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PEDIATRIC AIRWAY SYNDROMES

ANESTHETIC CONSIDERATIONS

  • 1. Considerations for the Pediatric (cooperation, physiology, pharmacokinetics)

  • 2. Potential Difficult Intubation/Ventilation

    • a. Surgical airway vs intubation attempt

    • b. Awake vs asleep

    • c. Spontaneous ventilation vs paralyzed

  • 3. Potential Aspiration Risk & Co-existing Obstructive Sleep Apnea

  • 4. Potential for Unstable or Immobile Cervical Spine

  • 5. Coexisting multiple congenital anomalies particularly CHD

  • ANESTHETIC GOALS

    • 1. Establish

      • a. Aspiration Risk

      • b. Cervical Spine Stability

      • c. Presence of OSA & Associated Anomalies

  • 2. Minimize anesthetic related post operative respiratory depression

  • KEY ANESTHETIC ISSUES

    • 1. Simple maneuvers may relieve airway obstruction prone positioning, pulling tongue forward (suture in tongue, oro/nasopharyngeal airway, LMA)

    • 2. Pre-operative assessment of associated organ system involvement (CHD, renal)

    • 3. Don’t forget option of intraop tracheostomy if postop airway management is going to be very difficult

    • 4. Awareness of the dynamic status of the airway as patient grows or the disease progresses

    • 5. Specific Syndromes

      • a. Cleft lip and palate

    i.

    associated with Pierre Robin, CHD

    ii.

    L sided cleft may be a difficult laryngoscopy

    • b. Pierre-Robin syndrome

    i.

    Micrognathia, cleft palate, glossoptosis

    ii.

    CHD

    • c. Beckwith-Wiedemann syndrome

    i.

    Macrosomia, macroglossia

    ii.

    Hypoglycemia

    iii.

    Associated with omphalocele

    iv.

    Likely easy laryngoscopy but difficult BMV

    HISTORY

    Previous anesthetics and experience with AW management (but kids grow!)

    • Noisy breathing/ stridor

    Airway

    Details of congenital syndrome

    Evidence of acute on chronic AW obstruction (infection, foreign body)

    • Snoring

    • Symptoms of OSA Complete perinatal Hx

    Complications of Pregnancy History of acute/ chronic airway obstruction

    Aspiration symptoms/ reflux

    Symptoms of cervical spine involvement

    • Occipital/ neck/ upper extremity pain or paresthesias

    • Limited range of motion Coexisting syndrome abnormalities e.g. cardiac (cyanosis, murmur)

    PHYSICAL

    Signs of distress including agitation, retractions, cyanosis, anxiety, weak or absent cry, or

    Oral cavity (macroglossia, microstomia [small mouth], distortion [hemangioma,

    stridor Anatomical AW abnormalities including:

    glottic webs])

    Anterior mandibular space (micrognathia, anterior larynx)

    Maxilla (hypoplasia, nasal obstruction/mouth breathing)

    TMJ (restriction to opening [fixed vs. secondary], reduced translocation [inability to jut jaw forward])

    Vertebral column (restricted movement, instability) Resp: pattern and rate, wheeze (aspiration?), stridor, positional dyspnea

    CVS: pulses, cap refill, murmur, pulm HTN (OSA)

    Signs of associated congenital pathology or previous surgery

    INVESTIGATIONS

    C-spine XR, PA & lateral (mobility, instability)

    Echo (if congenital heart disease)

    OPTIMIZATION

    EMLA

    ENT consult/ backup

    Caution with Premedication with co-existing OSA

    Aspiration prophylaxis

    Antisialagogue

    SBE Prophylaxis if CHD

    ANESTHETIC OPTIONS

    Maintenance of spontaneous ventilation is paramount in those patients with anticipated

    difficult ventilation/ intubation avoid muscle relaxants Awake instrumentation of the AW is virtually impossible in the pediatric patient

    Recognition of conflicts of difficult A/W plus: pediatric patient, full stomach, raised ICP, CHD with shunt & pulmonary hypertension, malignant hyperthermia

    ANESTHETIC SETUP

    Experienced help

    Difficult Airway Cart

    LMAs oral and nasal A/W’s available to facilitate PPV

    Selection of preferred blades (curved may be better with macroglossia)

    Multiple ETT sizes

    Surgeon/ Tracheostomy/ Percutaneous cricothyroidotomy equipment

    ANESTHESIA MANAGEMENT

    Inhalational Sevo but recognized relatively quick offset

    TIVA Propofol/remi infusion +/- bolus

    Consider preop lidocaine nebulization to facilitate FOB intubation under sedation, or to facilitate placement of oral airway under light plane of anesthesia

    BACKGROUND

    Pediatric vs Adult Airway - 5 Major Anatomical Differences Large Tongue

    • In proportion to rest of oral cavity, and so more easily obstructs airway Position of Larynx:

    • Infant: (C3-4); adult (C4-5); premature baby C3

    Epiglottis narrower/stiffer and angled away from the axis of trachea Vocal Cords have lower attachment anteriorly

    • Whereas in adults they rest perpendicular to trachea; this difference can sometimes lead to difficulty with intubation of infant Subglottic area (cricoid cartilage) is narrowest part of airway

    • Whereas in an adult is the rima glottides

    SPECIFIC SYNDROMES

    Cleft lip & palate:

    Most common congenital A/W abnormality (lip 1/1000 live births, palate 1/2500) Cardiac abnormalities in 20% Possibly chronic aspiration Associated with many other craniofacial disorders (over 150), commonly Pierre Robin syndrome (also Mohr, Shpritzen, 4P) Technical specifics:

     Consider preop lidocaine nebulization to facilitate FOB intubation under sedation, or to facilitate placement of
    • Isolated cleft lip usually doesn’t present AW difficulties

    • Obstruction by tongue is usually overcome with oral AW

    • Left-sided cleft palate may interfere with normal positioning of laryngoscope blade

    Pierre Robin syndrome:

    Micrognathia, glossoptosis (abnormal downward or back placement of the tongue), respiratory obstruction +/- cleft palate and CHDPulm HTN, cor pulmonale, or pulm edema secondary to chronic A/W

    obstructionTechnical specifics:

    • May obstruct A/W when supine

     Consider preop lidocaine nebulization to facilitate FOB intubation under sedation, or to facilitate placement of
    • Oral, nasal A/W and prone positioning may facilitate ventilation

    • Fiberoptic laryngoscopy often required

    • Older patients may be easier to intubate as mandible grows

    Craniofacial dysostosis:

    Craniosynostosis (premature closure of cranial sutures) Midface hypoplasia (normal-sized mandible with prognathia) +/- hydrocephalus, hypertelorism, proptosis, cervical fusion, tracheal rings

    Associated with Apert, Crouzon (towering skull, beaked nose) and

    Pfeiffer’s syndromes

     Consider preop lidocaine nebulization to facilitate FOB intubation under sedation, or to facilitate placement of

    May have obstructive apnea severe enough to require tracheostomy May have inc ICP May require massive transfusion if multiple sutures (large bore IV, blood products)

    Technical specifics:

    • Mask ventilation may be difficult, as well as obstructed PPV due to high, arched palate resulting in nasal obstruction & choanal stenosis

    • Intubation is not usually a problem unless there are associated C-spine abnormalities which limit mobility, or the presence of tracheal rings (may require smaller ETTs)

    Treacher Collins syndrome (mandibulofacial dysostosis):

    Maxillary, zygomatic and mandibular hypoplasia with high, arched palate and small mouth May be associated with cleft palate and velopharyngeal incompetence (leading to nasal speech) as well as CHD OSA (cor pulmonale) post op HDU, consider trach Associated dysmorphisms include laterally sloping palpebral fissures, notched lower eyelids, coloboma (split/gap in iris, eyelid, lens etc.) of eye and hearing loss Older children may develop basilar cranial kyphosis Technical specifics:

     Technical specifics:  Mask ventilation may be difficult, as well as obstructed PPV due to
    • Often very difficult ventilation and intubation requiring fiberoptic intubation or tracheostomy

    Hemifacial microsomia (includes Goldenhar syndrome):

    Uni or bilateral mandibular hypoplasia and soft-tissue loss May have auricular abnormalities and facial nerve weakness Bilateral may be mistaken for Pierre Robin Associated with Goldenhar syndrome (which includes normal IQ, CHD (20%), macrostomia, bony spinal deformity, possible hydrocephalus) Technical specifics:

     Technical specifics:  Mask ventilation may be difficult, as well as obstructed PPV due to
    • Severity of deformity reflects magnitude of A/W challenges

    • Instrumentation of A/W may be more difficult after surgery to jaw or TMJ due to soft tissue contractures

    • Goldenhar may have limited neck mobility making BMV and direct laryngoscopy very difficult

    KlippelFeil syndrome:

    Cervical spine abnormalities including fusion, A-O instability, spinal canal stenosis, and scoliosis Associated with neurological, cardiovascular, genitourinary and auditory abnormalities Technical specifics:

    • BMV usually not difficult

    • Fiberoptic intubation may be necessary due to limited mobility

    • Careful positioning due to possible c-spine instability

     Technical specifics:  Mask ventilation may be difficult, as well as obstructed PPV due to

    Beckwith-Wiedemann syndrome:

    Gigantism highlighted by prominent macroglossia

    Characterized by exophthalmos, hyperplasia of kidneys and pancreas (leading to hyperinsulinemic hypoglycemia)Severe obstruction may ultimately lead to cor pulmonale

     Technical specifics:  Mask ventilation may be difficult, as well as obstructed PPV due to

    Technical specifics:

    • BMV may be impossible

    • Direct laryngoscopy is usually possible with adequate displacement of tongue (may require assistant)

    • Often present for glossectomy requiring nasal intubation

    Down syndrome (trisomy 21):

    See Down syndrome discussion elsewhereTechnical specifics:

    • Atlantoaxial instability requires careful positioning

    • Large tongue and narrow nasopharynx may lead to post-operative obstruction, while small larynx and cricoid predisposes to acquired subglottic stenosis

     Technical specifics:   BMV may be impossible  Direct laryngoscopy is usually possible with

    Freeman-Sheldon (Whistling Face) syndrome (craniocarpotarsal dysplasia):

    Myopathic dysplasia leading to facial contractures with circumoral fibrosis and microstomiaAssociated with camptodactyly (flexion deformity of the finger) and talipes equinovarus (clubfoot)Technical specifics:

    • MH RISK.

    • Limited mobility may make direct laryngoscopy difficult

     Technical specifics:   BMV may be impossible  Direct laryngoscopy is usually possible with

    Fibrodysplasia ossificans progressiva:

    Heterotopic ossification of head, neck, spine and shoulder girdleTechnical specifics

    • Limited mobility may make direct laryngoscopy difficult.

    Hurler syndrome (Mucopolysaccharidosis Type 1H)

     Technical specifics:   BMV may be impossible  Direct laryngoscopy is usually possible with

    Inborn error of metabolism leading to infiltration and thickening of various AW structures including the tongue, vocal cords and nasal passages.Associated with cardiomyopathy, mental retardation, gargoyle facies, dwarfism, pectus excavatum, kyphoscoliosis, and hepatosplenomegalyTechnical specifics:

     Technical specifics:   BMV may be impossible  Direct laryngoscopy is usually possible with
    • Thick secretions may obscure visualization

    • Difficult BMV and intubation requiring possible rigid bronchoscopy or tracheostomy.

    • Patients may get more difficult to intubate with age.

    Vascular malformations:

    Hemangiomas, AVM and cystic hygromas (venous lymphatic

    malformations) can all complicate AW management. AVMs may be associated with high-output cardiac failure and consumptive thrombocytopenia (Kasabach-Merritt syndrome)Technical specifics:

    • Anatomic distortion by vascular masses may make BMV and direct visualization difficult

     Technical specifics:   BMV may be impossible  Direct laryngoscopy is usually possible with
    • Trauma to lesion may lead to bleeding complications

    • May require prolonged AW protection, including possible tracheostomy, during extended treatment (chemotherapy, sclerotherapy, radiation etc.)

    Juvenile onset rheumatoid arthritis:

    Multi-system disease which involves destruction of joints

    including TMJ, c-spine and crico-arytenoidsTechnical specifics:

    • Mobility problems may complicate proper positioning.

    • Laryngeal involvement may restrict passage of ETT

     May require prolonged AW protection, including possible tracheostomy, during extended treatment (chemotherapy, sclerotherapy, radiation etc.)
     May require prolonged AW protection, including possible tracheostomy, during extended treatment (chemotherapy, sclerotherapy, radiation etc.)

    REFERENCES

    • 1. Nargozian, The Airway in Patients with Craniofacial Abnormalities, 2004 Pediatric Anesthesia

    • 2. Infosino, Pediatric Upper Airway and Congenital Anomalies, 2002 Anes Clin of NA

    • 3. Hall, The Difficult Pediatric Airway Recognition, Evaluation and Management, 2001 CJA

    • 4. Faust’s Anesthesiology Review, Ch 163.

    Syndrome

    Airway Defect

    Multisystem Associations

    Anesthetic Significance

    Cleft Lip±Palate

    Isolated or associated

    swallowing, speech,

    BMV-difficult because airway obstruction

    (CL ± CP)

    with syndrome

    URTI & middle ear infections

    by tongue falling into cleft: reversible with oral airway

    pulmonary aspiration- feed upright with cleft nipple

    DL usually OK in isolated CP - but laryngoscope blade may “hang-up” on cleft

    20% associateded with CHD

    Cleft Palate surgery: oral RAE secured midline with gag (beware ETT

    High risk of airway obstruction postop

    Associated with Pierre Robin, Treacher Collins, Klippel Fiel, Down Syndrome

    displacement)

    (edema, blood, residual anesth drugs); tongue stitch often placed to help relieve obstruction

    Leave intubated if in doubt

    Alice says Oxford blade specifically blade made for clefty’s

    Mucopolysaccharidoses- genetic storage disease leading to accumulation of mucopolysaccharides throughout the body; 8 clinical types have been described of varying degrees of severity

    Hurler

    Macroglossia; narrowing

    widespread deposition of

    death before age 10

    potential difficult BMV & DL

    (TypeI) more

    laryngeal inlet &

    mucopolysaccharides

    Postop subglottic edema

    severe form

    Tracheobronchial tree →

    CAD, valvular disease,

    Potential for lower airway obstruction

    progressive AW

    CMO & arrhythmia,

    because tracheobronchial tree

    obstruction;

    sudden death

    abnormalities

    Joint stiffness with ↓ neck mobility

    OSA common Restrictive Lung Disease

    Periop resp failure Unstable C-spine

    Odontoid hypoplasia and Atlantoaxial subluxation

    & recurrent pulm infections

    Severe multisystem disease esp cardiac

    Worsen with age

     

    Bone Marrow Transplant

    M. Barker wants to emphasize awareness that Hurler’s gets worse with age

    has been effective treatments; may actually reverse the airway abnormalities

    Beckwith-

    Macroglossia-regresses

    genetic “overgrowth

    Chronic AWO and usual sequelae of pulm

    Wiedemann

    with age, may require

    syndrome”

    HTN & cor pulmonale

    (~common)

    partial glossectomy

    characteristized by exomphalos, macroglossia, and gigantism

    BMV Difficult (oral AW or LMA) DL usu OK May need to stay intubated post glossectomy b/c of sig swelling

    associateded with viscero- megaly (↑ liver, pancreas,

    Avoid hypoglycemia

    kidney,spleen), abdominal wall defects (omphalocele) & neonatal hypoglycemia CHD alice adds

    Mandibular hypoplasia

    Micrognathia Cleft Palate

     

    Sequelae of chronic AWO -

    May have severe airway obstruction especially when supine

     

    Pierre Robin

    Glossoptosis (tongue posteriorly

    pulm HTN, cor pulmonale

    most severe as infant, decreases as mandible grows

    displaced)

    Pulm aspiration

    treat AWO with oral/nasal airways & prone position but if very severe may require glossolabiopexy (tongue sutured to lower lip to keep anterior)

     

    BMV Difficult b/c AWO inhalational induction in lateral position or use LMA

     

    DL Difficult; may get easier as mandible

     

    grows

    Mandibulofacial dysostosis

    Malar & mandibular hypoplasia ±TMJ

     

    Sequelae of chronic AWO -

    Pulm aspiration

    BMV Difficult b/c severe AWO DL V. Difficult sedated FOI or LMA ± FOI

    Treacher Collins

    small mouth, CP & velopharyngeal (VP) incompetence

    pulm HTN & cor pulmonale

    AW Sx (eg palatoplasty) ↑ risk postop AW obstrⁿ Trach often indicated for intra- & post-op

    AW may worsen with

    ±CHD

    AW mgmt & resp care

    age

    with ↑basilar

    nETT contraindicated post repair VP

    kyphosis of cranial base

    insufficiency disrupt flap

    Hemifacial microsomia

    varying degrees of

    macrostomia

    Normal IQ

    eye/ear abN’s on

    ±CHD

    BMV Difficult - poor mask fit; AWO

     

    Goldenhar’s (oculo- auriculo-vertebral

    mandibular hypoplasia

    Hydrocephalus

    DL highly variable (?degree of mandibular deformity, mouth opening-previous Sx’s on

    syndrome)

    fused/hemivertebrae → limited neck mobility

    affected side

    jaw/TMJ, neck mobility) C-spine immobility

    may present bilaterally (confuse with Pierre-

     

    Potential ↑ ICP

     

    robin)

     

    Craniofacial dysostosis

    Crouzon Syndrome

    Midface hypoplasia, narrow, hi-arch palate ±

    Craniosynostosis ±hydrocephalus

    ± hydronephrosis,

    Chronic AWO±OSA; may need long term Trach

     

    Apert Syndrome

    (no assoc multisyst

    CP, choanal stenosis Mandible usu Normal ±↓C-spine mobility

    ± CHD

    PCKD

    BMV Difficult- poor mask fit; ~ obstructed nares so need patent oral AW during inductⁿ;

    use LMA

     

    effects)

    abN tracheal rings → smaller ETTs

     

    ± esophageal atresia

    DL usu OK unless C-sp prob’s, prepare smaller ETTs, nasal ETT not contraindicated

     

    (craniosynostosis & ↑ ICP only for

    Prone to post intubation croup Assoc’ed cardiac, renal, ↑ ICP

     

    Freeman-Sheldon

    Microstomia

     

    Crouzon) Rare genetic

    myopathic

     

    BMV OK

     

    /Whistling Face/

    short webbed neck with

    dysplasia
    dysplasia

    of facial, limb ±

    Difficult DL

    cranio-carpo-tarsal

    ltd mobility

    respiratory muscles

     

    Assoc with malignant

    dysplasia

    pot’l resp difficulties

    hyperthermia no inhalational

     
     

    induction. Trigger free anesthetic

     

    Klippel-Feil

    multiple skeletal abN’s

    spinal stenosis, scoliosis

    BMV usu OK

    syndrome

    incl fused C-spine, abN±unstable

    ±CVS (usu VSD) GU (may have renal

    Difficult DL b/c of neck rigidity & instability

    craniocervical junction

    insufficiency)

    FOI ± LMA has been successful

    Associated with cleft palate

    Trisomy 21 (most common chromosomal

    Small nasopharynx Lg tongue; CL±CP

    OSA CHD (endocardial cushion

    BMV & DL may be challenging; smaller ETTs available

    abN)

    Subglottic stenosis

    defects, TOF, PDA)

    C-spine instability

    atlantoaxial subluxation

    Tendency to obesity

    Consider postopICU esp post AW Sx

    Cleft Lip & Palate

    Cleft Lip & Palate

    Occurs in 1/750 births May be isolated or as part of broader syndrome (Pierre Robin/ Velocardiofacial) with 20% associated with CHD Airway management is usually straightforward though potential for tongue or laryngoscope blade to fall into cleft and impair ventilation or view of larynx

    Craniofacial Dysostosis (Apert/ Crouzon/ Pfeiffer)

    Craniofacial Dysostosis (Apert/ Crouzon/ Pfeiffer)

    These patients have craniosynostosis with a degree of midface hypoplasia & a high arching palate with choanal stenosis and preferential mouth breathing Typically the mandible is of normal size Often bag mask ventilation is difficult with improper fit due to midface hypoplasia Usually intubation is easy, however Apert’s may have CHD, renal anomalies and increased ICP

    Mandibulofacial Dysostosis (Treacher Collins)

     

    These children have maxillary, zygomatic and mandibular hypoplasia Other features include laterally sloping palpebral fissures, coloboma, small mouth opening, and auricular atresia +/- cleft palate Difficult BMV and intubation Tracheostomy is common in this group Cardiac Defects occasionally present

    These children have maxillary, zygomatic and mandibular hypoplasia Other features include laterally sloping palpebral fissures, coloboma,

    Hemifacial Microsomia (Goldenhar’s)

    Hemifacial Microsomia (Goldenhar’s)

    Mandibular hypoplasia, Ear abnormalities, Macrostomia and Vertebral Anomalies This may be confused with Pierre Robin Cardiac Defects possible

    Klippel Feil Syndrome

    Klippel Feil Syndrome

    Cervical and occipital fusion accompanied by spinal stenosis and scoliosis Cardiac and genitourinary anomalies may also occur Although BMV is typically easy, direct laryngoscopy is hampered by immobile cervical spine which may also demonstrate instability

    Beckwith Weidemann Associated with omphalocele: macgroglossia, giagantism, hypoglycemia, polycythemia Chronic airway obstruction may be severe, leading
    Beckwith Weidemann
    Associated with omphalocele: macgroglossia, giagantism,
    hypoglycemia, polycythemia
    Chronic airway obstruction may be severe, leading to cor
    pulmonale
    Suggested to have an assistant hold tongue while
    performing DL
    Down’s Syndrome
    Reviewed elsewhere (macroglossia, high arched palate,
    subglottic stenosis, A/O instability)
    Whistling Face Syndrome (Freeman Sheldon)
    Facial contractures, microstomia and microcephaly:
    Reported to have MH RISK
    Potential for increased ICP
    Mucopolysaccharidosis
    Infiltration of mucopolysaccharides results in
    macroglossia associated with cardiomyopathy &
    accelerated CAD, aortic insufficiency
    Tonsillar hypertrophy
    Odontoid hypoplasia and Atlantoaxial Subluxation
    Achondroplasia
    Potential for Hydrocephalus
    Odontoid hypoplasia with atlantoaxial instability
    CHARGE/VA(C)TER(L)
    Coloboma; Heart disease (TOF, PDA
    ..
    )
    choanal Atresia,
    Retarded growth, Genital anomalies (hypogonadism) and
    Ear anomalies
    Vertebral anomalies; Anal atresia, (Cardiac), TEF, Renal
    anomalies, Limb anomalies

    DiGeorge/Velocardiofacial

    DiGeorge/Velocardiofacial

    Hypoplasia of thymus, parathyroid, micrognathia, cleft

    palate, laryngeal web, cardiac defects

    Turner’s Sydrome (Noonan’s)

     

    45 X genotype: Short Neck; Micrognanthia; Webbed

    45 X genotype: Short Neck; Micrognanthia; Webbed

    neck; Kyphoscoliosis; Aortic Stenosis; Ao Coarct; Accelerated CAD; Hypothyroidism; Diabetes; Liver disease/coagulopathy (factor XI deficiency)

    (Noonan’s resembles Turners, though has normal karyotype

    and pulmonic stenosis and risk of HOCM)