous central venous silastic catheters in newborn infants with birth weights of 510 to 3,920 grams. Pediatrics 1986;78:245-50
Moller JC, Reiss I, Schaible T. Vascular access in neonates and infants: indications, routes, techniques and devices, complications. Intensive Care World 1995;12:48-53 Mulloy RH, Jadavji T, Russell ML. Tunneled central venous catheter sepsis: risk factors in a pediatric hospital. JPEN J Parenteral Enteral Nutr 1991;15:460-3 Neubauer AP. Percutaneous central i.v. access in the neonate: experience with 535 silastic catheters. Acta Paediatr 1995;84:756-60 Puntis JW, Holden CE, Smallman S, et al. Staff training: a key factor in reducing intravascular catheter sepsis. Arch Dis Child 1991;66:335-7 Rand T, Kohlhauser C, Popow C, et al. Sonographic detection of internal jugular vein thrombosis after central venous catheterization in the newborn period. Pediatr Radiol 1994;24:577-80 Randolph AG, Cook DJ, Gonzales CA, et al. Benefit of heparin in peripheral venous and arterial catheters: systematic review and meta-analysis of randomised controlled trials. BMJ 1998;316:969-75 Shah PS, Shah VS. Continuous heparin infusion to prevent thrombosis and catheter occlusion in neonates with peripherally placed percutaneous central venous catheters. The Cochrane Database of Systematic Reviews 2008, Issue 2. Art. No.: CD002772 Sherman MP, Vitale DE, McLaughlin GW, et al. Percutaneous and surgical placement of fine silicone elastomer central catheters in high-risk newborns. JPEN J Parenteral Enteral Nutr 1983;7:75-8 Soong WJ, Jeng MJ, Hwang B. The evaluation of percutaneous central venous catheters: a convenient technique in pediatric patients. Intensive Care Med 1995;21:759-65
Evidence Level: V Last amended August 2013 Last reviewed August 2013
Neonatal Resuscitation
Prepared By:
Dr . Sami Sayegh
Neonatal Resuscitation The NRP has been carefully designed in a modular fashion, with each module emphasizing a particular skill vital for a successful resuscitation. The modules represent the progressive steps that would occur during a full resuscitative effort of a severely depressed neonate. The modules are as follows: 1. 2. 3. 4. 5. 6. Preparation for delivery Initial stabilization Ventilation - bag and mask Chest compressions Endotracheal intubation Medications
A nurse might be trained up to and including chest compressions and how to assist with endotracheal intubation, while physicians would be trained to provide all resuscitative steps. For either nurse or physician, the instructional material is the same for each module, thus ensuring a common ground for good communication and teamwork during an actual resuscitation. Each resuscitation should go through A,B,C, D A : means airway. B: means brething. C : means circulation . D : means drugs.
Asphyxia - The Basics Apnea 1. The asphyxiated infant passes through a series of events: o rapid breathing and fall in heart rate o primary apnea o irregular gasping, further fall in heart rate and drop in blood pressure o secondary apnea 2. Most infants in primary apnea will resume breathing when stimulated. Once in secondary apnea, infants are unresponsive to stimulation. 3. Apnea at birth should be treated as secondary apnea of unknown duration (i.e. began in utero) and resuscitation should begin at once. Clearing Fetal Lung Fluid 1. The first few breaths of a normal infant are usually adequate to expand the lungs and clear the alveolar lung fluid. 2. The pressure required to open the alveoli for the first time may be two to three times that for normal breaths. 3. Expect problems in lung fluid clearance with: o apnea at birth o weak initial respiratory effort caused by: prematurity depression by asphyxia, maternal drugs, or anaesthesia Pulmonary Circulation 1. At birth, pulmonary blood flow increases rapidly as the lung arterioles open up and blood is no longer diverted through the ductus arteriosus. 2. With asphyxia, hypoxemia and acidosis perpetuate pulmonary vasoconstriction and maintain the fetal pattern of circulation.
Systemic Circulation and Cardiac Function 1. Early in asphyxia, vasoconstriction in the gut, kidneys, muscles and skin redistributes blood flow to the heart and brain as an attempt to preserve function. 2. With progressive hypoxemia and acidosis, myocardial function deteriorates and cardiac output declines.
Preparation for Delivery Anticipate Need for Resuscitation 1. Antepartum and intrapartum history may help to alert deliveryroom staff about the possibility of a depressed or asphyxiated newborn. Antepartum Factors Age > 35 years Maternal diabetes Pregnancy-induced hypertension Chronic hypertension Other maternal illness (e.g. CVS, thyroid, neuro) Previous Rh sensitization Drug therapy (e.g. magnesium, lithium adrenergic-blockers) Maternal substance abuse Intrapartum Factors Abnormal presentation Operative delivery Premature labour Premature rupture of membranes Precipitous labour Prolonged labour Indices of fetal distress (FHR abnormalities, biophysical profile) Maternal narcotics (within 4 hrs of delivery) General anaesthesia Meconium-stained fluid Prolapsed cord Placental abruption
No prenatal care Previous stillbirth Bleeding - 2nd/3rd trimester Hydramnios Oligohydramnios Multiple gestation Post-term gestation Small-for-dates fetus Fetal malformations
Personnel 1. At every delivery, at least one individual should be capable of performing a complete resuscitation (i.e. including endotracheal intubation and the use of medications). In many cases, this is the person delivering the infant. 2. A second person who will be primarily responsible for the infant, must be present in the delivery room as well, even for cases when a normal infant is expected. This person must be able to initiate a resuscitation and if a complete resuscitation becomes necessary, assist the fully-trained person. 3. When neonatal asphyxia is anticipated, two individuals whose sole responsibility is to the infant, should be present in the delivery room and be prepared to work as a team to perform a complete resuscitation. The person delivering the mother must not be considered as one of the two resuscitators. 4. With multiple births, a team is needed for each infant. 5. There should be no delay in initiating resuscitation; waiting a few minutes for someone "on-call" to arrive is an unacceptable practice and invites disaster.
Equipment 1. Equipment and medications should be checked as a daily routine and then prior to anticipated need. Used items should be replenished as soon as possible after a resuscitation. 2. The delivery room should be kept relatively warm and the radiant heater should be preheated when possible. Prewarming of towels and blankets can also be helpful in preventing excessive heat loss from the neonate. Resuscitation Equipment in the Delivery Room Suction with manometer Bulb syringe Suction catheters: - 5F or 6F, 8F and 10F Endotracheal tubes: - 2.5, 3.0, 3.5, and 4.0 mm ET tube stylet Laryngoscope with straight blades: - No. 0 & 1 Umbilical vessel catheterization tray Umbilical catheters: - 3.5 & 5F Needles, syringes Feeding tube 8F + syringe
Radiant Heater Stethoscope ECG monitor Wall oxygen with flowmeter and tubing Neonatal resuscitation bag (with manometer) Face masks, Oral airways: - newborn and premature Medications: - Epinephrine (1:10,000) - Naloxone (0.4 or 1 mg ml-1) - Volume expander - Sodium bicarb (0.5mEq ml-1)
Initial Stabilization Prevent Heat Loss 1. Place the infant under an overhead radiant heater to minimize radiant and convective heat loss. 2. Dry the body and head to remove amniotic fluid and prevent evaporative heat loss. This will also provide gentle stimulation to initiate or help maintain breathing. Open the Airway 1. Position the infant supine or on his or her side with the neck either in a neutral position or slightly extended. Avoid overextension or flexion which may produce airway obstruction. A slight Trendelenburg position may also be helpful. 2. A folded towel (approximately 2.5 cm thick) placed under the infant's shoulders may be useful if the infant has a large occiput. 3. If the infant has absent, slow or difficult respirations, apply suction first to the mouth and then nose. If the nose were cleared first the infant may gasp and aspirate secretions in the pharynx. If mechanical suction with an 8F or 10F catheter is used, make sure the vacuum does not exceed -13.3 kPa (-100 mmHg). Limit suctioning to 5 seconds at a time and monitor heart rate for bradycardia which may be associated with deep oropharyngeal stimulation. 4. If meconium is present in the amniotic fluid, special suctioning may be required in the depressed infant. (See Endotracheal Intubation below.) Tactile Stimulation 1. If drying and suctioning do not induce effective breathing, additional safe methods include: o slapping or flicking the soles of the feet o rubbing the back gently 2. Do not waste time continuing tactile stimulation if there is no response after 10 - 15 seconds.
Evaluate the Infant 1. Respirations: Infants who are apneic or gasping despite brief stimulation attempts should receive positive-pressure ventilation. If there is adequate spontaneous breathing, go to next step. 2. Heart Rate: Monitor either by auscultating the apical beat or by palpating the base of the umbilical cord. If the heart rate is below 100 bpm, begin positive-pressure ventilation, even if the infant is making some respiratory efforts. If the heart rate is above 100 bpm, go to the next step. 3. Colour: The presence of central cyanosis indicates that although there is enough oxygen passing through the lungs to maintain the heart rate, the infant is still not well oxygenated. Free-flow 100% oxygen at 5 lmin-1 using a mask held closely to the infant's face should be administered until the infant becomes pink, when the oxygen should be gradually withdrawn.
Prostaglandin 201315
PROSTAGLANDIN INFUSION
DOSAGE
Ranges from 550 nanogram/kg/min (higher doses may be used on the recommendation of a tertiary specialist) Antenatal diagnosis of duct dependent lesion: start at 5 nanogram/kg/min Cyanotic baby or with poorly palpable pulses who is otherwise well and non-acidotic: start at 1015 nanogram/kg/min Acidotic or unwell baby with suspected duct dependent lesion: start at 20 nanogram/kg/min. If no response within first hour, increase to up to 50 nanogram/kg/min
Desired response Suspected left-sided obstruction: aim for palpable pulses, normal pH and normal lactate Suspected right-sided obstruction: aim for SpO2 7585% and normal lactate Suspected or known transposition of the great arteries (TGA) or hypoplastic left or right heart syndrome with SpO2 <70% or worsening lactates liaise urgently with cardiology and/or intensive care/retrieval team (e.g. KIDS) as rapid assessment and atrial septostomy may be necessary
PREPARATIONS
Dinoprostone (Prostaglandin E2) is the recommended prostaglandin* Dinoprostone infusion Standard dinoprostone infusion: Make a solution of 500 microgram in 500 mL by adding 0.5 mL of dinoprostone 1 mg in 1 mL to a 500 mL bag of suitable diluent (glucose 5% or 10% or sodium chloride 0.45% and 0.9%) Transfer 50 mL of this solution into a 50 mL Luer lock syringe and label Discard the 500 mL bag immediately into clinical waste single patient and single dose use only Infusion rate: 0.3 mL/kg/hr = 5 nanogram/kg/min Other information Stability: syringe stable for 24 hr Compatibility: ideally infuse dinoprostone via separate line when there are access issues, infusion can be run with IV line also running maintenance sodium chloride/glucose solutions containing potassium chloride, calcium chloride, dopamine, dobutamine, morphine, heparin Flush: sodium chloride 0.9% at same rate as infusion Administration: continuously (short half-life). Ensure 2 working points of IV access at all times infusions can be given via long line, UVC or peripherally extravasation can cause necrosis use central access if available *If IV Dinoprostone not available, use Alprostadil (Prostaglandin E1) IV as alternative (See Neonatal Formulary) Oral Dinoprostone (See Neonatal Formulary) Used temporarily on very rare occasions when IV access is extremely difficult Discuss with cardiac centre before using May not be as effective as IV prostaglandin
SIDE EFFECTS
Common Apnoea tends to occur in first hour after starting prostaglandin or when dose increased. Consider ventilation
Prostaglandin 201315
Hypotension due to systemic vasodilatation. Consider sodium chloride 0.9% bolus 10 mL/kg Fever Tachycardia Hypoglycaemia
Uncommon Hypothermia Bradycardia Convulsions Cardiac arrest Diarrhoea Disseminated intravascular coagulation (DIC) Gastric outlet obstruction Cortical hyperostosis Gastric hyperplasia (prolonged use)
MONITOR
Heart rate Blood pressure Respiratory rate Temperature Oxygen saturations Blood gases Blood glucose and lactate
EQUIPMENT
Forceps: fine non-bend watchmakers or dissecting Cotton wool balls and gallipots Dressing towel Plastic apron Size 15 scalpel blade and no. 3 handle 25 gauge needle (orange top) 23 gauge needle (blue top) 21 gauge needle (green top) for drawing up lidocaine 2 mL syringe Cleaning solution as per Unit policy Lidocaine 1% Bottles of culture medium Sterile gloves Steristrips Dressings: 1 small transparent dressing (e.g. Tegaderm/Opsite) gauze swabs elasticated cotton or other bandage
SAMPLE REQUIREMENTS
At least 1 mm x 1 mm of skin (ideally 2 mm x 2 mm) from preferred site (e.g. inner side of forearm or posterior aspect just above elbow) choose site carefully as even a small scar on coloured skin will be very obvious if post-mortem, take skin from over scapula as this leaves less obvious damage (see Post-mortem specimens)
PROCEDURE
Consent Inform parents of reason for biopsy, explain procedure and risks including: healing and scarring possibility of contamination poor growth Obtain and record consent Technique Maintain strict asepsis using no touch technique Wash hands and put on apron and sterile gloves Cleanse site ensure cleaning fluid does not pool beneath baby Sedation if appropriate
Inject lidocaine 1%, a little intradermally and remainder subcutaneously to anaesthetise an area 1.5 x 1 cm Wait 5 min to ensure site anaesthetised Cleanse again, wipe off and dry using sterile cotton wool or gauze swabs
Method A Using fine forceps, grip a fold of skin between blades so that a length of skin 3 mm x 2 mm protrudes slice off in one stroke by running scalpel blade along upper edge of forceps blades if skin too thick or oedematous to grip, proceed to Method B Method B Pierce skin with 23 or 21 gauge needle and lift to produce tenting cut off tip of tent to produce a round O shaped piece of skin approximately 2 mm Place into culture medium bottle immediately (lid of bottle removed by assistant for shortest possible time) Complete request form with: clinical details date and time of sampling Dressing wound Although it may bleed freely, wound is usually partial thickness and should not require stitching apply pressure to stanch bleeding apply Steristrips and sterile dressing, bandage if necessary Remove bandage after a few hours, but leave dressing for several days Reassure parents that scar, when visible, will be seen as a fine line Transport Once sample taken, send to Inherited Metabolic Diseases Laboratory as soon as possible if unable to arrange transport immediately, store sample at +4oC for maximum of 12 hr before despatch, do not freeze sample
POST-MORTEM SPECIMENS
In accordance with Human Tissue Act, post-mortem samples must be taken only on licensed premises (or satellites thereof). Check with your pathology laboratory manager Specimens taken after death present a high risk of infection and possible failure of culture. Follow strict aseptic technique Take 2 biopsies from over scapula (as this leaves less obvious damage), as soon as possible after death, ideally before 48 hr have elapsed Send sample to Inherited Metabolic Disease Laboratory immediately, or store at +4oC before dispatch for maximum of 12 hr, do not freeze Include clinical details, date and time of sampling, and date and time of death on request form
SKIN CARE
INTRODUCTION
Neonatal skin care is very important, especially if baby is premature and/or in a critical condition. Special emphasis is placed on skin barrier properties, transcutaneous absorption, transepidermal water loss and maintaining skin integrity
PURPOSE
To maintain integrity of the skin Prevent/minimise skin damage Minimise water loss and heat loss Protect against absorption of toxic materials and drugs Treat skin damage Ensure optimal healing of wounds
RISK FACTORS
Prematurity Birth weight <1000 g Oedema Immobility Congenital skin problems Invasive procedures
Birth weight <1250 g Careful handling Most serious injuries can occur in first hours and days after birth when baby often requires intensive care monitoring Bathing Initial bath not recommended When temperature normal and physiological condition stable, wash babies face and remove meconium from anal area using warm water and cotton wool without soap If stable after 710 days, bathe baby twice per week after 2 months, baby can be bathed daily using mild soap Frequent bathing changes skin pH, disrupts protective acid mantle and is not recommended Preventing/minimising risk of skin injury/infection in all babies Ensure adequate hand hygiene to protect babys skin from cutaneous infection e.g. Staphylococcus aureus Change babys position 46 hrly as condition dictates and place intravenous lines and monitoring leads away from skin Check all substances that come into contact with babys skin. Avoid using substances with potential percutaneous absorption Protect areas of skin from friction injury with soft bedding and supporting blanket rolls Use of pressure-relief mattresses (e.g. Spenco) Change nappy 46 hrly as condition dictates. Wash nappy area with warm water and dry well Nurse baby especially extremely low birth weight, in humidity of 6090% to protect skin, maintain body temperature and prevent water loss Do not use ECG leads on babies <26 weeks gestation Disinfectants Disinfect skin surfaces before invasive procedures such as intravenous cannulation, umbilical vessel catheterisation, chest drain insertion, intravenous puncture or heel pricks for laboratory samples
Adhesives In all newborns, use adhesives sparingly to secure life support, monitoring and other devices Wherever possible, use Duoderm under adhesive tape. Duoderm adheres to skin without the use of adhesive and will prevent epidermal stripping Remove adhesives carefully with warm water on a cotton wool ball. Alcohol is very drying, is easily absorbed and should be avoided
CORD CARE
Immediate Clean cord and surrounding skin surface as needed with cleanser used for initial or routine bathing and rinse thoroughly or cleanse with sterile water Clean umbilical cord with warm water and cotton wool and keep dry Ongoing Keep cord area clean and dry. If cord becomes soiled with urine or stool, cleanse area with water Educate staff and families about normal mechanism of cord healing Teach parents or care-givers to keep area clean and dry, avoid contamination with urine and stool, keep nappy folded away from area and wash hands before handling babys umbilical cord area
NAPPY DERMATITIS
To maintain optimal skin environment Change nappy frequently Use nappy made from absorbent gel materials Use cotton wool and warm water. Do not use commercially available baby wipes Encourage/support breastfeeding throughout infancy Prevention strategies for babies at risk Use petrolatum-based lubricants or barriers containing zinc oxide Avoid use of products not currently recommended for newborns (e.g. polymer barrier films) Treat significant skin excoriation Identify and treat underlying cause Protect injured skin with thick application of barrier containing zinc oxide Presence of red satellite lesions/culture indicates Candida albicans nappy rash Rash will become more intense if covered by occlusive ointments. Treatment includes antifungal ointments or cream and exposure to air and light Do not use powders in treatment of nappy dermatitis Avoid use of antibiotic ointments
Frequent bathing is not recommended? A randomised trial in 53 premature infants (Quinn, 2005) compared bathing every other day (n=28) to bathing every 4th day (n=25). No statistically significant difference was noted in the skin flora count of either group and none of the babies developed infections. The authors concluded that bathing every 4th day was adequate and safe.
Quinn D, Newton N, Piecuch R. Effect of less frequent bathing on premature infant skin. J Obstet Gynecol Neonatal Nurs 2005;34:741-6
Evidence Level: II The use of antibiotic ointment to treat nappy dermatitis should be avoided? A Cochrane systematic review of 4 RCTs (Conner, 2003) found that infants treated with prophylactic topical ointment were at increased risk of coagulase negative staphylococcal infection (typical RR 1.31; 95% CI 1.02 - 1.70; typical risk difference 0.04; 95% CI 0.00 - 0.08); and any nosocomial infection (typical RR 1.20; 95% CI 1.00 1.43; typical risk difference 0.05; 95% CI 0.00 - 0.09).
Conner JM, Soll R, Edwards WH. Topical ointment for preventing infection in preterm infants. Cochrane Database of Systematic Reviews 2003, Issue 4. Art. No.: CD001150
Evidence Level: I Gestational Cut-off for use of Electro Cardio leads on babies is <26 weeks gestation? Insufficient evidence found for any cut-off period.
EQUIPMENT
Cold light source Black drapes to cover incubator
PROCEDURE
Dim lights Expose babys chest and abdomen Remove all non-essential monitoring leads Cover outside of incubator with black drapes Place cold light tip perpendicular to and touching babys skin Shine light from the side, in the 5 positions shown in diagram, comparing right side with left (5th position shines through the liver and is used as a control) Clean cold light tip with an alcohol wipe after use
1. Right side just below axilla 2. Left side just below axilla 3. Right side approximately 5th/6th intercostal space 4. Left side approximately 5th/6th intercostal space 5. Right side just below diaphragm (liver)
DIAGNOSIS
Pneumothorax confirmed if chest fluoresces bright red Compare both sides of chest (babies can have bilateral pneumothoraces) Compare degree of fluorescence with that seen over liver Liver and lung without pneumothorax, shine dull dark red
ACTION
Once pneumothorax is confirmed in a ventilated or unstable baby, perform immediate needle thoracocentesis in 2nd intercostal space, mid-clavicular line on the side of the chest that fluoresced brightly. Do NOT wait for a chest X-ray
UAC 201315
INDICATIONS
Frequent blood gas analysis: ventilated babies non-ventilated baby requiring >40% oxygen at 46 hr of age Continuous monitoring of arterial blood pressure Exchange transfusion
CONTRAINDICATIONS
Umbilical sepsis Necrotising enterocolitis (NEC) Evidence of vascular compromise in legs or buttocks Congenital abnormality of the umbilicus (e.g. exomphalos or gastroschisis)
EQUIPMENT
Umbilical artery catheterisation pack Sterile gown and gloves Sterile drape Infusion pump Sodium chloride 0.9% or 0.45% infusion containing heparin 1 unit/mL Umbilical tape Cleaning solution as per unit policy Zinc oxide tape or Elastoplast
PROCEDURE
Consent Wherever possible inform parents of need and associated risks before procedure; if an emergency, delay explanation until after insertion Risks include sepsis and thrombosis See Consent guideline Non-sterile preparation Monitor babys vital signs during procedure Estimate length of catheter to be inserted using formula: (weight in kg x 3) + 9 cm alternative method for UAC length is twice distance from umbilicus to mid-inguinal point, plus distance from umbilicus to xiphisternum prefer high catheter position: tip above diaphragm (T6T10 vertebral bodies) add length of cord stump to give final length Inspect lower limbs and buttocks for discolouration Tie an umbilical tape loosely around base of cord Sterile preparation Scrub up, put on gown and gloves Use sterile technique Ask assistant (if available) to gently hold babies legs and arms away from umbilical site Clean cord stump and surrounding skin with cleaning solution Attach 3-way tap to catheter and flush all parts with sodium chloride 0.9%. Leave syringe attached Place all equipment to be used on a sterile towel covering a sterile trolley Place sterile sheet with a hole in the centre over the umbilical stump. Pull the stump through the hole ready for catheter insertion
UAC 201315 Insertion of arterial catheter Cut cord cleanly leaving a 23 cm stump; remember to measure length of cord stump and add to calculated placement to give final advancement distance Clamp across cord with artery forceps Apply gentle upward traction Cut along underside of forceps with a scalpel blade to reveal either the cut surface of the whole cord, or use a side-on approach cut part way through the artery at a 45o angle Identify vessels single thin-walled vein two small thick-walled arteries that can protrude from the cut surface Support cord with artery forceps placed near to chosen artery Dilate lumen using either dilator or fine forceps Insert catheter with 3-way tap closed to catheter. If resistance felt, apply gentle steady pressure for 3060 sec Advance catheter to the calculated distance Open 3-way tap to check for easy withdrawal of blood and for pulsation of blood in the catheter If catheter will not advance beyond 45 cm and blood cannot be withdrawn, it is likely that a false passage has been created. Remove catheter and seek advice from a more experienced person Securing catheter If an umbilical venous catheter (UVC) is also to be inserted, site both catheters before securing either. Secure each catheter separately to allow independent removal To secure catheter: place purse string suture around UAC knot suture round umbilicus three or four times place two sutures into cord, one on either side of catheters, allowing suture ends to be at least 3 cm long beyond cut surface of the cord sandwich catheter and ends of the two sutures between zinc oxide or Elastoplast tape as close to cord as possible without touching cord (like a flag). The sutures should be separate from the catheter on either side as this allows easy adjustment of catheter length, should this be necessary If suture is used to stitch edge of tape to umbilical stump for extra security, use extra caution not to nick the catheter If catheter requires adjustment, cut zinc oxide or Elastoplast tape between catheter and the two suture ends, pull back catheter to desired length and retape; never advance once tape applied as this is not sterile Connect catheter to infusion Confirm position of catheter by X-ray: unlike a UVC, a UAC will go down before it goes up a high position tip (above diaphragm but below T6) is preferred if catheter below the diaphragm resite at L3L4 (low position) if catheter position too high, withdraw to appropriate length Acceptable UAC tip positions Tip position T6 to T10 vertebra L3L4 T11 L5 T12L2 Above T6 Femoral artery Acceptable or Unacceptable Acceptable Acceptable Can be used with caution Can be used with caution Not acceptable Not acceptable Not acceptable Precautions/ Adjustments Ideal high UAC position Low UAC position Monitor blood sugar Monitor leg perfusion Risk of bowel or renal ischemia, pull back to L3L4 Pull back to T6T10 Risk of leg ischemia, replace with new UAC
Avoid L1, the origin of the renal arteries Never attempt to advance a catheter after it has been secured; either withdraw it to the low position or remove it and insert a new one
UAC 201315
DOCUMENTATION
Record details of procedure in babys notes, including catheter position on X-ray and whether any adjustments were made Always label umbilical arterial and venous catheters correctly, using the appropriately coloured and labelled stickers
AFTERCARE
Nurse baby supine while UAC is in situ Monitor circulation in lower limbs and buttocks while catheter is in situ Leave cord stump exposed to air Infuse sodium chloride 0.9% or 0.45% 0.5 mL/hr containing 1 unit of heparin/mL Do not infuse any other solution through UAC. Glucose or drugs may be administered through UAC only in exceptional situations, on the authority of a consultant
COMPLICATIONS
Bleeding following accidental disconnection Vasospasm: if blanching of the lower limb occurs and does not resolve within 10 min remove catheter Embolisation from blood clot or air in the infusion system Thrombosis involving: femoral artery, resulting in limb ischaemia renal artery, resulting in haematuria, renal failure and hypertension mesenteric artery, resulting in necrotising enterocolitis (NEC) Infection: prophylactic antibiotics are not required
REMOVAL
Do not attempt to carry out this procedure unsupervised unless you have been trained to do so and have demonstrated your competence
INDICATIONS
Catheter no longer required No longer patent Suspected infection Complications (e.g. NEC, white toes)
EQUIPMENT
Sterile stitch cutter Sterile blade Umbilical tape Cleaning solution as per unit policy
PROCEDURE
Wash hands and put on sterile gloves Clean cord stump with cleaning solution if umbilical tissue adherent to catheter, loosen by soaking cord stump with gauze swab soaked in sodium chloride 0.9% Ensure an umbilical tape is loosely secured around base of umbilicus Turn infusion pump off and clamp infusion line Withdraw catheter slowly over 23 min taking particular care with last 23 cm If bleeding noted, tighten umbilical tape Do not cover umbilicus with large absorbent pad, a small piece of cotton gauze should suffice Inspect catheter after removal: if any part missing, contact consultant immediately
AFTERCARE
Nurse baby supine for 4 hr following removal, and observe for bleeding
COMPLICATIONS
UVC 201315
INDICATIONS
All babies <1000 g Babies >1000 g and ventilated (a double lumen catheter may be indicated if baby requires significant support) Exchange transfusion Central venous pressure monitoring Administration of hypertonic solutions (e.g. glucose 12.5%, parenteral nutrition or inotropes)
CONTRAINDICATIONS
Umbilical sepsis Necrotising enterocolitis (NEC) Gastroschisis/exomphalos
EQUIPMENT
Umbilical vein catheterisation pack Gown and gloves Sterile drape Infusion pump Sodium chloride 0.9% infusion containing heparin 1 unit/mL Umbilical tape Cleaning solution as per unit policy Zinc oxide tape or Elastoplast
PROCEDURE
Consent Wherever possible inform parents of need and associated risks before procedure; if an emergency, delay explanation until after insertion Risks include sepsis and thrombosis See Consent guideline Non-sterile preparation Monitor all vital signs during procedure Estimate length of catheter to be inserted: use formula (weight in kg x 1.5) + 5.5 cm Alternatively, measure distance from umbilicus to xiphisternum for length of UVC high catheter placement preferred: tip at level of diaphragm but not in heart if tip in liver, pull back to lower border of liver (acceptable lower position) and check whether catheter is still sampling freely before use Remember to add length of cord stump to give final distance catheter needs to be advanced Tie umbilical tape loosely around base of cord Sterile preparation Scrub up, and put on gown and gloves Use sterile technique Clean cord stump and surrounding skin with cleaning solution Attach 3-way tap to catheter and flush all parts with sodium chloride 0.9%. Leave syringe attached Put sterile towels into incubator Place all equipment to be used on sterile towel covering sterile trolley Drape umbilical stump with sterile towels Place sterile sheet with a hole in the centre over the cord. Pull the cord through the hole
UVC 201315 Cut cord Clamp across cord with artery forceps Apply gentle upward traction Cut along underside of forceps with scalpel blade cleanly to leave 23 cm stump or, if also placing an umbilical arterial catheter (UAC) and you have been trained in this procedure, consider using side-on technique (see Umbilical artery catheterisation guideline) Remember to measure length of cord stump and add to calculated placement distance to give final length catheter needs to be advanced Insert catheter Identify vessels: single thin walled vein two small thick-walled arteries that can protrude from cut surface Support cord with artery forceps placed near to vein Locate lumen of vein using either a dilator or fine forceps Insert catheter (3.5 F for babies with birth weight <1500 g and 5 F for those >1500 g) with 3-way tap closed to catheter; if resistance felt, apply gentle steady pressure for 3060 sec Advance catheter to desired distance, and open 3-way tap to check for easy withdrawal of blood If catheter will not advance beyond 45 cm and blood cannot be withdrawn, it is likely that a false passage has been created. Remove catheter and seek advice from a more experienced senior person Securing catheter If a UAC is also to be inserted, site both catheters before securing either. Secure each catheter separately to allow independent removal To secure catheter: place purse string suture around umbilical venous catheter (UVC) knot suture around umbilicus three or four times place two sutures into cord, one on either side of the catheter, allowing suture ends to be at least 3 cm long beyond cut surface of cord sandwich catheter and ends of the two sutures between zinc oxide or Elastoplast tape as close to the cord as possible without touching cord. Sutures should be separate from the catheter on either side as this allows easy adjustment of catheter length should this be necessary if suture used to stitch edge of tape to umbilical stump for extra security, take extra care not to nick catheter if catheter requires adjustment, cut zinc oxide or Elastoplast tape between catheter and two suture ends, pull back catheter to desired length and retape; never advance once tape has been applied as it is not sterile Connect catheter to infusion Confirm position of catheter in IVC by X-ray. A UVC goes straight up if catheter found to be in right atrium, withdraw it to avoid risk of cardiac tamponade or cardiac arrhythmia. If catheter needs to be moved back for long distance (e.g. >2 cm), repeat X-ray to confirm new tip position if catheter in liver, withdraw it to lower border of liver so that it lies in IVC, or remove it and insert replacement
DOCUMENTATION
Record in notes details of procedure, including catheter position on X-ray and whether any adjustments were made Always label umbilical arterial and venous catheters correctly, using the appropriately coloured and labelled stickers
AFTERCARE
Monitor circulation in lower limbs and buttocks whilst catheter is in situ Leave cord stump exposed to air The catheter may remain in place for up to 710 days (longer at consultant request). There is a risk of infection if left longer than 7 days
UVC 201315 Any infusions must be connected to UVC in sterile manner using hand hygiene and sterile gloves
COMPLICATIONS
Air embolism Bleeding resulting from accidental disconnection Refractory hypoglycaemia due to malpositioning of catheter Infection: prophylactic antibiotics not required Thrombus formation Cardiac tamponade (see below)
Cardiac tamponade Suspect in presence of: tachycardia poor perfusion soft heart sounds increasing cardiomegaly decreasing oxygen saturation arrhythmias Confirm diagnosis by: chest X-ray: widened mediastinum and enlarged cardiac shadow echocardiogram (if available) If there is cardiovascular compromise, consider drainage (see Pericardiocentesis guideline)
REMOVAL
Do not attempt to carry out this procedure unsupervised unless you have been trained to do so and have demonstrated your competence under appropriate supervision
INDICATIONS
Central venous access no longer required Concerns regarding sepsis Remove after a maximum of 10 days
EQUIPMENT
Sterile stitch cutter Sterile blade Cleaning solution as per unit policy Gown and gloves
PROCEDURE
Wash hands and put on gown and gloves Clean cord stump with cleaning solution Turn infusion pump off and clamp infusion line Ensure umbilical tape secured loosely around base of umbilicus Withdraw catheter slowly If any bleeding noted, tighten umbilical tape Confirm catheter is intact Send catheter tip for culture and sensitivity (if unit policy)
AFTERCARE
Nurse baby supine for 4 hr following removal and observe for bleeding
COMPLICATIONS
Bleeding Loss of UVC tip Infection
Venepuncture 201315
VENEPUNCTURE
INDICATIONS
Blood sampling in a baby without indwelling arterial line, or when sampling from arterial line or capillary sampling is inappropriate
EQUIPMENT
Local anaesthetic cream (as per local policy). Do not use EMLA/AMETOP cream in babies Cleaning solution or cleaning swab follow local infection control policy Appropriately labelled blood bottles and request cards Non-sterile gloves 23 gauge blood sampling needle or needle-safe cannula Do not use a broken needle Sterile gauze/cotton wool to apply to wound post-procedure
Analgesia Unless contraindicated, give sucrose If available, second person to provide containment holding see Pain assessment and management guideline
PROCEDURE
Preparation Wash hands and wear gloves Assistant to employ containment holding and give sucrose Identify suitable vein (typically back of hand or foot) Avoid sampling from potential IV infusion site or long-line vein (e.g. cubital fossa or long saphenous) whenever possible Place paper towels under limb to avoid blood dripping onto bed linen Insertion and sampling Apply hand pressure around limb to distend vein Clean the puncture site then do not touch again Place thumb on skin slightly distal to proposed puncture site Hold needle at a 1020O angle and puncture skin Advance needle toward vein. Resistance may diminish slightly as needle enters vein and blood will be seen to flow Collect required volume taking care to mix but not shake blood When sampling complete, place gauze/cotton wool over insertion point and withdraw needle Maintain pressure on site until bleeding ceases Keep track of all needles used and dispose of them in sharps container Label all samples and investigation forms at cot side Arrange for transfer of samples to laboratory
DIFFICULT VENEPUNCTURE
If small quantities of blood required (<1 mL), use heel prick, but remember that squeezing can cause haemolysis and elevate serum potassium, defer to a more experienced operator Transillumination of limb can help identify suitable vein
Evidence Level: I Sucrose may be used for analgesia? A double-blind trial in 304 newborns (Dilen, 2010) compared four selected 2 mL solutions (10, 20, 30% glucose, and placebo) administered orally before venepuncture. Pain was scored using a validated pain scale (the "Leuven Pain Scale"). A significantly lower average pain score was noted in the 30% glucose group (3.99) when compared with the placebo group (8.43). The average pain scores in the 20% glucose group (5.26) and the 10% glucose group (5.92) were also significantly lower than those in the placebo group. A double-blind trial of 330 healthy term newborns (Taddio 2011) found that sucrose was more effective than liposomal lidocaine for reducing pain during venepuncture. They also found that the addition when liposomal lidocaine and sucrose were used in combination it did confer any benefits to sucrose alone. Before venipuncture, neonates received (1) 1 g of liposomal lidocaine cream topically, (2) 2 mL of 24% sucrose solution orally, or (3) sucrose and liposomal lidocaine. The facial grimacing score (0-100) was used to assess pain. Facial grimacing scores were lower in the sucrose group compared with those in the liposomal lidocaine group (mean difference: -27 [95% confidence interval (CI): -36 to -19; P < .001) and for the sucrose plus liposomal lidocaine group compared with those in the liposomal lidocaine group (mean difference: -23 [95% CI: -31 to -14]; P < .001). The sucrose and sucrose plus liposomal lidocaine groups did not differ (mean difference: -5 [95% CI: -13 to 4]; P = .3).
Dilen B, Elseviers M. Oral glucose solution as pain relief in newborns: results of a clinical trial. Birth 2010;37:98-105 Taddio A, Shah V, Stephens D et al. Effects of liposomal lidocaine and sucrose alone and in combination for venepuncture pain in newborns. Pediatrics 2011; 127: 940-7.
Evidence Level: II
RENAL FAILURE
DEFINITION
Failure of the kidneys to maintain metabolic stability in relation to fluid balance, electrolyte balance and excretion of nitrogenous waste
MAIN CAUSES
Congenital Usually affects term babies Often diagnosed antenatally with renal abnormality/hydrops Most commonly an obstructive uropathy posterior urethral valves bilateral pelvi-ureteric junction (PUJ) obstruction Non-obstructive cause renal agenesis polycystic kidneys (autosomal recessive) secondary to congenital heart disease hypoperfusion severe acidosis Asphyxia Occurs in severe hypoxic ischaemic encephalopathy direct hypoxic effect or secondary to hypoperfusion usually transient poor prognosis for intact survival when severe Prematurity Normally caused by poor renal perfusion secondary to: hypovolaemia hypotension hypoxaemia sepsis Inappropriate ADH in ventilated babies causes transient oliguria will correct spontaneously as lung compliance improves Other Renal vein thrombosis Renal artery thrombosis
DIAGNOSIS
Renal abnormalities (prenatal) Risk factors (i.e. severe asphyxia/sepsis/hypotension/congenital heart disease) Oliguria (<1 mL/kg/hr) Hypovolaemia Electrolyte disturbance (particularly raised potassium) Rising creatinine after 48 hr
PREVENTION
This is the most important approach in the preterm baby Ensure adequate fluid intake particularly in very preterm babies with excessive transepidermal water loss (see Fluid balance below)
Extra care required when using radiant heaters in contrast to high humidification in incubator (see Hypothermia guideline) Maintain a safe blood pressure (see Hypotension guideline)
INVESTIGATIONS
Monitor Weigh 12-hrly BP 12-hrly Cardiac monitor to detect arrhythmias Urine Dipstick (proteinuria; sediment, such as blood, casts, tubular debris, indicate intrinsic problem; WBC and nitrites suggest infection) Microscopy and culture Electrolytes, urea, creatinine, osmolality Blood U&E, creatinine 8-hrly Blood gas, pH 48 hrly Blood cultures, CRP Glucose 4-hrly Calcium, phosphate, magnesium, albumin Blood count (film and platelets) Typical biochemical changes in acute renal failure (ARF) Increased urea, creatinine, K+, PO42Reduced Na+, Ca2+, HCO3-, pH Imaging If umbilical artery catheter (UAC) in place, abdominal X-ray to check position confirm UAC tip does not sit at L1 Renal ultrasound scan to detect congenital causes, post-renal causes, pyelonephritis and renal vein thrombosis
TREAMENT
Correct underlying cause Surgical approach to uropathy unless prognosis hopeless (e.g. Potters syndrome) Correct hypovolaemia, but avoid over-hydration in established renal failure 20 mL/kg sodium chloride 0.9% IV if blood loss known or suspected, give 20 mL/kg packed red cells over 3060 min If hypotensive in absence of fluid depletion: start inotrope infusion: if central access, give dopamine 5 microgram/kg/min or if no central access (e.g. UVC), dobutamine 10 microgram/kg/min (see Hypotension guideline) Open duct in duct-dependent circulation in congenital heart disease (see Cardiovascular guidelines) Antibiotics for sepsis Stop all nephrotoxic drugs (e.g. aminoglycosides, vancomycin, furosemide) if possible In the majority of cases the kidneys will recover in 2448 hr Supportive Assess fluid balance when problem recognised
Signs of depletion Cold peripheries Delayed capillary refill Tachycardic Oliguric (<1 mL/kg/hr) or anuric Signs of overload Tachypnoeic Oedema Excessive weight gain Raised blood pressure Gallop rhythm Hepatomegaly Fluid balance If baby hypovolaemic/hypotensive, it is important to correct this before instituting fluid restriction (see above) Strictly monitor all intake and output Restrict fluid intake to minimal maintenance fluids Calculate maintenance fluid: maintenance fluid = insensible losses + urine output + GIT losses insensible losses: <1500 g (at birth) = 5080 mL/kg/day >1500 g (at birth) = 1535 mL/kg/day for babies in well-humidified incubator or receiving humidified respiratory support, use lower figure Replace maintenance fluid as glucose1020% (electrolyte-free) Electrolytes will be required if electrolyte losses ongoing (e.g. diarrhoea, fistula) Weigh twice daily best guide to change in hydration is change in body weight stable weight indicates overhydration and need to reduce fluid intake further aim to achieve 1% loss of body weight daily Hyperkalaemia See Hyperkalaemia guideline Acidosis Monitor pH 8-hrly if pH <7.2, give sodium bicarbonate 1 mmol/kg IV over at least 2 min Hyponatraemia Low sodium is more likely to indicate fluid overload than a deficit in body sodium Unless evidence of dehydration, treatment should be fluid restriction with maintenance sodium intake of 23 mmol/kg/day If severe (Na <120 mmol/L) and associated with neurological symptoms, such as seizures: use hypertonic saline (sodium chloride 3%) 4 mL/kg over a minimum of 15 min: check serum sodium immediately after completion of infusion If baby still fitting, dose can be repeated after assessing serum sodium concentration During recovery phase, babies rarely become polyuric, when sodium chloride 0.45% is typically required, although this will depend on a measurement of urinary sodium concentration Dialysis Hardly ever used in this population because of technical difficulty and poor prognosis
MONITORING
Most useful variable is urine output In newborn renal failure, anuria/oliguria is the normal situation and increasing urine output indicates recovery Creatinine estimation is often misleading in first few days: in utero, creatinine is cleared by the placenta after delivery, creatinine production by muscles is not stable and can be influenced heavily by muscle damage resulting from delivery/hypoxia/sepsis after 4872 hr, it can be used, but the trend is much more valuable than the absolute concentration Urea estimation is misleading it is influenced by tissue breakdown (e.g. bruises/swallowed blood) conversely, little is produced when protein intake is compromised
CONCLUSION
In the newborn baby, the vast majority of cases of renal failure will recover if the underlying cause is addressed and supportive management provided to maintain fluid and electrolyte balance until recovery takes place, normally over 2448 hr
Evidence Level: IV When treating severe hyponatraemia, what is the correct dosage of hypertonic saline (sodium chloride) to be used? (e.g sodium chloride 3% 4 ml/kg over minimum of 15 min vs- 2-5 mL/kg over 3060 min?) The BNF for Children (2013), referring to sodium chloride administered intravenously, recommend that if sodium chloride is required, the deficit should be corrected slowly to avoid the risk of osmotic demyelination syndrome; the rise in plasma-sodium concentration should be no more than 10 mmol/litre in 24 hours for children. Aside from this, there appears to be little other than anecdotal evidence for details on dosage levels for severe hyponatraemia in neonates. Gouyon & Guinard (2000) recommend for example that Sodium Chloride (5-8 ml/kg of a 3% solution) is slowly given IV over 2 -3 hours.
BNF For Children. 2013. http://www.medicinescomplete.com/mc/bnfc/current/PHP13998-intravenoussodium.htm Gouyon, JB, Guignard, JP. Management of renal failure in newborns. Pediatric Nephrology 2000: 14: 1037-1044.
Evidence Level: V
POSTNATAL MANAGEMENT
Indications for intervention Urgent Bilateral RPD 10 mm +/- thick-walled bladder: suspect posterior urethral valve (boys) Unilateral RPD 15 mm, suspect pelviureteric junction (PUJ) obstruction Significant abnormalities of kidney(s)/urinary tract if risk of renal insufficiency: check serum potassium, blood gas for metabolic acidosis and serum creatinine Non-urgent All other abnormalities of urinary tract in the antenatal scan
IMMEDIATE MANAGEMENT
For urgent indications If posterior urethral valve (PUV)/PUJ obstruction suspected, check urine output/stream and monitor weight trend Arrange urgent renal ultrasound scan within 2448 hr (low GFR may give a false normal appearance if checked too early but, if there is gross dilatation, do not delay) If postnatal scan suggests posterior urethral valve: check serum creatinine arrange urgent micturating cysto-urethrogram (MCUG) after confirmation by MCUG, refer baby urgently to paediatric urologist (can be done within 48 hr). Catheterisation of bladder often advised If unilateral RPD 20 mm, discuss with urologist and arrange MAG3 renogram as soon as possible Significant abnormalities of kidney(s)/urinary tract if risk of renal insufficiency: check serum potassium, blood gas for metabolic acidosis and serum creatinine start trimethoprim 2 mg/kg as single night-time dose Discuss with consultant before discharge For non-urgent indications Renal ultrasound scan at 26 weeks of age (according to local practice) Consultant review with results Antibiotic prophylaxis For RPD 10 mm, give trimethoprim 2 mg/kg as single night-time dose until criteria for stopping is met (see below)
SUBSEQUENT MANAGEMENT
Subsequent management depends on findings of ultrasound scan at 26 weeks Severe pelviectasis (RPD 15 mm) Arrange MAG3 scan timing depends on severity of obstruction as soon as possible if RPD 20 mm if MAG 3 scan shows obstructed pattern, discuss with paediatric urologist Repeat ultrasound scan at 36 months of age (depending on cause of dilatation, a complete obstruction requires closer monitoring) Continue antibiotic prophylaxis until advised otherwise by urologist Moderate unilateral pelviectasis (RPD 1014 mm) and/or ureteric dilatation Presumed mild obstruction or vesico-ureteric reflux (VUR) If RPD increasing beyond 15 mm, arrange MAG3 scan Continue prophylaxis for VUR grade 4 (marked dilatation of ureter and calyces) until 2 yr of age if infection-free Repeat scan every 6 months until RPD <10 mm, then follow advice below Normal or mild isolated pelviectasis (RPD <10 mm) Stop antibiotic prophylaxis Repeat scan after 6 months if 6 month scan normal or shows no change and there have been no urinary tract infections (UTIs), discharge If unwell, especially pyrexial without obvious cause, advise urine collection Multi-cystic dysplastic kidney (MCDK) DMSA to clarify nil function of MCDK and normal uptake pattern of other kidney
Repeat ultrasound scan 612 monthly to observe involution of kidney (may take several years) Beware of 20% risk of vesico-ureteric reflux (VUR) in normal kidney, advise parents to recognise UTI/pyelonephritis (especially if fever is without obvious focus) MCUG or prophylaxis until continent ONLY if dilated pelvis or ureter in good kidney Annual blood pressure check until kidney involuted Urology referral if cysts persistent, enlarging or hypertension
Ureterocoele MCUG (if VUR or PUV suspected) MAG3 to check function and drainage from both moieties of the duplex system Prophylaxis until problem resolved Urology referral sooner if obstruction suspected Solitary kidney/unilateral renal agenesis Kidney ultrasound at 6 weeks to confirm antenatal findings and rule out other urogenital structure abnormalities DMSA to confirm absence of other kidney Renal parenchymal problem requiring nephrology review Bright kidneys Multiple cysts Other conditions Single renal artery in cord increased risk of renal abnormality but postnatal ultrasound scan only if antenatal scan missed or abnormal Ear abnormalities: ultrasound examination only if associated with: syndrome other malformations maternal/gestational diabetes family history of deafness
Significance Most babies born <34 weeks gestation have primary apnoea of prematurity (PAP) multiple aetiologic factors can exacerbate apnoea in preterm babies sudden increase in frequency warrants immediate action Consider causes other than apnoea of prematurity if occurs: in term or near-term baby (>34 weeks gestation) on first day after birth in preterm baby Causes Sepsis septicaemia necrotising enterocolitis meningitis Respiratory inadequate respiratory support upper airway obstruction surfactant deficiency CNS intracranial haemorrhage seizure congenital malformations CVS patent ductus arteriosus anaemia GI gastro-oesophageal reflux Other metabolic abnormalities, especially hypoglycaemia haematological: anaemia
MANAGEMENT
Terminate episode If apnoea not self-limiting, perform the following in sequence to terminate episode: stimulate baby by tickling feet or stroking abdomen if aspiration or secretions in pharynx suspected, apply brief oropharyngeal suction face mask ventilation consider emergency intubation Once stable, perform thorough clinical examination to confirm/evaluate cause Screen for sepsis If apnoea or bradycardia increasingly frequent or severe, screen for sepsis as apnoea and bradycardia can be sole presenting sign
TREATMENT
Treat specific cause, if present Primary apnoea of prematurity is a diagnosis of exclusion and may not require treatment unless pauses are: frequent (>8 in 12 hr) or severe (>2 episodes/day requiring positive pressure ventilation)
Pharmacological treatment Caffeine citrate 20 mg/kg loading dose (oral/IV over 30 min) followed, after 24 hr, by maintenance dose 5 mg/kg oral/IV (over 10 min) once daily, increasing to 10 mg/kg if required Non-pharmacological treatment CPAP, SiPAP/BiPAP See CPAP guideline If CPAP fails, intubate and ventilate
BLOODSPOT SCREENING
INTRODUCTION
Screen babies at 58 days of age (date of birth = day 0) for the following conditions: sickle cell disease phenylketonuria (PKU) congenital hypothyroidism (CHT) cystic fibrosis (phased implementation) medium chain acyl co-A dehydrogenase (MCADD) deficiency Obtain pre-transfusion bloodspot samples as previous blood transfusions can falsify results
TIMING
If transfused before day 5 Collect first bloodspot card before transfusion fill 2 circles mark card pre-transfusion Collect second bloodspot card at 58 days of age and at least 72 hr after blood transfusion fill 4 circles record whether plasma or red cells transfused Staple pre-transfusion and second bloodspot card together and send to West Midlands screening centre Multiple transfusions between 5 and 8 days of age Collect 4 bloodspots within this window. Complete with as much time-lapse as possible from any transfusion Depending on circumstances, screening laboratory will request repeat bloodspot No transfusions before day 5 Collect routine bloodspot card at day 5 fill 4 circles and send to West Midlands screening centre, irrespective of milk feeds or gestational age Preterm babies <32 weeks (<31 weeks and 6 days) will require repeat sample at 28 days or discharge home, whichever is the sooner
Further Information Detailed information available from UK Newborn screening programme centre website: http://newbornbloodspot.screening.nhs.uk/
CHEST PHYSIOTHERAPY
INTRODUCTION
All units caring for babies requiring intensive care and high dependency care and providing a chest clearance service should have access to a paediatric/neonatal specialised respiratory physiotherapist with experience in assessing and treating premature and sick newborn babies Proviso: If paediatric/neonatal specialised respiratory physiotherapist not available, follow local guidelines
INDICATIONS
To improve respiratory function and care of babies on the Neonatal Unit, particularly when excessive secretions and mucus plugging compromise respiratory function
PERCUSSION
Chest percussion followed by suction is the most commonly used treatment and should only be undertaken by fully trained nursing staff
Definition Rhythmical patting action over chest wall using a palm cup percussor Produces pressure changes in the airways stimulating mucous clearance by ciliary stimulation, gas liquid interaction and release of pulmonary chemical mediators improving ciliary transport speed Indications for ventilated baby Chest X-ray changes: infective collapse/consolidation segmental +/- lobar lung collapse secondary to mucus plugging aspiration tenacious secretions not cleared effectively with suction +/- sodium chloride 0.9% Signs of acute secretion retention: decreased: SpO2/PO2//tidal volumes in pressure-controlled mode/chest movement increased PCO2, (can be acute in compensated respiratory acidosis)/peak airway pressure in volume-controlled mode/airway resistance/endotracheal tube (ETT) leak audible secretions on auscultation Non-ventilated baby Residual secretion clearance issues associated with indications listed above Secretion retention/infection in chronic lung disease Congenital neuromuscular pathologies resulting in poor airway protection. May require prophylactic physiotherapy and parental training before discharge
CONTRAINDICATIONS
Undrained pneumothorax Acute pulmonary haemorrhage Metabolic bone disease Intraventricular haemorrhage (IVH) within 2448 hr Extreme prematurity (<1500 g/<26 weeks gestation) in first week of life Platelet levels <50 x 109/L and/or prolonged clotting
Risks of percussion Vigorous percussion in vulnerable extremely preterm babies and poor use of supportive developmental care techniques have previously been linked with IVH and encephaloclastic porencephaly
Chest physiotherapy 201315 Special precautions/cautions Poor skin integrity Cardiovascular instability Platelet levels <100 x 109/L Avoid chest drain sites and Broviac lines/proximity of wounds/stomas Effectiveness reduced in chest wall oedema Recent retinopathy of prematurity (ROP) laser therapy Over areas of PIE
EQUIPMENT
Single use vinyl percussor (pink or blue), neonatal or paediatric according to size of thorax Suction catheters Clean or sterile gloves according to unit infection control policy Sodium chloride 0.9% in sterile ampoules Containment/nesting equipment
PROCEDURE
Plan treatment episodes pre-feed or no less than 30 min post-feed Pre-procedure assessment Cardiovascular status Response to handling/level of comfort Ventilatory review: oxygen requirement/mode of ventilation/tidal volumes, pressures, resistance, percentage of ETT leak/arterial/capillary gases/desaturations/apnoeas Latest chest X-ray Colour, chest movement, and auscultation Microbiology results sputum and blood cultures Drug history e.g. antibiotics/steroids Developmental care during physiotherapy Ensure nesting and developmental care support throughout procedure (see Developmental care guideline and Positioning guideline) Pace treatment according to babys needs and cues and minimise stress Before intervention, give baby an auditory approach signal (talking by nurse or parent), and tactile approach with hand placed gently but firmly on babys chest Positioning See Positioning guideline If presenting with acute collapse/infective productive consolidation, position baby with that area uppermost consider whether baby tolerates a position, especially where there is ventilation/perfusion mismatch or contraindication e.g. prone with umbilical lines, or post abdominal surgery side-lying, prone and supine positions are frequently used increasing oxygen will be necessary to maintain SpO2 9095% Do not disconnect baby from the ventilator for a turn If full side-lying is not tolerated, tilt between supine and side-lying. A quarter turn prone facing right, with head towards that side is effective in facilitating ventilation and reinflation for persistent right upper lobe collapse Frequency of turning will depend on clinical status (e.g. chest X-ray and tolerance/comfort) and observations (gases/saturations etc) Whilst frequent position changes are discouraged, do not leave baby in one position for very prolonged periods dependant lung can retain secretions/collapse Never use head-down tilt due to risk of IVH/reflux/respiratory compromise Percussion Stabilise head with one hand at all times during percussion
Chest physiotherapy 201315 Ensure whole circumference of the percussor makes contact with babys chest, ideally directly on babys skin, producing a cushion of air. If not practical, a layer of vest is acceptable. The pressure should not cause any movement of baby/skin reaction ideal rate approximately 3/sec Ensure short percussion episodes, to a maximum of 12 min according to babys stability/tolerance/gestational age, followed by suction. More robust self-ventilating babies can receive up to 23 min of treatment Reassess baby. If further percussion necessary, repeat the above Address signs of stress by pacing baby or giving time-out/containment holding Unless there is an acute deterioration necessitating treatment between sessions, carry out regular percussion at a maximum of 4-hrly/when clinically indicated. Use a maximum of 2 positions Keep percussor in the incubator. Wash with soap and warm water and Alco wipe
Post-percussion suction ETT suctioning (see local policy where available) Catheter for open suction must have a Mlly tip (larger end hole and 2 opposite pressure relieving side-eyes) and be no larger than two-thirds of diameter of ETT Use graduated catheter to minimise cardiovascular instability and trauma Maintain normal saturation range for gestational age throughout procedure, by titrated pre/post-oxygenation. Avoid hyperoxia Recommended suction pressures 810 kPa according to size of baby (12 kPa in a term baby) Use sodium chloride 0.9% instillation via ETT before suction to mobilise tenacious secretions/mucus plug(s) and secretions partially occluding the ETT warmed unopened ampoules to babys temperature in incubator. Standard volumes are 0.20.3 mL per instillation (0.5 mL in a term baby) Oral suction must follow to clear secretions from around the ETT use a catheter no larger than 10 FG Rate of respiration to assist recruitment of lung can be increased temporarily post-suction using manual breath/inspiratory breath facility on the ventilator If tidal volumes improve above volumes for gestational age, review ventilation to reduce risk of volutrauma and hypocarbia When not in use, turn suction off to reduce noise HFOV Use percussion if clinically indicated pre-use of closed suction technique which minimises lung derecruitment After suction, increase mean airway pressure by 1 cm H2O to recruit lung at the discretion of medical staff Nonventilated babies Oral suction with size 8 or 10 catheter Babies with neurological issues resulting in impaired swallowing/chronic lung disease/cystic fibrosis have individualised percussion and suction treatment plans
AFTERCARE
Clinical effectiveness is judged by: increased secretion clearance/increased air entry on auscultation improved oxygenation improved CO2 clearance reduction in ventilatory support improvement in chest X-ray appearance inflation of collapse, reduction in infective consolidation It is imperative to assess the need for ongoing percussion daily and wean/discontinue when desired clinical parameters achieved
Key neonatal documents regarding provision of service Neonatal Toolkit 2009 http://www.neonatal.org.uk Principle 2.2.5.1 NICE Quality Standards 2010 http://www.nice.org.uk Standard 3 b Bliss Baby Charter 2011 http://www.bliss.org.uk Standard 3.2 BAPM Neonatal Service Standards http://www.bapm.org Standard 6.2.3. Standard 4.2.2
Evidence Level: II Last amended July 2013 Last reviewed July 2013
Evidence Level: IV How do different dexamethasone dosing regimes compare in terms of risks v benefits? Three Cochrane systematic reviews (Halliday, 2009i; Halliday, 2009ii; Halliday, 2003) have concluded that the benefits of early (<96 hours), moderately early (7-14 days) and late (>3 weeks) treatment with corticosteroids may not outweigh the actual or potential adverse effects. In particular, no study to date has been sufficiently powered to detect important adverse long-term neurosensory outcomes (Halliday, 2004i). In view of this, the recommendation is to reserve treatment for those infants who cannot be weaned from mechanical ventilation, and to minimise the dose and duration of any course of treatment. A further Cochrane review of 5 trials comparing inhaled versus systemic corticosteroids (Shah, 2004) found no advantage for inhaled steroids, either in effectiveness or in side-effect profiles. Significant adverse effects, in terms of spontaneous gastrointestinal perforation, cessation of weight gain, and smaller head circumference have been recorded at moderate dose levels (0.150.02mg/kg over 10 days) (Stark, 2001) as well as the more usual dose of 0.5-0.05mg/kg over 12 days (Anon, 2002). A retrospective, two-centre study (van der Heide-Jalving, 2003) compared 25 hydrocortisonetreated patients (tapering dose of 5-1mg/kg for 22 days) and 25 controls with 23 dexamethasonetreated patients (tapering dose of 0.5-0.1 mg/kg for 21 days) and 23 controls. Effectiveness was
found to be equal, but both short and long term adverse effects were significantly fewer in the hydrocortisone group. A more appropriately-powered study needs to be conducted in order to confirm these findings.
Anon. Postnatal corticosteroids to treat or prevent chronic lung disease in preterm infants. American Academy of Pediatrics Committee on Fetus and Newborn. Canadian Paediatric Society Fetus and Newborn Committee. Pediatrics 2002;109:330-38 Halliday HL, Ehrenkranz RA, Doyle LW. Early (< 8 days) postnatal corticosteroids for preventing chronic lung disease in preterm infants. Cochrane Database of Systematic Reviews 2009, Issue 1. Art. No.: CD001146 Halliday HL, Ehrenkranz RA, Doyle LW. Moderately early (7-14 days) postnatal corticosteroids for preventing chronic lung disease in preterm infants. The Cochrane Database of Systematic Reviews 2003, Issue 1. Art. No.: CD001144 Halliday HL, Ehrenkranz RA, Doyle LW. Delayed (>3 weeks) postnatal corticosteroids for chronic lung disease in preterm infants. The Cochrane Database of Systematic Reviews. 2009, Issue 1. Art. No.: CD001145 Shah SS, Ohlsson A, Halliday H, et al. Inhaled versus systemic corticosteroids for the treatment of chronic lung disease in ventilated very low birth weight preterm infants. The Cochrane Database of Systematic Reviews 2003, Issue 1. Art. No.: CD002057 Stark AR, Carlo WA, Tyson JE, et al. Adverse effects of early dexamethasone treatment in extremely-lowbirth-weight infants. N Engl J Med 2001;344:95-101 van der Heide-Jalving M, Kamphuis PJ, van der Laan MJ, et al. Short-and long-term effects of neonatal glucocorticoid therapy: is hydrocortisone an alternative to dexamethasone? Acta Paediatr 2003;92:827-35
Evidence Level: I What is the role of diuretics? Lung disease in preterm infants is often complicated with lung oedema. A Cochrane review of 6 small studies (Stewart, 2011i) found that, in preterm infants > 3 weeks of age with CLD, acute and chronic administration of distal diuretics improved pulmonary mechanics. The authors warn that positive effects should be interpreted with caution as the numbers of patients studied are small in surprisingly few randomized controlled trials. Another Cochrane review by the same team (Stewart, 2011ii) concluded that: In view of the lack of data from randomized trials concerning effects on important clinical outcomes, routine or sustained use of systemic loop diuretics in infants with (or developing) CLD cannot be recommended based on current evidence. Randomized trials are needed to assess the effects of furosemide administration on survival, duration of ventilatory support and oxygen administration, length of hospital stay, potential complications and long-term outcome.
Stewart A, Brion LP, Ambrosio-Perez I. Diuretics acting on the distal renal tubule for preterm infants with (or developing) chronic lung disease. Cochrane Database of Systematic Reviews 2011, Issue 9. Art. No.: CD001817 Stewart A, Brion LP. Intravenous or enteral loop diuretics for preterm infants with (or developing) chronic lung disease. Cochrane Database of Systematic Reviews 2011, Issue 9. Art. No.: CD001453
Evidence Level: I Last amended July 2013 Last reviewed July 2013
CLD 201315
Bronchopulmonary dysplasia Mild Moderate Severe In air at 36 weeks CGA or discharge <30% oxygen at 36 weeks CGA or discharge >30% oxygen +/- CPAP or ventilation at 36 weeks CGA or discharge In air by 56 days postnatal age or discharge <30% oxygen at 56 days postnatal age or discharge >30% oxygen +/- CPAP or ventilation at 56 days postnatal age or discharge
Investigations Chest X-ray: homogenous opacification of lung fields developing after first week of life (Type 1) or coarse streaky opacities with cystic translucencies in lung fields (Type 2) Exclude infections by appropriate investigations, including microbiology of endotracheal secretions for bacteria, viruses and ureaplasma Exclude patent ductus arteriosus (PDA) clinically or with echocardiography, depending on local expertise available
TREATMENT
Optimise ventilation strategies Use lowest possible ventilator pressures to deliver appropriate tidal volumes to minimise barotrauma and volutrauma. Volume-targeted ventilation may be helpful if available Optimise nutrition Ensure adequate calorie intake (at least 120 Kcal/kg/day) because of increased work of breathing If growth unsatisfactory, involve dietitian Avoid fluid overload 1. Corticosteroids If ventilator-dependent and requiring increasing or persistently high oxygen intake, consider using corticosteroids between 1421 days of age Treatment with corticosteroids is a consultant only decision Inform parents of potential short-term and long-term adverse effects Obtain oral consent and record in notes Short-term side effects Risk of infection Poor growth Reversible ventricular hypertrophy Gastrointestinal perforation and bleeding Adrenal suppression Glucose intolerance
CLD 201315
Long-term side effects Increased risk of neurodisability Doses Use Neonatal Formulary for dexamethasone dosage regimen If respiratory status worsens after initial improvement, consultant only to consider repeating course of corticosteroids Monitoring while on corticosteroids Daily BP and urinary glucose 2. Diuretics Use of diuretics is a consultant decision. They can be considered if baby >3 weeks old and either ventilator-dependent or requiring >40% supplemental oxygen. Diuretics of choice are chlorothiazide 10 mg/kg oral 12-hrly and spironolactone 1 mg/kg oral 12-hrly. Avoid amiloride due to its lung fluid retaining properties Side-effects include hyponatraemia, hypo/hyperkalaemia, hypercalciuria (leading to nephrocalcinosis) and metabolic alkalosis Echocardiography and ECG Any baby with chronic lung disease on long-term oxygen, echocardiography and ECG for evidence of pulmonary hypertension
SUBSEQUENT MANAGEMENT
Monitoring treatment Continuous Aim for SpO2 of 9295% until 36 weeks corrected gestational age After 36 weeks gestational age, maintain saturations >95% to prevent pulmonary hypertension and cor pulmonale Warm and humidify supplemental oxygen unless low-flow Beware of infections, particularly fungal Twice weekly Blood gases Electrolytes: if treated with diuretics Weekly Electrolytes: if not treated with diuretics Weight and head growth
Parent information Offer parents the following information, available from: http://www.bliss.org.uk/wp-content/uploads/2012/02/Ventilation_and_CLD.pdf
CPAP NM 04.09.13
Benefits Improves oxygenation Reduces work of breathing Maintains lung volume Lowers upper airway resistance Conserves surfactant
INDICATIONS
Early onset respiratory distress in preterm babies Respiratory support following extubation Respiratory support in preterm babies with evolving chronic lung disease Recurrent apnoea (in preterm babies) Atelectasis Tracheomalacia
CPAP following extubation Indicated for majority of babies of <32 weeks gestation 'Rescue' CPAP Applied in first hours following extubation used for apnoeas and/or desaturations and/or increase in oxygen requirement apnoeas and/or desaturations appearing later than the first few hours after extubation may indicate atelectasis but consider an additional underlying clinical condition (e.g. sepsis)
CONTRAINDICATIONS
Any baby fulfilling the criteria for ventilation Irregular respirations Pneumothorax without chest drain Nasal trauma/deformity that might be exacerbated by use of nasal prongs Larger, more mature babies often do not tolerate the application of CPAP devices well Congenital anomalies: diaphragmatic hernia choanal atresia tracheo-oesophageal fistula gastroschisis When in doubt about CPAP indications or contraindications, discuss with consultant
TYPES OF CPAP exact CPAP device will vary from unit to unit
1. Standard CPAP 2. Two-level CPAP 3. Bubble CPAP
1. STANDARD CPAP
Equipment Short binasal prongs and/or nasal mask Circuit Humidification
CPAP NM 04.09.13
CPAP generating device with gas mixing and pressure monitoring All require high gas flow (usual starting rate 8 L/min)
Fixing nasal CPAP device: short binasal prongs (preferred) To avoid loss of pressure, use largest prongs that fit nostrils comfortably Ensure device is straight and not pressed hard against nasal septum or lateral walls of nostrils. Excessive pressure can cause tissue damage Nasal mask Fit securely over nose consider alternating mask with prongs, particularly if baby developing excoriation or erosion of nasal septum. Masks can also result in trauma, usually at the junction between the nasal septum and philtrum Masks can give a poor seal and have a tendency to obstruct Procedure Position baby Prone position is preferable Avoid excessive flexion, extension or rotation of the head Set up equipment (see specific manufacturer instructions) Connect humidification to CPAP Connect CPAP circuit with prongs to CPAP device Place CPAP hat on baby Attach CPAP circuit to CPAP hat and apply prongs/mask Turn on CPAP flow and set pressure Pressure range Start at 56 cm H2O initially and increase by 1 cm H2O increments Optimum pressure depends on illness type and severity watch baby and use lowest pressure required to improve work of breathing High pressures (10 cm H2O) may restrict pulmonary blood flow, increase air leak risk and cause over-distension CPAP 'failure' 'Failure of CPAP' implies a need for ventilation. Consider intubation and surfactant for preterm babies on CPAP as initial therapy if: early chest X-ray demonstrates RDS and if any of the following apply: - FiO2 consistently >0.5 - marked respiratory distress - persistent respiratory acidosis - recurrent significant apnoea - irregular breathing Checks Before accepting apparent CPAP 'failure' exclude: pneumothorax insufficient pressure insufficient circuit flow inappropriate prong size or placement airway obstruction from secretions open mouth Complications Erosion of nasal septum: reduce risk by careful prong placement and regular reassessment Gastric distension: benign, reduce by maintaining open nasogastric tube
CPAP NM 04.09.13
Weaning CPAP When Start when baby consistently requiring FiO2 <0.35, pressure 5 cmH2O and stable clinical condition If nasal tissue damage significant, consider earlier weaning Note: High-flow humidified oxygen therapy Increasingly used as non-invasive respiratory support Offers theoretical advantages over CPAP in ventilating upper airway spaces and producing less nasal tissue damage When weaning CPAP, consider using 56 L/min of high-flow humidified oxygen (e.g. Vapotherm or Optiflow) rather than low-flow nasal cannulae oxygen How: 'Pressure reduction' or 'Time off' Pressure reduction more physiological approach although can increase the work of breathing if pressure is too low wean pressures in steps of 1 cm H2O every 1224 hr. If no deterioration, discontinue CPAP after 24 hr of 3 cm H2O Time off CPAP plan using 2 x 12 or 3 x 8 hr time periods The following regimen of cycling CPAP can be adapted to individual situations Day 1 Day 2 Day 3 Day 4 Day 5 Day 6 1 hr off twice a day (1 off, 11 on) 2 hr off twice a day (2 off, 10 on) 3 hr off twice a day (3 off, 9 on) 4 hr off twice a day (4 off, 8 on) 6 hr off twice a day (6 off, 6 on) Off CPAP
Failure of weaning Increased oxygen requirement, increasing respiratory distress and worsening respiratory acidosis during weaning should necessitate a review and consider escalation of support
2. TWO-LEVEL CPAP
Two-level CPAP at a rate set by clinician (biphasic) or triggered by baby using an abdominal sensor (biphasic trigger or Infant Flow SiPAP) Inspiratory time, pressures and apnoea alarm limit set by clinician Indications/contraindications as CPAP and can be used when babys clinical condition is not improving despite CPAP
Theoretical advantages over CPAP Improved thoraco-abdominal synchrony Better chest wall stabilisation Reduced upper airway resistance Reduced work of breathing Specific modes of two-level CPAP (specific names vary with manufacturer) CPAP and apnoea CPAP with added advantage of apnoea monitoring via a sensor attached to abdomen Apnoea alarm is triggered when no breaths detected within set time-out period Biphasic Bi-level pressure respiratory support with or without apnoea monitoring Higher level pressure rise above baseline CPAP that is delivered intermittently at pressure, rate and inspiratory time set by clinician Not synchronised with respiratory effort Biphasic trigger (tr) Bi-level pressure respiratory support with inbuilt apnoea monitoring
CPAP NM 04.09.13
Higher level pressure rise above baseline CPAP at rate determined by, and in synchrony with, babys respiratory effort sensed through an abdominal sensor Pressure, inspiratory time and back-up rate set by clinician
Clinical use Biphasic Begin with CPAP pressure of 56 cm H2O Set peak inspiratory pressure (PIP) at 34 cm H2O above CPAP and rate 30 breaths/min Keep Ti and apnoea alarm delay at default setting If CO2 retention occurs, review baby and consider increase in rate and/or PIP Avoid over-distension and keep PIP to a minimum for optimum chest expansion Weaning By rate and pressure If rate >30 bpm, wean to 30 bpm Reduce MAP, by reducing PIP by 1 cm H2O every 1224 hr When baby breathing above 30 bpm change to Biphasic tr mode When MAP 56 cm H2O, change to CPAP Biphasic trigger Begin with CPAP pressure of 56 cm H2O with PIP at 34 cm H2O Keep Ti and apnoea alarm delay at default setting Set back-up rate at 30 bpm Weaning Reduce MAP by reducing PIP by 1 cm H2O every 1224 hr Once MAP 56 cm H2O, change to standard CPAP If deterioration occurs during weaning process, assess baby and consider returning to biphasic mode
3. BUBBLE CPAP
This is an alternative method of CPAP that may reduce work of breathing through facilitated diffusion Equipment Fisher & Paykel bubble CPAP system: delivery system: humidifier chamber, pressure manifold, heated circuit, CPAP generator patient interface: nasal tubing, nasal prongs, baby bonnet, chin strap Procedure Connect bubble CPAP system to baby as per manufacturers instructions Ensure appropriate size nasal prongs used Bubble CPAP nasal prongs are designed not to rest on nasal septum. Ensure prongs are not resting on the philtrum nor twisted to cause lateral pressure on septum, and allow a small gap between septum and prongs Commence at pressures of 5 cm H2O Bubble CPAP failure See CPAP failure in 1. STANDARD CPAP Before inferring bubble CPAP failure Ensure baby has been receiving bubble CPAP appropriately by checking for continuous bubbling in CPAP generator, lack of bubbling can result from pressure leaks in the circuit or baby
HFOV 201315
INDICATIONS
Rescue following failure of conventional ventilation (e.g. PPHN, MAS) To reduce barotrauma when conventional ventilator settings are high Airleak (pneumothorax, PIE) Less effective in non-homogenous lung disease Terminology Frequency MAP Amplitude High frequency ventilation rate (Hz, cycles per second) Mean airway pressure (cmH2O) Delta P or power is the variation around the MAP
Mechanism Oxygenation and CO2 elimination are independent Oxygenation is dependent on MAP and FiO2 Ventilation (CO2 removal) dependent on amplitude MAP provides constant distending pressure equivalent to CPAP, inflating the lung to constant and optimal lung volume, maximising area for gas exchange and preventing alveolar collapse in the expiratory phase The wobble superimposed around the MAP achieves alveolar ventilation and CO2 removal
MANAGEMENT
Preparation for HFOV If there is significant leakage around the ET tube (ETT), insert a larger one Optimise blood pressure and perfusion, complete any necessary volume replacement and start inotropes, if necessary, before starting HFOV Invasive blood pressure monitoring if possible Correct metabolic acidosis Ensure adequate sedation Muscle relaxants are not necessary unless already in use Initial settings on HFOV MAP Optimal (high) lung volume strategy (aim to maximise recruitment of alveoli) Low volume strategy (aim to minimise lung trauma)
If changing from conventional ventilation, set MAP 24 cmH2O above MAP on conventional ventilation If starting immediately on HFOV, start with MAP of 8 cmH2O and increase in 12 cmH2O increments until optimal SpO2 achieved Set frequency to 10 Hz Set MAP equal to MAP on conventional ventilation Set frequency to 10 Hz
Optimal (high) volume strategy preferred but consider low volume strategy when air leaks are present
Amplitude (delta P on SLE ventilator) Gradually increase amplitude until chest seen to wobble well Obtain early blood gas (within 20 min) and adjust settings as appropriate Change frequency only after discussion with consultant
HFOV 201315 Making adjustments once HFOV established Poor oxygenation Over-oxygenation Decrease MAP Adjust MAP (12 cmH2O) when Either (+/- 12 cmH2O)* FiO2 <0.4 Or Increase FiO2 Decrease FiO2
* both over and under inflation can result In hypoxia. If in doubt, perform chest X-ray
MONITORING
Amplitude maximal when chest wobbling, minimal when movement imperceptible Frequent blood gas monitoring (every 3060 min) in early stages of treatment as PaO2 and PaCO2 can change rapidly If available, transcutaneous TcPCO2
Chest X-ray Within 1 hour to determine baseline lung volume on HFOV (aim for 8 ribs at midclavicular line) if condition changes acutely and/or daily to assess expansion/ETT position, repeat chest X-ray
TROUBLESHOOTING ON HFOV
Chest wall movement Suction indicated for diminished chest wall movement indicating airway or ETT obstruction Always use an in-line suction device to maintain PEEP increase FiO2 following suctioning procedure MAP can be temporarily increased by 23 cmH2O until oxygenation improves Low PaO2 Suboptimal lung recruitment increase MAP consider chest X-ray Over-inflated lung reduce MAP: does oxygenation improve? Check blood pressure consider chest X-ray ETT patency check head position and exclude kinks in tube check for chest movement and breath sounds check there is no water in ETT/T piece Air leak/pneumothorax Transillumination see Transillumination of the chest guideline urgent chest X-ray High PaCO2 ETT patency and air leaks (as above) Increase amplitude, does chest wall movement increase? Increased airway resistance (MAS or BPD) or non-homogenous lung disease, is HFOV appropriate? Persisting acidosis/hypotension Over-distension Exclude air leaks; consider chest X-ray reduce MAP: does oxygenation improve? Spontaneous breathing Usually not a problem but can indicate suboptimal ventilation (e.g. kinking of ETT, buildup of secretions) or metabolic acidosis
HFOV 201315
WEANING
Reduce FiO2 to <0.4 before weaning MAP (except when over-inflation evident) When chest X-ray shows evidence of over-inflation (>9 ribs), reduce MAP Reduce MAP in 12 cm decrements to 89 cm 12 hrly or as tolerated If oxygenation lost during weaning, increase MAP by 34 cm and begin weaning again more gradually. When MAP is very low, amplitude may need increasing In air leak syndromes (using low volume strategy), reducing MAP takes priority over weaning the FiO2 Wean the amplitude in small increments (515%) depending upon PCO2 Do not wean the frequency When MAP <8 cmH2O, amplitude 2025 and blood gases satisfactory, consider switching to conventional ventilation or extubation to CPAP
Evidence Level: I Last amended July 2013 Last reviewed July 2013
Evidence Level: IV
Should HFOV be used as a first line treatment or as rescue treatment? A Cochrane Review (Bhuta, 2001) found no randomised controlled trial data to support the routine use of rescue HFOV in term or near term infants with severe pulmonary disease, and called urgently for more research on this topic. Only 1 trial (involving 81 infants) was identified in the review (Clark, 1994) and this found HFOV to be a safe and effective rescue technique in the treatment of neonates with respiratory failure in whom conventional ventilation fails. There were, however, no significant differences in the number of patients needing extracorporeal membrane oxygenation (RR 2.05, 95% CI 0.85-4.92), or in days on a ventilator, in oxygen or in hospital. Neither was there any evidence of a reduction in mortality at 28 days (RR 0.51, 95% CI 0.05-5.43). Another Cochrane Review by the same team (Bhuta, 1998) found a similar lack of evidence in preterm infants and recommended that any future use of HFOV as rescue therapy for preterm infants with severe RDS should be within randomized controlled trials and address important outcomes such as longer term pulmonary and neurological function. A BestBETS report (Shah, 2003) concluded that HFOV is probably not superior to conventional ventilation as primary mode of ventilation in preterm infants with respiratory distress syndrome for prevention of chronic lung disease or mortality at 36 weeks. However, use of HFOV is safe and not associated with increased risk of intraventricular haemorrhage or airleaks. This report included data from two multicentre, randomised trials in 500 infants (Courtney, 2002) and 400 infants (Johnson, 2002) respectively that appeared after the most recent Cochrane update. A prospective study in 77 infants (Ben Jaballah, 2006) found that HFOV as an early rescue intervention resulted in rapid and sustained decreases in mean airway pressure, F IO(2), OI, and P AO(2) Pa O(2) (P </= 0.01). The authors also identified a need for RCTs to confirm the perceived benefits of HFOV vs conventional ventilation.
Ben Jaballah N, Mnif K, Khaldi A, et al. High-frequency oscillatory ventilation in term and near-term infants with acute respiratory failure: early rescue use. Am J Perinatol 2006;23:403-11 Bhuta T, Clark RH, Henderson-Smart DJ. Rescue high frequency oscillatory ventilation vs conventional ventilation for infants with severe pulmonary dysfunction born at or near term. The Cochrane Database of Systematic Reviews 2001, Issue 1. Art. No.: CD002974 Bhuta T, Henderson-Smart DJ. Rescue high frequency oscillatory ventilation vs conventional ventilation for pulmonary dysfunction in preterm infants. The Cochrane Database of Systematic Reviews 1998, Issue 2. Art. No.: CD000438 Clark RH, Yoder BA, Sell MS. Prospective, randomized comparison of high-frequency oscillation and conventional ventilation in candidates for extracorporeal membrane oxygenation. J Pediatr 1994;124:447-54 Courtney SE, Durand DJ, Asselin JM, et al. High-frequency oscillatory ventilation versus conventional mechanical ventilation for very-low-birth-weight infants. N Engl J Med 2002;347:643-52 Johnson AH, Peacock JL, Greenough A, et al. High-frequency oscillatory ventilation for the prevention of chronic lung disease of prematurity. N Engl J Med 2002;347:633-42 Shah S. Is elective high-frequency oscillatory ventilation better than conventional mechanical ventilation in very low-birth-weight-infants? http://www.bestbets.org/cgi-bin/bets.pl?record=00586
Evidence Level: I (For No evidence) What should the starting settings be when commencing HFOV? Although frequencies between 3-50 Hz may be used during HFOV, 7-15 Hz is most commonly employed (Greenough, 1999). 10-20 Hz is also mentioned frequently as producing the best results (Chan, 1993; Hoskyns, 1991; Froese, 1987). New Zealand guidelines (Battin, 2001) recommend 10 Hz as an appropriate starting frequency.
Battin M. Newborn services clinical guidelines: High frequency ventilation (HFV). 2001 http://www.adhb.govt.nz/newborn/guidelines/respiratory/hfov/hfov.htm Chan V, Greenough A. Determinants of oxygenation during high frequency oscillation. Eur J Pediatr 1993;152:350-3
Froese AB, Butler PO, Fletcher WA, et al. High-frequency oscillatory ventilation in premature infants with respiratory failure: a preliminary report. Anesth Analg 1987;66:814-24 Greenough A, Roberton NR. Acute respiratory disease in the newborn. In: Rennie JM, Roberton NR, rd eds. Textbook of neonatology, 3 ed. Edinburgh, Churchill Livingstone, 1999. p569 Hoskyns EW, Milner AD, Hopkin IE. Combined conventional ventilation with high frequency oscillation in neonates. Eur J Pediatr 1991;150:357-61
Evidence Level: V Should a high volume strategy be used? A Cochrane Review (Henderson-Smart, 2003) included a subgroup of 3 trials in which a high volume strategy was used. In this subgroup, there was a significant reduction in the risk of chronic lung disease in survivors at 28-30 days (RR 0.53; 95% CI 0.36-0.76). In certain situations (gas trapping, severe lobar emphysema), a low-volume strategy appears to be more appropriate (Greenough, 1999).
Greenough A, Roberton NR. Acute respiratory disease in the newborn. In: Rennie JM, Roberton NR, rd eds. Textbook of neonatology, 3 ed. Edinburgh, Churchill Livingstone, 1999. p569 Henderson-Smart DJ, Bhuta T, Cools F, et al. Elective high frequency oscillatory ventilation versus conventional ventilation for acute pulmonary dysfunction in preterm infants. The Cochrane Database of Systematic Reviews 2003, Issue 4. Art. No.: CD000104
Evidence Level: I What are the indications for endotracheal suction during HFOV? No information with which to answer this question has been identified. How should an infant be weaned from HFOV? New Zealand guidelines (Battin, 2001) recommend the following: Reduce FiO2 to < 40% before weaning MAP (except when over-inflation is evident) Reduce MAP when chest x-ray shows evidence of over-inflation (> 9 ribs) Reduce MAP in 1 -2 cm increments to 8-9 In air leak syndromes (low volume strategy), reducing MAP takes priority over weaning the FiO2 Wean the amplitude in 4 cm H2O increments Do not wean the frequency Consider switching to conventional ventilation when MAP < 10 cm H2O, Amplitude 20 25 and blood gases satisfactory Suction is indicated for diminished chest wall movement indicating airway or ET tube obstruction or if there are visible/audible secretions in the airway Avoid in the first 24 hours of HFV, unless clinically indicated Avoid hand-bagging during the suctioning procedure: use PEEP protector and continue with patient on the ventilator Increase FiO2 following the suctioning procedure MAP may be temporarily increased 2-3 cm H2O until oxygenation improves A review (Mehta, 2004) states that Routine scheduled assessments of readiness for weaning and extubation may be more important than specific weaning modes and weaning criteria.
Battin M. Newborn services clinical guidelines: High frequency ventilation (HFV). 2001 http://www.adhb.govt.nz/newborn/guidelines/respiratory/hfov/hfov.htm Mehta NM, Arnold JH. Mechanical ventilation in children with acute respiratory failure. Curr Opin Crit Care 2004;10:7-12
Evidence Level: V
Should an infant be extubated directly from HFOV or weaned to conventional ventilation first? Weaning to conventional ventilation is common clinical practice (Courtney, 2002), although a technique known as sprinting (Seller, 2001) has been used in some difficult cases to achieve extubation directly from HFOV. Courtney SE, Durand DJ, Asselin JM, et al. High-frequency oscillatory ventilation versus conventional mechanical ventilation for very-low-birth-weight infants. N Engl J Med 2002;347:643-52 Seller L, Mullahoo K, Liben S, et al. Weaning to extubation directly from high-frequency oscillatory ventilation in an infant with cystic lung disease and persistent air leak: a strategy for lung protection. Respir Care 2001;46:263-6 Evidence Level: V Last amended August 2013 Last reviewed August 2013
Evidence Level: V What is a normal blood pressure for a neonate, at term and preterm? The normal physiologic blood pressure range ensuring appropriate organ perfusion in the neonate is unknown (Seri, 2001). Hypotension affects close to half of all ELBW infants, yet an agreement on its definition is still lacking (Fanaroff, 2006). A systematic review (Dempsey, 2007) failed to find evidence for a definitive threshold BP that was predictive of poor outcome. In low birthweight or preterm infants, the range of normal values is dependent on age in terms of weeks gestation and birthweight (Hegyi 1996; Hegyi 1994). New Zealand guidelines (Knight, 2000) suggest that, for VLBW infants, a good rule of thumb is to aim for the babys gestational age as the desired minimum mean blood pressure. A postal questionnaire sent to all 120 neonatal ICUs in Canada (Dempsey, 2006), which had a 79% return rate (95 replies), found that 25.8% relied on blood pressure values as the sole criteria for intervention. A blood pressure less than gestational age in weeks was the most common trigger for treatment. Premature neonates stabilize their BP after 14 days of life, and at this time they have a BP similar to that of term infants (Kent, 2009).
Dempsey EM, Barrington KJ. Treating hypotension in the preterm infant: when and with what: a critical and systematic review. J Perinatol 2007;27:469-78 Dempsey EM, Barrington KJ. Diagnostic criteria and therapeutic interventions for the hypotensive very low birth weight infant. J Perinatol 2006;26:677-81 Fanaroff AA, Fanaroff JM. Short- and long-term consequences of hypotension in ELBW infants. Semin Perinatol 2006;30:151-5 Hegyi T, Carbone MT, Anwar M, et al. Blood pressure ranges in premature infants. I. The first hours of life. J Pediatr 1994;124:627-33 Hegyi T, Anwar M, Carbone MT, et al. Blood pressure ranges in premature infants. II. The first week of life. Pediatrics 1996;97:336-42 Kent AL, Meskell S, Falk MC, et al. Normative blood pressure data in non-ventilated premature neonates from 28-36 weeks gestation. Pediatr Nephrol 2009;24:141-6 Knight D. Newborn Services Clinical Guidelines: Hypotension. http://www.adhb.govt.nz/newborn/Guidelines/Cardiac/Hypotension.htm Seri I, Evans J. Controversies in the diagnosis and management of hypotension in the newborn infant. Curr Opin Pediatr 2001;13:116-23
Evidence Level: V What is the role of clinical assessment (e.g. skin turgor, urine output) in deciding whether or not to treat at a specific blood pressure reading? No evidence can be identified with which to answer this question. There is general agreement that decisions to treat hypotension should be based on the general condition of the infant, not on the mean arterial blood pressure alone (Dasgupta, 2003), but no detail is given. Urine output is considered to be an unreliable indicator of hypotension (Dasgupta, 2003).
Dasgupta SJ, Gill AB. Hypotension in the very low birthweight infant: the old, the new, and the uncertain. Arch Dis Child Fetal Neonatal Ed 2003;88:F450-F454
Evidence Level: V Should IV fluid be used as a first line for the treatment of hypotension? If so, what type and how much? Although hypovolaemia is a relatively uncommon cause of hypotension in the sick preterm infant, moderate fluid replacement is a reasonable precaution and so usually the first line treatment (Dasgupta, 2002).Two RCTs, in 63 (So, 1997) and 41 infants (Oca, 2003) respectively, have demonstrated that saline 0.9% is as effective as 5% albumin for treating neonatal hypotension. Isotonic saline has the further advantages of being cheap, of carrying no infection risk, and of causing less fluid retention in the first 48 hours (So, 1997). A recommended amount to use is 10-20 ml/kg over 30 minutes (Dasgupta, 2002).
Dasgupta SJ, Gill AB. Hypotension in the very low birthweight infant: the old, the new, and the uncertain. Arch Dis Child Fetal Neonatal Ed 2003;88:F450-F454 Oca MJ, Nelson M, Donn SM. Randomized trial of normal saline versus 5% albumin for the treatment of neonatal hypotension. J Perinatol 2003;23:473-6 So KW, Fok TF, Ng PC, et al. Randomised controlled trial of colloid or crystalloid in hypotensive preterm infants. Arch Dis Child Fetal Neonatal Ed 1997;76:F43-F46
Evidence Level: II Should dopamine remain the first line drug treatment? A Cochrane review of 5 RCTs (Subhedar, 2003) concluded that dopamine was more successful than dobutamine for short-term treatment of hypotension, with fewer infants having treatment failure (RD 0.23; 95% CI 0.34 to 0.13). There was, however, no difference in mortality and no data on long term benefit or safety. A meta-analysis (Sassano-Higgins, 2011) found that dopamine increases mean arterial blood pressure (12 studies; N=163; r=0.88, 95% CI=0.76 to 0.94) and systolic blood pressure (8
studies; N=142; r=0.81, 95% CI=0.42 to 0.94). For the increase in blood pressure, dopamine administration was associated with a significantly greater overall efficacy than dobutamine (seven studies; N=251; r=0.26; 95% CI=0.20 to 0.32), colloid (two studies; N=67; r=0.60; 95% CI=0.41 to 0.74) and hydrocortisone (one study; N=28; r=0.40; 95% CI=0.034 to 0.67). CBF increased following dopamine administration (five studies; N=75; r=0.36; 95% CI=-0.059 to 0.67) and the increase in CBF was greater in hypotensive than normotensive preterm infants (eight studies; N=153; r=0.16; 95% CI=-0.0080 to 0.32). There were no statistically significant differences in adverse neurological outcome between dopamine and dobutamine (three studies; N=118; r=-0.13; 95% CI=-0.31 to 0.059), epinephrine (two studies; N=46; r=0.06; 95% CI=-0.23 to 0.34), colloid (two studies; N=80; r=0.0070; 95% CI=-0.218 to 0.23) or hydrocortisone administration (one study; N=40; r=-0.10; 95% CI=-0.40 to 0.22).
Sassano-Higgins S, Friedlich P, Seri I. A meta-analysis of dopamine use in hypotensive preterm infants: blood pressure and cerebral hemodynamics. J Perinatol 2011;31:647-55.nhs.uk Subhedar NV, Shaw NJ. Dopamine versus dobutamine for hypotensive preterm infants. The Cochrane Database of Systematic Reviews 2003, Issue 3. Art. No.: CD001242
Evidence Level: I What is the role of dobutamine? Dobutamine is generally used as a second line drug, in patients unresponsive to dopamine (Dasgupta, 2003). A range of doses from 5-20 mcg/kg/min has been used, and there is no clear evidence as to which of these is correct (Subhedar, 2003). New Zealand guidelines (Knight, 2000) suggest starting at the lower dose and increasing incrementally to the higher, after the dopamine dose has been increased to 10-20 mcg/kg/min without response.
Dasgupta SJ, Gill AB. Hypotension in the very low birthweight infant: the old, the new, and the uncertain. Arch Dis Child Fetal Neonatal Ed 2003;88:F450-F454 Knight D. Newborn Services Clinical Guidelines: Hypotension. http://www.adhb.govt.nz/newborn/Guidelines/Cardiac/Hypotension.htm Subhedar NV, Shaw NJ. Dopamine versus dobutamine for hypotensive preterm infants. The Cochrane Database of Systematic Reviews 2003, Issue 3. Art. No.: CD001242
Evidence Level: II What is the role of adrenaline (epinephrine)? A Cochrane review (Paradisis, 2004) identified only one on-going randomised study comparing adrenaline (epinephrine) with dopamine and this indicated that both agents significantly increased heart rate and mean BP, with no statistically significant effect on left or right ventricular outputs. No other outcomes were reported. The review concluded that there was insufficient data to make any recommendations, and called for larger trials to be carried out. A retrospective chart review in 31 very low birthweight infants not responding to dopamine (Heckmann, 2002) found that the mean arterial blood pressure (=7 (-1 to 13) mmHg, p=0.000001) and the heart rate (+10 (-10 to 42) bpm, p=0.000036) increased significantly in all cases in response to a continuous infusion of adrenaline (epinephrine) in doses of 0.05-2.6 mcg/kg(-1)/min within the first 24 hours. No decrease in urine output was recorded. An increase in metabolic acidosis was noted as a potential adverse effect.
Heckmann M, Trotter A, Pohlandt F, et al. Epinephrine treatment of hypotension in very low birthweight infants. Acta Paediatr 2002;91:566-70 Paradisis M, Osborn DA. Adrenaline for prevention of morbidity and mortality in preterm infants with cardiovascular compromise. The Cochrane Database of Systematic Reviews 2004, Issue 1. Art. No.: CD003958
Evidence Level: I What is the role of steroids? A single dose of steroids as rescue therapy is successful in most babies (Dasgupta, 2003). A randomised, double-blind, controlled trial in 20 premature infants not responding to dopamine and receiving adrenaline (epinephrine) infusion (Gaissmaier, 1999) found that 5/8
given dexamethasone (0.25 mg/kg) vs 1/9 given placebo (3 were excluded) were able to discontinue adrenaline (epinephrine). A randomised comparison between dopamine and hydrocortisone in 40 very low birthweight infants (Bourchier, 1997) found the two treatments broadly equivalent in efficacy. A retrospective review of 21 preterm infants given hydrocortisone as rescue therapy (Seri, 2001) noted a rapid increase in blood pressure (from 29.3 +/- 4.1 to 34.1 +/- 5.2 after 2 hours, rising to 41.8 +/- 6.6 mmHg after 6 hours). Steroids may also have a role in the prevention of hypotension in preterm infants with low cortisol levels (Subhedar, 2003). A Cochrane systematic review (Subhedar, 2007) concluded that in view of the scanty evidence for benefit and lack of long-term safety data, dexamethasone could not be recommended for routine use in preterm hypotension. A retrospective observational study in 117 infants (Baker, 2008) found that treatment with hydrocortisone increased the mean arterial pressure at 2, 6, 12 and 24 h after initiation, decreased the total inotrope dose at 6, 12 and 24 h and was associated with resolution of oliguria. A meta-analysis of 12 studies (Higgins, 2010) confirmed that hydrocortisone increases blood pressure (seven studies; N=144; r=0.71, 95%CI=0.18 to 0.92) and decreases the requirement for vasopressors (five studies; N=93; r=0.74, 95%CI=0.0084 to 0.96), but without demonstrating clear clinical benefit. A Cochrane systematic review of 4 studies in a total of 123 babies (Ibrahim, 2011) found that, in one study, persistent hypotension was more common in hydrocortisone treated infants as compared to those who received dopamine as primary treatment for hypotension (RR 8.2, 95% CI 0.47 to 142.6; RD 0.19, 95% CI 0.01 to 0.37). In two studies comparing steroid versus placebo, persistent hypotension (defined as a continuing need for inotrope infusion) was less common in steroid treated infants as compared to controls who received placebo for refractory hypotension (RR 0.35, 95% CI 0.19 to 0.65; RD -0.47, 95% CI - 0.68 to - 0.26; NNT = 2.1, 95% CI 1.47, 3.8). There were no statistically significant effects on any other short or long-term outcome. The authors concluded that: With long term benefit or safety data lacking, steroids cannot be recommended routinely for the treatment of hypotension in preterm infants.
Baker CF, Barks JD, Engmann C, et al. Hydrocortisone administration for the treatment of refractory hypotension in critically ill newborns. J Perinatol 2008;28:412-9 Bourchier D, Weston PJ. Randomised trial of dopamine compared with hydrocortisone for the treatment of hypotensive very low birthweight infants. Arch Dis Child Fetal Neonatal Ed 1997;76:F174-F178 Dasgupta SJ, Gill AB. Hypotension in the very low birthweight infant: the old, the new, and the uncertain. Arch Dis Child Fetal Neonatal Ed 2003;88:F450-F454 Gaissmaier RE, Pohlandt F. Single-dose dexamethasone treatment of hypotension in preterm infants. J Pediatr 1999;134:701-5 Higgins S, Friedlich P, Seri I. Hydrocortisone for hypotension and vasopressor dependence in preterm neonates: a meta-analysis. J Perinatol 2010;30:373-8 Ibrahim H, Sinha IP, Subhedar NV. Corticosteroids for treating hypotension in preterm infants. Cochrane Database of Systematic Reviews 2011, Issue 12. Art. No.: CD003662 Seri I, Tan R, Evans J. Cardiovascular effects of hydrocortisone in preterm infants with pressor-resistant hypotension. Pediatrics 2001;107:1070-4 Subhedar NV. Treatment of hypotension in newborns. Semin Neonatol 2003;8:413-23 Subhedar NV, Duffy K, Ibrahim H. Corticosteroids for treating hypotension in preterm infants. Cochrane Database of Systematic Reviews 2007, Issue 1. Art. No.: CD003662
Evidence Level: I What are the best parameters for assessing hypovolaemia in the neonate? Hypovolaemia is uncommon and also difficult to diagnose in the sick preterm infant, due to the unreliability of indicators such as urine output and capillary refill time (Dasgupta, 2003). In view of the danger of excessive volume expansion (Ewer, 2003), it has been suggested (Evans, 2003) that echocardiography should be used to define systemic blood flow.
Dasgupta SJ, Gill AB. Hypotension in the very low birthweight infant: the old, the new, and the uncertain. Arch Dis Child Fetal Neonatal Ed 2003;88:F450-F454 Evans N. Volume expansion during neonatal intensive care: do we know what we are doing? Semin Neonatol 2003;8:315-23 Ewer AK, Tyler W, Francis A, et al. Excessive volume expansion and neonatal death in preterm infants born at 27-28 weeks gestation. Paediatr Perinat Epidemiol 2003;17:180-6
Evidence Level: V What is the maximum dose of dopamine, dobutamine and adrenaline (epinephrine) in neonatal hypotension? The maximum dose of both dopamine and dobutamine is 20 mcg/kg/min (Subhedar, 2004; Osborn, 2002; Knight, 2000). The maximum dose of adrenaline (epinephrine) is 0.5 mcg/kg/min (Paradisis, 2004; Knight, 2000).
Knight D. Newborn Services Clinical Guidelines: Hypotension. 2000. http://www.adhb.govt.nz/newborn/Guidelines/Cardiac/Hypotension.htm Osborn D, Evans N, Kluckow M. Randomized trial of dobutamine versus dopamine in preterm infants with low systemic blood flow. J Pediatr 2002;140:183-91 Paradisis M, Osborn DA. Adrenaline for prevention of morbidity and mortality in preterm infants with cardiovascular compromise. The Cochrane Database of Systematic Reviews 2004, Issue 1. Art. No.: CD003958 Subhedar NV, Shaw NJ. Dopamine versus dobutamine for hypotensive preterm infants. The Cochrane Database of Systematic Reviews 2003, Issue 3. Art. No.: CD001242
Evidence Level: I Does neonatal hypotension increase the risk of developmental delay? A prospective cohort study in 945 infants (Logan, 2011) found that, after adjustment for potential confounders, no indicators of hypotension were associated with either a Bayley Mental Development Index (MDI) score or a Psychomotor Development Index (PDI) score of <70 at 24 months of age.
Logan JW, O'Shea TM, Allred EN, et al. Early postnatal hypotension and developmental delay at 24 months of age among extremely low gestational age newborns. Arch Dis Child Fetal Neonat Ed 2011;96:F321-8
Evidence Level: III Last amended September 2012 Last reviewed August 2013
Intubation 201315
INTUBATION
See also Difficult intubation guideline This procedure must be undertaken or supervised by an experienced person Do not attempt to carry out this procedure unsupervised unless you have demonstrated your competence
ELECTIVE INTUBATION
Use pre-medication Equipment Suction Oxygen with pressure limiting device and T-piece or 500 mL bag and appropriate size mask Endotracheal tubes (ETT) 3 sizes (diameter in mm): Weight of baby (g) <10001250 12503000 >3000 ETT 2.5 3.0 3.5
Hat for baby to secure tube, ETT fixing device, forceps and scissors Laryngoscopes x 2, stethoscope, oropharyngeal airway
Preparation Ensure cannula in place and working Ensure all drugs drawn up, checked, labelled and ready to give Check no contraindications to drugs Ensure monitoring equipment attached and working reliably If nasogastric tube (NGT) in place, aspirate stomach (particularly important if baby has been given enteral feeds) Premedication Give 100% oxygen for 2 min before drug administration Continue to give 100% oxygen until laryngoscopy and between attempts if more than one attempt necessary Drugs Choice of drugs depends on local practice Analgesia and muscle relaxation can improve likelihood of successful intubation Muscle relaxants Administer muscle relaxants only if you are confident that the team can intubate baby quickly. Do not use a muscle relaxant unless adequate analgesia has been given
PROCEDURE
Lift laryngoscope: do not tilt Avoid trauma to gums Cricoid pressure: by person intubating or an assistant Suction secretions only if they are blocking the view as this can stimulate the vagal nerve and cause a bradycardia and vocal cord spasm Insert ETT Advance ETT to desired length at the lips
Intubation 201315
General recommendation is to advance ETT no further than end of black mark at end of tube (2.5 cm beyond cords), but this length is far too long for extremely preterm babies See Table: Length of ETT for where approximate markings of the ETT should be at the lips
Table: Length of ETT Gestation of baby 2324 2526 2729 3032 3334 3537 3840 4143
Actual weight of baby (kg) 0.50.6 0.70.8 0.91.0 1.11.4 1.51.8 1.92.4 2.53.1 3.24.2
Length of ETT (cm) at lips 5.5 6.0 6.5 7.0 7.5 8.0 8.5 9.0
Remove stylet if used and check to ensure it is intact before proceeding If stylet not intact, remove ETT immediately and prepare to reintubate Check chest wall is moving and heart rate increases If uncertain about correct placement of ETT, use CO2 detector Auscultate chest to check for bilateral equal breath sounds if air entry unequal and louder on right side, withdraw ETT by 0.5 cm and listen again repeat until air entry equal bilaterally Do not leave baby with unequal air entry
stabilise tube using ETT fixation method in accordance with unit practice request chest X-ray: adjust ETT length so that tip is at level of T12 vertebrae and document on nursing chart and in babys hospital notes
Intubation failure Definition: Unable to intubate within 30 seconds If intubation unsuccessful, seek help from someone more experienced If there is a risk of aspiration, maintain cricoid pressure Continue mask ventilation with 100% oxygen until successful intubation achieved
INTUBATION DIFFICULT
BACKGROUND
In most babies, direct laryngoscopy results in a clear view of the larynx. The laryngeal view is classified by Cormack and Lehane as follows:
Visualisation of entire laryngeal aperture There should be no difficulty in intubation Visualisation of just the posterior portion of the laryngeal aperture May be slight difficulty Cricoid pressure should improve visualisation Visualisation of only the epiglottis Can result in severe difficulty; cricoid pressure may be helpful Visualisation of soft palate only, not even the epiglottis is visible Always difficult and usually accompanies obvious pathology but may also occur totally unexpectedly. Senior support may be required
MANAGEMENT PLAN
Difficult neonatal intubation may occur at or after delivery and may be: anticipated (e.g. Pierre Robin sequence, TreacherCollins, cleft lip and palate, Goldenhar syndrome, Apert/Crouzon syndrome, Downs syndrome) or unanticipated (e.g. subglottic stenosis, laryngeal atresia, laryngeal or tracheal webs, glottic oedema post extubation) Where difficult intubation is anticipated, ensure senior help is available before commencing (senior experienced middle grade, consultant or, if indicated, ENT consultant/anaesthetist)
Difficult airway pack Infant oropharyngeal airways (Guedel, sizes 000, 00, 0) ETT Size 24.5 with stylet for intubation ETT size 24.5 with scissors, to cut short for use as nasopharyngeal airway support ETT fixation equipment Straight bladed laryngoscopes for big and small baby Forceps Laryngeal mask airways (size 1) Size 2.54.5 endotracheal bougies for railroading ETT Video laryngoscope and blades if available on your unit CO2 detector e.g. Pedicap Can ventilate, cannot intubate (Good chest excursion and rising/good heart rate but baby still needs intubation) No more than 4 attempts at intubation (2 per individual resuscitator), to avoid laryngeal oedema and convert this into a cannot intubate, cannot ventilate scenario ventilate between attempts at intubation maximum 30 seconds per attempt to limit hypoxia Call for senior help If intubation attempts fail, stop. Continue either bag and mask ventilation or laryngeal mask airway ventilation until senior help available it is safer to maintain ventilation with mask ventilation with adequate chest expansion until help arrives, as baby is less likely to survive repeated unsuccessful ETT attempts Two further attempts by senior trainee/neonatologist
Try indirect laryngoscopy using video laryngoscope if available. If this fails, call for ENT support for rigid bronchoscopy or surgical tracheostomy, or ENT/anaesthetist for flexible fibrescope assisted intubation depending on your hospitals availability Use end tidal CO2 detectors (e.g. Pedicap) to confirm tracheal intubation
Cannot ventilate, cannot intubate Reconfirm the following, and call for senior help: neutral head position (overextension can limit vision) correct size face mask being used, create a tight seal use correct size oropharyngeal airway (Guedel airway): too big may cause laryngospasm and too small may worsen obstruction. (tip of the Guedel airway should reach the angle of the jaw when aligned with lip on side of face) For specific conditions (e.g. Pierre Robin sequence, micrognathia) nasopharyngeal airway may be useful. To make, take an ETT and shorten it by measuring distance between nasal tip and ear tragus. Choose a size that does not blanch the nares completely when inserted Laryngeal mask ventilation (smallest size = size 1, suitable for babies >1.5 kg) When senior help arrives: re-attempt intubation use a small towel roll under babys shoulder to improve vision use indirect laryngoscopy with video laryngoscope if available Call ENT or anaesthetist for support (ENT for rigid bronchoscopy or surgical tracheostomy, or anaesthetist for flexible fibrescope assisted intubation as above, depending on your hospitals availability) Use end tidal CO2 detector (e.g. Pedicap) to confirm tracheal intubation Prevent/anticipate difficult intubation/re-intubation For ventilated babies due for extubation, risk of difficult re-intubation can be reduced by preextubation dexamethasone to reduce cord oedema, especially in babies who had difficult initial intubations or chronic ventilatory course if ETT leak <1015%, consider dexamethasone Common problems with intubation Problem Oesophageal intubation Blade placed too deep, cords not visualised Tongue obscures vision
Cannot intubate
Action Retry with shallow blade insertion and use cricoid pressure Sweep tongue to left side using blade Use a more anterior lift Use straight blade (Miller) Ensure head not hyper-extended Use small towel roll under babys shoulders Do not panic Calmly maintain chest excursions through bag or T piece/face or laryngeal mask ventilation until help arrives Use Guedel oral airway if necessary Call for senior help
See senior support in the following situations Blind intubation: in small baby where poor visualisation due to size Laryngeal mask airway (size 1): can be inserted by juniors while awaiting senior support if trained Video laryngoscope: if available, to guide intubation through the cords Railroad technique: if laryngeal aperture narrow, insertion of stylet through cords, and railroading ETT over it: usually a two-person procedure and can be carried out under direct vision/blind, depending on visual field and equipment carefully insert a bougie through vocal cords, not more than 2 cm beyond aperture opening
keep bougie steady while colleague threads ETT over top end of stylet and into trachea. Note: using a stylet from the ETT pack carries risk of oesophageal/tracheal perforation Ultra-small fibre-optic bronchoscopy (if available locally): with railroading via bronchoscope Surgical tracheostomy: not undertaken by neonatal consultants seek ENT support NB: Prolonged procedure: additional dose of muscle relaxant can be used under senior guidance ensure venous access obtained support cardiac system with IV fluid boluses as required use inotropic agents as required, based on perfusion and blood pressure Keep baby warm using techniques supported by your local unit e.g. transwarmer, bubble wrap Empty stomach contents regularly while on face mask/T piece ventilation
Evidence Level: IV Is fentanyl superior to morphine for sedation? A small, double-blind, randomised trial in 20 preterm neonates (Pereira e Silva, 2007) compared intubation conditions (ease of laryngoscopy, position of the vocal cords, coughing, jaw relaxation and movement of the limbs) in two equal-sized groups given morphine or remifentanil. Conditions were rated as Excellent, Good or Poor. Morphine scored 0, 6, 4 respectively, compared to 6, 4, 0 for remifentanil. The authors concluded that conditions with remifentanil were significantly better (p = 0.0034) than with morphine. Although small, the study had a statistical power of 83%. Many units who give sedation use morphine, probably because of familiarisation rather than proven efficacy (Wyllie, 2008). Although fentanyl or morphine are the most commonly used sedative agents, there is a need for larger trials to determine the most effective regimen (Carbajal, 2007). A small randomised study in 30 infants (Cignacco, 2008) failed to demonstrate any pain relief from the use of morphine as measured by three assessment tools (Bernese Pain Scale for Neonates, Premature Infant Pain Profile and Visual Analogue Scale).
Carbajal R, Eble B, Anand KJ. Premedication for tracheal intubation in neonates: confusion or controversy? Semin Perinatol 2007;31:309-17 Cignacco E, Hamers JP, van Lingen RA, et al. Pain relief in ventilated preterms during endotracheal suctioning: a randomized controlled trial. Swiss Med Week 2008;138:635-45 Pereira e Silva Y, Gomez, RS, Marcatto JO, et al. Morphine versus remifentanil for intubating preterm neonates. Arch Dis Child Fetal Neonatal Ed 2007;92:F293-4 Wyllie JP. Neonatal endotracheal intubation. Arch Dis Child Educ Pract Ed 2008;93:44-9
How often do nasogastric tubes need changing routinely and why? Only two papers addressing this question were identified. The first (Rogahn, 1998) commented that no other previously published articles on the subject had been found. The author carried out a survey of 14 NICUs with 10 or more ventilators to establish their current practice on changing nasogastric tubes and whether or not this was evidence-based. Practice varied from changing the tubes daily to weekly (median 3 days), and was based on experience rather than evidence in all cases. The second paper (Mears, 2001) observed that little had changed since the publication of the previous paper in 1998. The author surveyed 36 neonatal units in the Thames region and found that, despite her own unit changing the tubes at 48 hour intervals, 64% of those surveyed changed them at 4-7 days, with no reported complications. The author also contacted the maker of the tubes used in her own unit (Vygon UK Ltd) for advice. This was that tubes may be safely left in situ for up to seven days, after which the integrity of the PVC used in their manufacture could not be guaranteed. The authors unit conducted an audit on the basis of this information and altered the frequency of tube change from 48 hours to 5 days, with some tubes being left in situ for up to 7 days if an infant was deemed too unwell to tolerate removal. A re-audit was performed 6 months after completion of the original study, which confirmed that leaving the tubes in situ for up to 7 days was not associated with any recorded adverse effects.
Mears M. Changing nasogastric tubes in the sick and preterm infant: a help or a hindrance? J Neonatal Nurs 2001;7:202-6 Rogahn J. Intragastric feeding in preterm infants: a survey of frequency of tube change. J Neonatal Nurs 1998;4:31-3
Evidence Level: V Last amended August 2013 Last reviewed August 2013
The Cormack-Lehane system (Cormack 1984), which classifies into four grades, views of the glottic opening during direct laryngoscopy, has been found anecdotally to be appropriate for neonates (Wheeler 2007). Wheeler (2007) argues however, that additional procedures are required to assess severity and that the four grade system is more useful as a means to facilitate communication of the degree of difficulty between providers and not as a screening tool for predicting a difficult airway at the bedside A number of researchers focusing on adult medicine including Yentis (1998) have argued for a more sensitive scoring system. Yentis, when comparing identification of difficult intubations among 663 adult patients using the Cormack-Lehane system and a modified system which divides grade 2 into 2a (part of the vocal cords are visible) and 2b (only arytenoids or very posterior origin of cords visible), found the latter system to be superior and more useful for anaesthetists.
Cormack, R. S.; Lehane, J. (1984). Difficult tracheal intubation in obstetrics. Anaesthesia 1984 39 (11): 11051111 Wheeler DS Assessment and Management of the pediatric airway In Wheeler DS, Wong HR and Shanley THP (eds.) Pediatric Critical Care Medicine : Basic Science and Clinical Evidence, 224-252. Yentis, S. M.; Lee, D. J. Evaluation of an improved scoring system for the grading of direct laryngoscopy. Anaesthesia 1998 53 (11): 10411044
Evidence Level: III Last amended August 2013 Last reviewed August 2013
NITRIC OXIDE
INDICATIONS
Persistent pulmonary hypertension of the newborn in term babies, proven on clinical grounds or by echocardiography see Persistent pulmonary hypertension of the newborn (PPHN) guideline Oxygen Index >25 Initiate treatment with nitric oxide (NO) only after discussion with on-call consultant
CAUTIONS
Preterm baby (not routinely recommended following Cochrane review 2007) Grade 4 intraventricular haemorrhage (IVH) Recent pulmonary haemorrhage Platelets <50 x 109/L
CONTRAINDICATIONS
Congenital heart disease
MONITORING
Use SpO2 to monitor response Blood gases 4-hrly Monitor methaemoglobin before starting NO, 1 hr after starting and then 12-hrly. Maximum proportion of total haemoglobin is reached after 8 hr normal <1% 23% is acceptable 4% requires action: reduce NO and repeat in 1 hr - if still >4%, stop NO - if >6%, treat with methylene blue 1 mg/kg IV over 1 hr NO inhibits platelet function and can trigger bleeding if baby has bleeding problem or thrombocytopenia. Check FBC daily while baby receiving NO
Stenson BJ, Tarnow-Mordi, WO, Darlow, BA et al. Oxygen saturation and outcomes in preterm infants N Engl J Med 2013 368:2094-2104. Tlucek PS, Corff KE, Bright BC, et al. Effect of decreasing target oxygen saturation on retinopathy of prematurity. J Am Assoc Pediatr Ophthalmol Strabismus 2010;14:406-11
Evidence Level: I Last amended August 2013 Last reviewed August 2013
OXYGEN ON DISCHARGE
OBJECTIVE
To put an effective plan in place to allow oxygen-dependent babies to be cared for safely at home
Discharge checklist Discharge plan implemented See Discharge guideline Plan discharge for beginning of week to ensure staff available in event of problems Oxygen supply and equipment installed in the home Baby will go home on prescribed amount of oxygen; this may be altered on direction of medical or nursing staff, or in event of emergency GP and other relevant professionals (also fire and electricity companies, although oxygen supplier usually does this) informed of date and time of discharge Community team briefed to arrange home visit well in advance of discharge to ensure conditions suitable and equipment correctly installed Parents/carers trained to care for baby safely at home and have support contact numbers
AFTERCARE
As oxygen dependant babies (e.g. chronic lung disease) are at increased risk of contracting respiratory syncytial virus (RSV), give palivizumab and influenza vaccine (see Immunisations guideline and Palivizumab guideline) Community team will advise when baby ready to be weaned off oxygen
Oxygen 201315
PRINCIPLES
Usual unit target range SpO2 9295% for preterm babies <36 weeks corrected gestational age who are breathing on supplemental oxygen If different target range, see right-hand column of table below Prescribe oxygen on babys drug chart specifying target range
Setting alarm limits If currently <36 weeks corrected age Target range SpO2 9295% Babies breathing supplemental oxygen Low alarm at 88% and high alarm at 98% Babies breathing air Low alarm at 88% and high alarm at 100%
If currently >36 weeks corrected age OR born >34 weeks Target SpO2 95% Babies breathing supplemental oxygen Low alarm at 92% and high alarm at 99% Babies breathing air Low alarm at 92% and high alarm at 100%
Units using different saturation range: Set alarm at 35% below and above target range
Oxygen 201315
significant desaturations (<70%), double baseline inspired oxygen (increase by at least 20%) until SpO2 increases to 90%, then wean rapidly to within 3% of baseline inspired oxygen
Handling or procedures If history of significant desaturation with handling or procedures, increase inspired oxygen by 5 10% before handling or procedure increase PEEP (or PIP if CO2 rising) by 12 cm for a few minutes After procedure, once SpO2 stabilises, wean inspired oxygen rapidly back to baseline Labile cases Some sick babies will be particularly labile and it is challenging to maintain SpO2 in target range. It is important to remain patient and continue to follow guidance above In rare cases, individualised adjustments to alarm settings may be necessary after discussion with medical team
PPHN 201315
CLINICAL FEATURES
Usually present in first 12 hr of life SpO2 <95% or hypoxia (PaO2 <6 kPa) Mimics cyanotic heart disease CVS: tricuspid regurgitant murmur, right ventricular heave, loud second heart sound and systemic hypotension Idiopathic PPHN: respiratory signs mild or absent Secondary PPHN: features of underlying disease
INVESTIGATIONS
Blood gases show primary hypoxaemia (PaO2 <6 kPa) with oxygenation index >20, underlying disease will cause a mixed picture SpO2 >5% difference in pre- and post-ductal saturations (pre > post) Hyperoxia test (100% oxygen for 5 min) SpO2 may improve to 95% in early stage or may not respond, i.e., staying <95% in established PPHN (as in cyanotic heart disease) Chest X-ray: normal or minimal changes in idiopathic PPHN Electrocardiograph (ECG) often normal can sometimes show tall P waves in lead 2/V1/V2 or features of RVH (i.e. tall R waves V1/V2, right axis deviation or upright T waves in V1/V2) Echocardiogram (although not mandatory), will be useful, if available: to exclude cyanotic heart disease to assess pulmonary hypertension one or more of the following confirm PPHN in presence of normal cardiac structures: a) significant tricuspid regurgitation (gradient + 5 = estimated systolic pulmonary pressure) b) dilated right side of heart c) right-to-left shunting across PFO and/or PDA d) pulmonary regurgitation to evaluate ventricular function
PPHN 201315
MANAGEMENT
Once PPHN suspected, inform and involve consultant neonatologist immediately Aims of management are to: decrease pulmonary vascular resistance using ventilation and vasodilators to increase systemic blood pressure with volume expanders and vasopressors to treat any underlying condition
General measures Minimal handling, nurse in quiet environment Secure arterial and central venous access, see Arterial line insertion guideline or Umbilical artery catheterisation and Umbilical venous catheterisation guidelines Maintain normal temperature, biochemistry and fluid balance Keep Hb >120 g/L Give antibiotics (sepsis particularly GBS is difficult to exclude) Surfactant may be beneficial in MAS or GBS sepsis, discuss with consultant If perfusion poor, fluid bolus (10 mL/kg sodium chloride 0.9% or if coagulopathy, fresh frozen plasma) Correct acidosis Once PaCO2 in acceptable range (i.e.<6 kPa), correct metabolic acidosis to maintain pH 7.357.45 using half or full correction with sodium bicarbonate. If repeated correction necessary, slow bicarbonate infusion of calculated dose can be given over 612 hr (see Neonatal Formulary) Ventilation Positive pressure ventilation Use muscle relaxants and sedation in babies with high ventilatory and oxygen requirements and in asynchrony on ventilator PaCO2 4.55.5 kPa (accept up to 6 kPa in parenchymal lung disease) avoid hypocarbia start on FiO2 1.0 and reduce as tolerated. Maintain SpO2 at 96100% and PaO2 at 1012 kPa High frequency oscillatory ventilation (HFOV) may improve oxygenation (see High frequency oscillatory ventilation guideline) Monitor oxygenation index (OI) OI = mean airway pressure (cmH2O) x FiO2 x 100 postductal PaO2 (kPa) x 7.5
Inotropes Use inotropes early Maintain systemic mean BP 4550 mmHg in term baby and systemic systolic BP 6070 mmHg or above pulmonary pressures (if available by echo) see Hypotension guideline Pulmonary vasodilatation If OI >25, use nitric oxide (NO) selective pulmonary vasodilator (see Nitric oxide guideline) If NO not available, use magnesium sulphate and/or epoprostenol (prostacycline) both can cause systemic hypotension Criteria for discussion with extracorporeal membrane oxygenation (ECMO) team Baby born 34 weeks or 2 kg with PPHN not responding or OI >30 despite NO, inotropes and/or HFOV OR unable to maintain BP targets with inotropes or persistent need for adrenaline/noradrenaline infusion OR no significant progression after 3 days Criteria for ECMO Baby born 34 weeks or 2 kg with PPHN oxygenation index >40
PPHN 201315 reversible lung disease (<10 days high pressure ventilation) no lethal congenital abnormalities
Exclusion criteria (if in doubt, discuss with ECMO team) Major intracranial haemorrhage Irreversible lung injury or mechanical ventilation >10 days Lethal congenital or chromosomal anomalies Severe encephalopathy Major cardiac malformation A baby accepted for transfer to ECMO centre will be retrieved by ECMO team ECMO centre will need: a cranial ultrasound scan maternal blood for group and crossmatching (check with ECMO centre) a referral letter copies of hospital notes/chest X-rays Outreach ECMO ECMO team may decide to start outreach ECMO in local neonatal intensive care unit before transfer to ECMO unit. Check with ECMO team regarding diathermy unit and number of packed cell units needed for procedure
Referral for ECMO Contact your ECMO co-ordinator/fellow at nearest ECMO Centre: Glenfield Hospital, Leicester on 0116 287 1471 Great Ormond Street Hospital, London on 0207 829 8652 Freeman Hospital, Newcastle, 0191 223 1016 Yorkhill Hospital, Glasgow, 0141 201 0000
PULMONARY HAEMORRHAGE
RECOGNITION AND ASSESSMENT
Definition Acute onset of bleeding from endotracheal tube (ETT) associated with cardiorespiratory deterioration and changes on chest X-ray Significant pulmonary haemorrhage is most likely to represent haemorrhagic pulmonary oedema. Differentiate from minor traumatic haemorrhage following endotracheal suction Risk factors Preterm babies Respiratory distress syndrome (RDS) Large patent ductus arteriosus (PDA) Excessive use of volume (>20 mL/kg) in first 2448 hr in babies 28 weeks gestation Coagulopathy Sepsis IUGR Grade 3 hypoxic ischaemic encephalopathy (HIE) Symptoms and signs Apnoeas, gasping respirations, desaturations Tachycardia >160/min, bradycardia, hypotension, shock, PDA, signs of heart failure Widespread crepitations, reduced air entry Pink/red frothy expectorate, or frank blood from oropharynx or ETT if intubated Investigations Blood gas (expect hypoxia and hypercarbia with mixed acidosis) FBC, clotting Chest X-ray (usually shows classic white-out with only air bronchogram visible but may be less striking and resemble RDS)
IMMEDIATE TREATMENT
Basic resuscitation Respiratory Intubate and ventilate Sedate and give muscle relaxant PEEP 68 cm, even higher PEEP of 1012 cm of water sometimes required to control haemorrhage PIP to be guided by chest expansion and blood gases Long inspiratory times (0.5 sec may be needed) Endotracheal suction (try to avoid but consider in extreme cases to reduce risk of ETT blockage) Ensure adequate humidification Avoid chest physiotherapy Establish arterial access
Fluid management If hypovolaemic, restore circulating volume over 30 min with 10 mL/kg sodium chloride 0.9% or O-negative packed cells if crystalloid bolus already given. Beware of overloading (added volume can be detrimental to LV failure) If not hypovolaemic and evidence of left ventricular failure, give furosemide 1 mg/kg IV Correct acidosis (see Neonatal Formulary) If PDA present, restrict fluids to 6080 mL/kg/24 hr in acute phase Further blood transfusion, vitamin K administration and FFP to be guided by haemoglobin concentration, PT and APTT (see Transfusion of red blood cells guideline and Coagulopathy guideline) Hypotension/cardiac dysfunction If still hypotensive or evidence of cardiac dysfunction after fluid resuscitation, treat hypotension with inotropes (See Hypotension guideline) Infection If infection suspected, request septic screen and start antibiotics
SUBSEQUENT MANAGEMENT
Once baby stable Inform on-call consultant Speak to parents Document event in case notes Consider single extra dose of natural surfactant in babies with severe hypoxaemia or oxygenation index >20 If PDA suspected, arrange echocardiogram (see Patent ductus arteriosus guideline) Perform cranial ultrasound scan to exclude intracranial haemorrhage as this may influence management see Cranial ultrasound scans guideline
SIPPV 201315
Most likely cause of baby fighting ventilator is ASYNCHRONY (see MANAGEMENT OF ASYNCHRONY)
SIPPV 201315
To improve oxygenation Increase FiO2 Rule out pneumothorax Increase PIP and/or PEEP Increase Tinsp (not more than 0.4 sec) To decrease PaCO2 Rule out pneumothorax Increase PIP Check if baby triggering adequately. If not, try shortening Tinsp, or increasing back-up rate Low PaCO2 Decrease PIP Decrease back-up rate if >35/min In a vigorous hypocapnic baby, transfer to SIMV (synchronised intermittent mandatory ventilation) at a rate of at least 20/min
GENERAL SUPPORT
Monitor SpO2 continuously Check arterial blood gases at least 46 hrly depending on stage of disease In babies successfully ventilated in SIPPV mode, sedation is unnecessary Remember, most common cause of baby fighting ventilator is ASYNCHRONY. Always carry out checks and adjustments (see MANAGEMENT OF ASYNCHRONY) If baby still fights ventilator, consider morphine bolus (100 microgram/kg) If baby continues to fight ventilator, use continuous sedation and change to conventional ventilation (SIMV) mode (see Conventional ventilation guideline) Do not use muscle relaxants at any stage unless, despite carrying out above checks, baby cannot be ventilated. If muscle relaxants necessary, revert to conventional ventilation (see Ventilation guideline)
NURSING OBSERVATIONS
While baby on SIPPV, hourly observations Back-up rate set Babys own respiratory rate Tidal volume (Vt in mL) Minute ventilation (MV in 1/min) If problems, check Synchrony between baby and ventilator Excessive water droplets in ventilator tubing Flow graph for evidence of blocked tube or excessive Ti
MANAGEMENT OF ASYNCHRONY
Checklist Is endotracheal tube (ETT) patent (look at flow graph and Vt) Is Tinsp too long? (is baby exhaling against ventilator?), if so shorten Tinsp to 0.240.3 sec Is back-up rate too high? If so, consider dropping to 3035 breaths/min Is there water condensation in ventilator tubing? If all above fails, consider morphine bolus (100 microgram/kg) over 35 min If baby still continues to fight ventilator, use continuous sedation and revert to SIMV
SIPPV 201315
Surfactant 201315
INDICATIONS
Prophylaxis (administration within 15 min of birth) Babies born <28 weeks gestation If requiring intubation for respiratory support during resuscitation/stabilisation or whose mothers have not had antenatal steroids, give surfactant as prophylaxis Consider INSURE technique. Assess the readiness of very preterm babies for extubation based on clinical parameters Early rescue treatment Babies born <27 weeks gestation If intubation for respiratory distress required and need FiO2 >0.30, give surfactant Babies born 27 weeks gestation If requiring intubation and needing FiO2 >0.40, give surfactant Other babies that can be considered for surfactant therapy (after discussion with consultant) Ventilated babies with meconium aspiration syndrome (may need repeat dose after 68 hr) Term babies with pneumonia and stiff lungs
EQUIPMENT
Natural surfactant, Poractant alfa (Curosurf) 200 mg/kg (80 mg/mL) round to the nearest whole vial; prophylaxis and rescue doses can differ, check dose with local policy Sterile gloves Trach Care Mac catheter [do not cut nasogastric tube (NGT)]
PROCEDURE
Preparation Calculate dose of surfactant required and warm to room temperature Ensure correct endotracheal tube (ETT) position check ETT length at lips listen for bilateral air entry and look for chest movement if in doubt, ensure ETT in trachea using laryngoscope and adjust to ensure bilateral equal air entry chest X-ray not necessary before first dose Refer to manufacturers guidelines and Neonatal Formulary Invert surfactant vial gently several times, without shaking, to re-suspend the material Draw up required dose
Surfactant 201315
Administer via Trach Care Mac device (note: it is no longer acceptable to administer surfactant via an NGT as this contravenes European conformity (CE marking) and NPSA guidance
Instillation With baby supine, instil prescribed dose down tracheal tube; give 2 boluses of Poractant alfa Wait for recovery of air entry/chest movement and oxygenation between boluses Post-instillation care Do not suction ETT for 8 hr (suction is contraindicated in Surfactant Deficiency Disease for 48 hr) Be ready to adjust ventilator/oxygen settings in response to changes in chest movement, tidal volume and oxygen saturation. Use of volume-target ventilation can facilitate responsiveness to rapid changes in lung compliance following surfactant instillation Take an arterial/capillary blood gas within 30 min
SUBSEQUENT MANAGEMENT
If baby remains ventilated at FiO2 >0.3 with a mean airway pressure of >7 cm of water, give further dose of surfactant Poractant alfa after 612 hr Third dose should be given only at the request of the attending consultant
DOCUMENTATION
For every dose given, document in case notes: indication for surfactant use time of administration dose given condition of baby pre-administration, including measurement of blood gas unless on labour ward when saturations should be noted response to surfactant, including measurement of post-administration blood gas and saturations reasons why second dose not given, if applicable reason(s) for giving third dose if administered Prescribe surfactant on drug chart
Evidence Level: II What surfactant preparations are recommended? Although natural and synthetic surfactants are both effective, a Cochrane review of 11 trials (Soll, 2001) concluded that natural surfactant rather than synthetic surfactant resulted in a significant reduction in the risk of pneumothorax (typical relative risk 0.63, 95% CI 0.53, 0.75; typical risk difference -0.04, 95% CI -0.06, -0.03) and the risk of mortality (typical relative risk 0.87, 95% CI 0.76, 0.98; typical risk difference -0.02, 95% CI -0.05, 0.00). Natural surfactant extract is associated with a marginal increase in the risk of intraventricular hemorrhage (typical relative risk 1.09, 95% CI 1.00, 1.19; typical risk difference 0.03, 95% CI 0.00, 0.06), but no increase in grade 3 to 4 intraventricular haemorrhage (typical relative risk 1.08, 95% CI 0.92, 1.28; typical risk difference 0.01, 95% CI -0.01, 0.03). The meta-analyses supported a marginal decrease in the risk of bronchopulmonary dysplasia or mortality associated with the use of natural surfactant preparations (typical relative risk 0.95, 95% CI 0.90, 1.01; typical risk difference -0.03, 95% CI 0.06, 0.00).
Soll RF, Blanco F. Natural surfactant extract versus synthetic surfactant for neonatal respiratory distress syndrome. The Cochrane Database of Systematic Reviews 2001, Issue 2. Art. No.: CD000144
Evidence Level: I What advantages and disadvantages does surfactant have? The benefits of surfactant administration (particularly natural preparations as opposed to synthetic) have been demonstrated in several Cochrane reviews (Stevens, 2007; Bahadue 2012; Soll 1997). RCPCH guidance (Anon, 2000), which does not seem to have been recently updated, states that The benefits of surfactant treatment for babies as small as 500g is thought to outweigh the risks. A systematic review of 13 RCTs in a total of 2218 treated and 2090 control infants (Sinn, 2002) found a lower rate of mild disability in the treated group at follow-up at 1 year
(OR 0.79; 95% CI 0.66-0.95). The treated group also showed a reduction in combined adverse outcome (death or severe disability) at 1 year (OR 0.8; 95% CI 0.72-0.89). Surfactant treatment has, however, failed to have a significant impact on the incidence of chronic lung disease in survivors (Ainsworth, 2002). Recorded side effects of surfactant treatment include increased cerebral blood flow velocity, which, due to the lack of cerebral vascular autoregulation in many sick preterm infants, can lead to intraventricular haemorrhage or periventricular leukomalacia. Evidence for this is, however, equivocal (Hentsche, 2002).
Ainsworth SB. Surfactant therapy for respiratory distress syndrome in premature neonates: a comparative review. Am J Respir Med 2002;1:417-33 Anon. Management of neonatal respiratory distress syndrome. London: Royal College of Paediatrics and Child Health, 2000. Hentsche R, Jorch G. Acute side effects of surfactant treatment. J Perinat Med 2002;30:143-8 Sinn JK, Ward MC, Henderson-Smart DJ. Developmental outcome of preterm infants after surfactant therapy: systematic review of randomized controlled trials. J Paediatr Child Health 2002;38:597-600 Soll RF. Prophylactic natural surfactant extract for preventing morbidity and mortality in preterm infants. The Cochrane Database of Systematic Reviews 1997, Issue 4. Art. No.: CD000511 Stevens TP, Harrington EW, Blennow M, et al. Early surfactant administration with brief ventilation vs. selective surfactant and continued mechanical ventilation for preterm infants with or at risk for respiratory distress syndrome. Cochrane Database of Systematic Reviews 2007, Issue 4. Art. No.: CD003063 Bahadue FL, Soll R. Early versus delayed selective surfactant treatment for neonatal respiratory distress syndrome. Cochrane Database of Systematic Reviews 2012, Issue 11. Art. No.: CD001456
Evidence Level: I How many doses of surfactant are recommended? An updated Cochrane systematic review (Soll, 2009) of 2 RCTs comparing single with multiple doses of surfactant showed a reduction in the risk of pneumothorax (RR 0.51, 95% CI 0.30-0.88) and a trend towards a reduction in mortality (RR 0.63, 95% CI 0.39-1.02) associated with the use of multiple doses. The review also identified an additional study of multiple vs. single dose synthetic
surfactant in infants at high risk of respiratory distress syndrome. This reported a decrease in necrotizing enterocolitis (relative risk 0.20, 95% CI 0.08, 0.51; risk difference-0.05, 95% CI -0.07, -0.02) and mortality (relative risk 0.56, 95% CI 0.39, 0.81; risk difference-0.07, 95% CI -0.12, -0.03)
The OSIRIS (Open Study of Infants at High Risk of or with Respiratory Insufficiency the role of Surfactant) trial (Anon, 1992) randomised 2690 infants to either 2 doses of surfactant 12 hours apart, or the option of third and fourth doses at 12-36 hour intervals if signs of RDS persisted or recurred. 4067 infants who later developed RDS were also added, giving a total of 3376 infants allocated up to four doses (45% of whom received more than two). No evidence of improved outcomes associated with more than 2 doses was found.
Anon. Early versus delayed neonatal administration of a synthetic surfactant the judgment of OSIRIS. Lancet 1992;340:1363-9 Soll R, zek E. Multiple versus single doses of exogenous surfactant for the prevention or treatment of neonatal respiratory distress syndrome. Cochrane Database of Systematic Reviews 2009, Issue 1
Evidence Level: I Last amended August 2013 Last reviewed August 2013
Transcutaneous 201315
(Adapted with permission, Guys and St Thomas NHS Trust nursing guideline)
INTRODUCTION
In babies requiring assisted ventilation, it is essential to monitor arterial partial pressure of oxygen (PaO2) and carbon dioxide (PaCO2) to ensure ventilation adequate Transcutaneous monitoring allows constant measurement (TcCO2 and TcO2) Use this guideline to set up and safely use transcutaneous monitoring equipment
Clinical indications Monitoring adequacy of arterial oxygenation and/or ventilation Nursing critically ill or unstable baby Advantages Reduction in number of blood gas measurements Immediate recognition of need for ventilation adjustment Potential problems Tissue injury (e.g. erythema, blisters, burns, and skin tears) as a result of failure to change site frequently enough (23 hrly) according to local protocol Inadequate measurement resulting from incorrect set-up
EQUIPMENT
Transducer: insert at end position of rack for easy accessibility Membranes Electrolyte solution Adhesive fixation rings Recalibration machine
Probe placement and application of fixation rings Preferred sites: if baby nursed prone: the back if baby nursed supine: the chest Avoid bony surfaces: use soft tissues (e.g. abdomen, buttock, thigh) and avoid placing over liver as this can prevent accurate clinical assessment of liver size Ensure chosen site is clean and dry Peel adhesive protection layer off ring Place ring on chosen site pressing gently on centre of ring before running finger around outside. Ensure effective seal as this will affect accuracy of measurement Place three drops of contact fluid in centre of ring Remove transducer from module into ring and turn one-quarter clockwise to secure
Transcutaneous 201315
Blood gas sampling Take blood gas 20 min after commencing transcutaneous monitoring to allow comparison between monitoring values and arterial partial pressures of O2 and CO2 levels, as discrepancy can occur If transcutaneous monitoring values change suddenly, check contact is in place before making ventilator changes. If any doubt about accuracy of values, check blood gas before making ventilator changes Changing measurement site Babies <29 weeks: change 2-hrly Babies 29 weeks: change 3-hrly Unscrew transducer before removing fixation rings Remove fixation rings when repositioning baby from supine to prone and vice-versa to avoid pressure-sore from lying on rings Remove rings 12-hrly on babies <29 weeks and 24-hrly on babies 29 weeks Calibration of membrane See Figure 15 Indications Transducer membrane has been replaced Monitor displays calibration required Measurement values in doubt Applying to a new baby Changing measurement site Ensure calibrator is turned off after use. Do not dispose of connecting tube. Contact technicians when calibrating gas empty Changing transducer membranes See Figure 610 All staff responsible for ventilated babies can change transducer membranes Indications When using a new transducer or if transducer has dried out For each new baby When membrane crinkled, scratched or damaged After 5 days continuous use Procedure Wash and dry hands To remove O-rings, unscrew protective cap from transducer and hook O-ring remover under them Remove both clear plastic membranes with your fingers To ensure correct values, clean transducer head, including grove and rim, with absorbent paper to remove all old electrolyte solution Apply approximately two drops of electrolyte solution to transducer head Press transducer head downward into an unused membrane replacer until replacer reacts as far as it can and a click is heard
Transcutaneous 201315
Evidence Level: IV Last amended August 2013 Last reviewed August 2013
Ventilation 201515
VENTILATION CONVENTIONAL
INTRODUCTION
Oxygenation Increase oxygenation by increasing: FiO2 peak end expiratory pressure (PEEP) peak inspiratory pressure (PIP) inspiratory time (Tinsp) CO2 Reduced by: increased PIP occasionally by reducing excessive PEEP (beware of effect on oxygenation)
VENTILATOR PARAMETERS
Peak inspiratory pressure (PIP) Use lowest possible PIP to achieve visible chest expansion and adequate gas exchange on blood gas analysis To minimise lung injury from barotrauma and inadvertent over-distension, avoid excessive PIP Need for higher PIP [e.g. mean arterial pressure (MAP) >12 cm] should lead to consideration of high frequency oscillatory ventilation (HFOV) see High frequency oscillatory ventilation guideline Peak end expiratory pressure (PEEP) Use a PEEP of at least 3 cm and increase incrementally up to 8 cm for improving oxygenation but when PEEP >6 cm is necessary, take senior advice Inspiratory time (Tinsp) Usually between 0.30.4 sec Avoid Tinsp >0.5 sec except in term babies with parenchymal lung disease where a Tinsp up to 1 sec may be used Rate Fast-rate (60/min) ventilation is associated with fewer air leaks and asynchrony compared to slow (2040/min) rates If rate >70/min required, HFOV may be a more appropriate option see High frequency oscillatory ventilation guideline Flow Flow of 58 L/min is generally sufficient Consider higher flows at faster ventilatory rates or shorter inspiratory times SLE ventilator has a fixed flow (5 L/min) that cannot be altered Tidal volume (VT) Target is 46 mL/kg
SETTING UP VENTILATOR
Setting 1 When an admission of a preterm baby requiring ventilatory support (for recurrent apnoea, see Setting 2) rate 60/min PIP 1618 cmH2O
Ventilation 201515
PEEP 4 cmH2O Tinsp 0.30.4 sec FiO2 0.40.6 flow 68 L/min (not applicable to SLE) Adjust ventilatory settings depending on chest movement, SpO2, and measured VT Sample blood gas within 30 min of commencing ventilatory support
Setting 2 For babies with normal lungs requiring supportive ventilation such as term babies with respiratory depression (asphyxia or drugs), babies with neuromuscular disorders or, in the post-operative period, and preterm babies with recurrent apnoea, set ventilator at following settings: rate 2040/min PIP/PEEP 1012/3 cmH2O Tinsp 0.350.4 sec FiO2 0.210.3
Low PaO2/SpO2
Ventilation 201515
High PaO2
High PaCO2
Low PaCO2
consultant (may need HFOV/iNO) Decrease FiO2 (unless already in air) Decrease PEEP (if >5 cm) Decrease PIP (especially if PaCO2 is also low) Shorten Tinsp Exclude airleak/displaced or blocked ETT Increase PIP Decrease PEEP (only if oxygenation adequate and PEEP >5 cm) Increase rate Decrease PIP Decrease rate Exclude displaced/blocked ETT Exclude air leak Increase PIP Consider further surfactant If no response, consider HFOV see High frequency oscillatory ventilation guideline
All ventilator changes must be prescribed and signed for on the intensive care chart Load all babies <30 weeks gestation with caffeine on day 0 with maintenance doses thereafter. Do not delay loading until the weaning stage
WEANING
While weaning baby off ventilator: reduce PIP (usually by 12 cm) until MAP of <7 cm reached thereafter, reduce rate to 1020/min, usually in decrements of 510 breaths/min
Extubation Extubate babies of <30 weeks gestation onto nasal CPAP for mode, see CPAP guideline more mature babies with no significant chest recessions can be extubated directly into incubator oxygen
Ventilation 201515
Hourly measurement of colour, and measured ventilatory parameters. If sudden drop in VT, check air entry Daily monitoring of intake, output and weight
Parent information Offer parents the following information, available from: http://www.bliss.org.uk/wp-content/uploads/2012/02/Ventilation_and_CLD.pdf
Antenatal 201315
Cleft lip and/or palate Obstetric team to refer to regional multidisciplinary cleft palate team, who will counsel parents, communicate plans for delivery and provide postnatal support for baby Hypoplastic left heart syndrome or other presumed duct-dependent lesions Obstetric team to refer to regional cardiac team, who will counsel parents and, where appropriate, confirm diagnosis and provide a plan of action Congenital diaphragmatic hernia Obstetric team to refer all cases to tertiary fetal medicine team (Birmingham or Liverpool) who will counsel, monitor and arrange delivery
Specific lesions See Urinary tract abnormalities on ultrasound scan, Gastroschisis, and Congenital heart disease: duct dependent lesions guidelines
Gestation <30 weeks 3032 weeks 03 days 37 days 610 days 1416 days
Additional scans If routine scans show a significant abnormality, discuss serial scanning with consultant Perform additional scans as clinically indicated or following a significant clinical event: necrotising enterocolitis major collapse repeated severe episodes of apnoea and bradycardia unexplained sharp fall in haemoglobin change in neurological status abnormal head growth pre- and post-operatively Follow-up If scan abnormal at 6 weeks, discuss the need for further imaging with consultant
Seizures In term babies with seizures, perform cranial ultrasound on admission and at 2 and 7 days while waiting for an MRI scan to be performed. MRI scan is the preferred imaging modality Neonatal encephalopathy Initial scan within 24 hr 2nd scan 34 days 3rd scan 714 days
In encephalopathic babies with significant birth trauma and low haematocrit, request noncontrast CT scan to exclude extra-axial bleed For babies with moderate-to-severe encephalopathy, MRI scan recommended between 714 days of life, if possible
PROCEDURE
Operator must achieve an acceptable level of competence before performing and reporting scans independently Record minimum set of coronal (6+ images): anterior to frontal horns of lateral ventricles at anterior horns of lateral ventricles and Sylvian fissures at 3rd ventricle and thalami at posterior horns of lateral ventricles (with choroids) posterior to choroids (posterior brain substance) if lateral ventricules are dilatated, measure the ventricular index at the level of 3rd ventricle at the foramina of Munro (ventricular index) and plot on appropriate chart Record minimum set of sagittal (5+ images): midline through 3rd ventricle, septum cavum pellucidum, cerebellum with 4th ventricle and foramen magnum through each lateral ventricle showing anterior and posterior horns, with caudothalamic notch imaged if possible through each hemisphere lateral to the ventricle for deep white matter Supplemental oblique, surface and axial images may be necessary to record pathology For detection of cerebellar lesions, scanning through posterior fontanelle (junction of lambdoid and sagittal sutures) and mastoid fontanelle (junction of posterior parietal, temporal and occipital bones) can be useful
SCAN REPORTING
Appropriately trained staff must interpret cranial ultrasound scans Scans must be reported using categories/terminology in Table below None Localised IVH without dilatation (germinal matrix haemorrhage, subependymal haemorrhage) IVH with ventricular dilatation Large IVH with parenchymal infarction Normal Enlarged (measure and plot ventricular index) None Periventricular flare Cystic lesions single large porencephalic cyst multiple cysts (cystic periventricular leukomalacia)
Intraventricular haemorrhage
Ventricular size
Parenchymal lesions
COMMUNICATION
Any member of neonatal team may communicate a normal result to parents but note that a normal scan does not equate to normal development and follow-up is essential Discuss an abnormal result with neonatal consultant before discussion with parents an abnormal scan does not equate to abnormal development, follow-up is essential Offer parents the Bliss information leaflet available for download from http://www.bliss.org.uk/wp-content/uploads/2012/02/Hydrocephalus1.pdf
DOCUMENTATION
Documentation is extremely important. Archive digital copies of scans for future review each image must contain patient identifiers Record following information on investigation chart: date scan requested date scan carried out Record ultrasound result (or file a written report) in babys notes (neonatal staff) Record a plan for performing future scans Record in notes any discussion with parents, especially of abnormal scans Include results of all scans in discharge summary, even if normal If eligible baby transferred to another hospital before scanning, communicate need for scan in transfer summary
PRINCIPLES OF MANAGEMENT
This is a medical emergency: involve consultant immediately Avoid gender assignment before expert evaluation Consultant to discuss with parents always use the term baby and avoid using he, she or, most importantly, it advise parents about delaying registration and informing wider family and friends until gender assignment complete Link with expert centre for appropriate evaluation Communicate openly with family Respect family concerns and culture DSD is not shameful potential for well-adjusted individual and a functioning member of society best course of action may not be clear initially parents need time to understand sexual development
First line investigations Blood pressure Karyotype (urgent) Imaging abdominal and pelvic ultrasound by an experienced paediatric sonographer Steroid chromatogram/profile, especially 17-OHP (delay until day 45 to allow maternal hormonal effects to decline) Testosterone and oestradiol LH, FSH U&E and glucose Cortisol Further investigations (locally and/or in conjunction with specialist advice) dHT (dihydrotestosterone) DHEA (dihydroepiandrosterone) Androstenedione Urine steroid analysis
ACTH LHRH hCG stimulation ACTH stimulation test AMH (anti-mullerian hormone) imaging studies Biopsy of gonad Molecular genetic studies (e.g. for CAIS)
TREATMENT
Check serum electrolytes and plasma glucose Involves a multidisciplinary team with an identified person (usually consultant neonatologist) acting as primary contact with family Specific treatment dependent on many factors and the diagnosis discuss with specialists
Cartlidge PH. Routine discharge examination of babies: is it necessary? Arch Dis Child 1992;67:1421-2 Glazener CM, Ramsay CR, Campbell MK, et al. Neonatal examination and screening trial (NEST): a randomised, controlled, switchback trial of alternative policies for low risk infants. BMJ 1999;318:627-32 Hall DM. The role of the routine neonatal examination: it has many aims, few of them evaluated. BMJ 1999;318:619-20 Hayes J, Dave S, Rogers C, et al. A national survey in England of the routine examination of the newborn baby. Midwifery 2003;19:277-84 Lee TW, Skelton RE, Skene C. Routine neonatal examination: effectiveness of trainee paediatrician compared with advanced neonatal nurse practitioner. Arch Dis Child Fetal Neonatal Ed 2001;85:F100-4 Moss GD, Cartlidge PH, Speidel BD, et al. Routine examination in the neonatal period. BMJ 1991;302:878-9 Patton C, Hey E. How effectively can clinical examination pick up congenital heart disease at birth? Arch Dis Child Fetal Neonatal Ed 2006;91:F263-7 Townsend J, Wolke D, Hayes J, et al. Routine examination of the newborn: the EMREN study. Evaluation of an extension of the midwife role including a randomised controlled trial of appropriately trained midwives and paediatric senior house officers. Health Technol Assess 2004;8:14 Wolke D, Dave S, Hayes J, et al. Routine examination of the newborn and maternal satisfaction: a randomised controlled trial. Arch Dis Child Fetal Neonatal Ed 2002;86:F155-60
Evidence Level: II Last amended February 2008 Last reviewed July 2013
EQUIPMENT
Maternal and baby notes Stethoscope Ophthalmoscope Measuring tape
AIMS
Identify congenital malformations Identify common neonatal problems and initiate management Continue with screening, begun antenatally, to identify need for specific interventions (e.g. immunisation)
PRE-PROCEDURE
Before undertaking clinical examination, familiarise yourself with maternal history and pregnancy records, including: maternal medical, obstetric and social history paternal medical history, if appropriate family health, history of congenital diseases identify drugs mother may have taken during pregnancy and in labour health of siblings identify pregnancy complications, blood tests, ultrasound scans, admissions to hospital identify maternal blood group, presence of antibodies, serology results for sexually transmitted diseases duration of labour, type of delivery, duration of rupture of membranes, condition of liquor Apgar scores and whether resuscitation required birth weight, gestational age, head circumference
Consent and preparation Introduce yourself to mother and gain oral consent. Ask about particular concerns Keep baby warm and examine in quiet environment
PROCEDURE
Skin examination Hydration Rashes: including erythema toxicum, milia, miliaria, staphylococcal skin infection, candida Pigmented lesions: naevi, Mongolian blue spots, birth marks, caf au lait spots Bruises: traumatic lesions, petechiae Cutis aplasia Tufts of hair not on head
Vascular lesions: haemangioma, port wine stain, simple naevus Colour: pink/cyanosis/jaundice/pallor/plethora Acrocyanosis Cutis marmorata
Facial examination General facial appearance to identify common syndromes Eyes Shape Slant Size Position Strabismus Nystagmus Red reflex Presence of colobomata Discharges Nose Nasal flaring Patency Ears Shape Position Tags or pits Mouth Size Cleft lip Symmetry of movement Swellings, Epsteins pearls, ranula, tongue tie (for parental reassurance) Teeth Cleft palate, hard/soft palate, (by both inspection and palpation) Sucking Skull Palpate: skull for sutures and shape/cranio-synostosis swellings on scalp, especially crossing suture lines, cephalhaematoma signs of trauma associated with birth (e.g. chignon from vacuum extraction) sutures for ridging or undue separation Neck Swellings Movement Webbing Traumatic lesions from forceps delivery Clavicles For fracture Arms and legs Position and symmetry of movement
Hands and feet Extra digits (polydactyly) Syndactyly, clinodactyly Palmer creases Skin tags Position and configuration of feet looking for fixed/positional talipes Overlapping toes Hips Developmental dysplasia using Ortolanis and Barlows manoeuvres, symmetry of gluteal skin folds (if abnormal or close family history of CDH: refer for hip scan) Spine Curvatures Dimples Sacrococcygeal pits Hairy patches/naevi Hairy tuft on spine Systems Examine (inspection, palpation, auscultation) each system Respiratory system Respiratory rate grunting nasal flaring Chest shape, asymmetry of rib cage, swellings nipple position, swelling/discharge/extra nipples Chest movement presence/absence of recession Auscultate for breath sounds Cardiovascular system Skin colour/cyanosis Palpate: precordium for thrills peripheral and femoral pulses for rate and volume central perfusion Auscultate for heart sounds, murmur(s), rate, rhythm pulse oximetry of right arm and left leg (<3% difference in SpO2 normal) Gastrointestinal tract Ask mother how well baby is feeding, whether baby has vomited and, if so, colour of vomit Bilious vomiting has a surgical cause and needs prompt stabilisation and referral Abdominal shape Presence of distension Cord stump for discharge or inflammation/umbilical hernia Presence and position of anus and patency Stools passed Palpate abdomen for tenderness, masses and palpable liver Auscultation is not routinely undertaken unless there are abdominal concerns
Genito-urinary system Ask mother if baby has passed urine, and how frequently Inspect appearance of genitalia: ambiguous?
Male genito-urinary system Penis size (>1 cm) Position of urethral meatus. Look for hypospadias Inguinal hernia Chordee Urinary stream Scrotum for colour Palpate scrotum for presence of two testes and presence of hydrocoele Female genito-urinary system Presence of vaginal discharge (reassure parents about pseudomenstruation) Skin tags Inguinal hernia Proximity of genitalia to anal sphincter Routine palpation of kidneys is not always necessary as antenatal scans will have assessed presence Neurological system Before beginning examination, observe babys posture Assess: muscle tone, grasp, responses to stimulation behaviour ability to suck limb movements cry head size in relation to body weight spine, presence of sacral pits, midline spinal skin lesions/tufts of hair If neurological concerns, initiate Moro and stepping reflexes Responses to passive movements: pull-to-sit ventral suspension Palpate anterior fontanelle size (<3 cm 3 cm) and tone
OUTCOME
Documentation Complete neonatal examination record in medical notes and sign and date it. Also complete child health record (Red book) Record any discussion or advice given to parents Normal examination If no concerns raised, reassure parents of apparent normality and advise to seek advice if concerns arise at home GP will re-examine baby when 6 weeks old Abnormal examination In first instance, seek advice from neonatal registrar/consultant Refer to postnatal ward guidelines for ongoing management Refer abnormalities to relevant senior doctor
HEARING SCREENING
INTRODUCTION
Early intervention improves the outcome for babies with a congenital hearing deficit Screening for congenital deafness is undertaken through the NHS Newborn Hearing Screening Programme (NHSP) by trained screeners according to national guidelines. They are automatically informed of all births and will ensure babies are screened Neonatal staff must understand how their local programme is organised, the risk factors for congenital deafness and know how to work with the screeners
INDICATIONS
Who All babies are eligible for screening, unless they have previously been diagnosed with bacterial meningitis or their ear canal is not patent on one or both sides neonatal staff must refer babies with meningitis to Audiology for an urgent assessment (inform screeners that referral has been made to ensure baby is not screened) screeners will refer babies with non-patent canal for urgent assessment
PROCEDURE
Consent Screening can only be performed with parental consent screeners will obtain consent from parents (if present) before screening if baby on Neonatal Unit (NNU) and parents absent, screeners will leave an explanatory leaflet and consent form for parents to complete file consent form in babys notes How Oto-Acoustic Emissions (OAE) +/- Automated Auditory Brainstem Response (AABR) according to national Well baby or NICU protocols neonatal staff must inform screeners if baby has ever spent >48 hr on NNU so that NICU protocol can be used babies on transitional care are screened using the Well baby protocol When Screen only when baby has reached 34 weeks (corrected age) Where Well babies In an area that undertakes newborn hearing screening in the community: in clinic/home visit In an area with a hospital-based service: Just before discharge from maternity unit if process not completed in hospital, screeners will arrange to see baby at home, in primary care or back in hospital NNU babies Arrange screening as close to discharge as possible, when baby is well enough to test and preferably once major medical treatment, ototoxic or other drug treatment complete Do not screen babies transferring to another NNU Complete screening of babies on NNU >48 hr by 44 weeks corrected age
FOLLOW-UP
Neonatal team must ensure all babies diagnosed with bacterial meningitis are referred for urgent audiology assessment and are not screened Screeners will arrange routine follow-up according to screening result and presence of other specific risk factors
Risk factors Neonatal staff must inform the screener of the following risk factors in order that the appropriate follow-up can be arranged: proven or possible congenital infection (CMV, Rubella, toxoplasmosis) cranio-facial anomalies, cleft palate, deformed pinnae (not simple ear tags) syndromes associated with hearing loss (Downs, Waardenburg, Alport, Usher etc) baby has been treated with ECMO Babies with the following risk factors are not followed up by Audiology, but data is collected for audit purposes: severe jaundice (at exchange level) multiple abnormalities with neurodegenerative disorder mechanical ventilation >5 days bacterial meningitis (not screened neonatal team arranges follow-up) Screener will determine presence of other risk factors before screening: family history of permanent hearing loss in childhood those with first-degree relative will be followed up in Audiology
FURTHER INFORMATION
Detailed information available from NHSP website: http://hearing.screening.nhs.uk Local details Alexandra Hospital, Redditch Russells Hall Hospital, Dudley Shrewsbury & Telford Hospitals Staffordshire General Hospital, Stafford New Cross Hospital, Wolverhampton Worcester Royal Hospital
Usually performed by trained health visitor in the community Babies on NNU usually screened before discharge
Birmingham City Hospital Birmingham Heartlands Hospital Birmingham Womens Hospital Good Hope Hospital, Sutton Coldfield Manor Hospital, Walsall University Hospital of North Staffordshire Solihull Hospital
Screening for all babies usually performed while still an in-patient, usually at bedside
Evidence Level: III Last amended July 2013 Last reviewed July 2013
Right-hand (pre-ductal) and either foot (post-ductal) saturations measured until consistent reading obtained
Comprehensive evaluation for causes of hypoxaemia (see next section and Flowchart 2). In the absence of non-cardiac findings to explain hypoxaemia, refer for diagnostic echocardiogram
Pulse oximetry screening 201315 Flowchart 2: Positive pulse-oximetry screen (abnormal test) Positive pulse-oximetry screen Pre or post-ductal SpO2 <90% SpO2 9094% or >2% pre and post-ductal difference on 3 measurements Symptomatic or abnormal examination findings
Respiratory/infective symptoms and/or Repeat pre and post-ductal SpO2 >94% with oxygen
Minimal respiratory symptoms and/or Abnormal CVS examination and/or Repeat pre or post-ductal SpO2 <95% with oxygen
Urgent in-house echo or Discuss with paediatric cardiologist Consider prostaglandin infusion
Repeat pre and post-ductal SpO2 >94% in air and Repeat CVS examination normal and No concerns
Repeat pre or post-ductal SpO2 <95% with oxygen or Diagnosis not established or Echo abnormal
Discharge
Retinopathy 201315
PROCEDURE
When to screen Indication Born <27 weeks gestation Born 2732 weeks gestation or 1500 g When to start screen 3031 weeks post-conceptual age 45 weeks postnatal age
If baby to be discharged before screening due, bring eye examination forward to be seen before discharge
How often to screen Determined by ophthalmologist but minimum recommendations are: weekly for vessels ending in zone I or posterior zone II; or any plus or pre-plus disease; or any stage 3 disease in any zone every 2 weeks in all other circumstances until criteria for discontinuing screening are met (see below) When to stop screening In babies without ROP, when vascularisation has extended into zone III, usually after 36 completed weeks postnatal age In babies with ROP, when the following are seen on at least 2 separate occasions: lack of increase in severity partial resolution progressing toward complete resolution change in colour of the ridge from salmon-pink to white transgression of vessels through demarcation line commencement of process of replacement of active ROP lesions by scar tissue How to screen Arrange screening with ophthalmologist Preparation for screening Prescribe eye drops for night before screening on drug chart Give cyclopentolate 0.5% and phenylephrine 2.5% 1 drop into each eye. Give 2 doses, 15 min apart, 30 min before examination (e.g. drops go in at 4.15 pm and 4.30 pm and baby seen at 5.00 pm). Timings may vary according to Trust practice check local guidance if in any doubt whether drop has gone into eye, give another drop immediately (pupil must be fully dilated) close eyelids after instillation of eye drops, wipe off any excess Care during procedure A competent doctor/ANNP available during eye examinations Use comfort care techniques (nesting, swaddling +/- pacifier) Consider oral sucrose 0.10.5 mL before examination (maximum 3 doses) If eyelid speculum or indentor to be used, topical anaesthesia (proxymetacaine 0.5% eye drops) administered before examination Avoid bright light and cover incubator/cot for 46 hr after examination
AFTERCARE
Complete ad hoc ROP form in BadgerNet documentation Eye examination results and recommendations for further screening must be included in transfer letter, together with ophthalmological status, future recommendations for screening intervals and out-patient follow-up arrangements Subsequent examinations must be documented by ophthalmologist in babys medical notes
Retinopathy 201315
VOLUME-TARGETED VENTILATION
(Volume guarantee/targeted tidal volume)
DEFINITION
In volume-targeted ventilation (VTV) primary gas delivery target is tidal volume (Vt) while the peak inspiratory pressure may vary depending on underlying lung compliance. Available as volume guarantee (VG) on Draeger babylog and targeted tidal volume (TTV) on SLE 5000 Benefits Compared with pressure-controlled ventilation, VTV can reduce: mortality bronchopulmonary dysplasia pneumothorax hypocarbia severe cranial ultrasound abnormalities
INDICATION
Primarily used in preterm babies with surfactant-deficient lung disease requiring ventilation May be useful in other situations requiring ventilation
MODE
VG/TTV combined with assist control or pressure-support ventilation (PSV) preferred these modes support all spontaneous breaths PSV has the additional advantage of synchronising expiration
PEAK PRESSURES
Start PIP limit (Pmax) of ~2530 cm H2O Adjust Pmax to 56 cm H2O above average PIP needed to deliver set tidal volume If PIP progressively increases or is persistently high or if set Vt not delivered, re-assess baby PEEP set at 46 cm water
VENTILATOR RATE
In baby with poor respiratory drive, use rates of 5060 bpm Lower back-up rates of 3040 bpm can be used with good respiratory drive Use Ti (inspiratory time) of 0.30.4 sec
WEANING
In assist-control or PSV, wean by reducing Vt in steps of 0.5 mL/kg Pressure weans automatically as lung compliance improves Avoid tidal volumes <3.5 mL/kg In SIMV, rate reduced as well as Vt Attempt extubation when: MAP falls consistently <8 cm baby has good respiratory drive and satisfactory gases