Anorectal Malformations in Neonates and Infants CA6039 v1.1

Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
A Clinical Guideline recommended for use
Neonatal Intensive Care Unit (NNUH)

In:
By: Neonatal & Paediatric medical, surgical & nursing staff
For: Neonates and infants admitted to NICU
Key words: Anorectal malformation, Imperforate anus, colostomy
Written by: Mr Richard England, Consultant Paediatric Surgeon (NNUH)
All Neonatologists, Paediatric / Neonatal Surgeons & Paed

Gastroenterologists
(Dr R Roy, Dr P Clarke, Dr M Dyke, Dr D Booth, Dr P
Supported by:
Muthukumar, Dr F Walston, Dr H O’Reilly, Mr T Tsang, Mr M S
Kulkarni, Mr A B Mathur, Mr A Minocha, Dr M A Morris & Dr G
Briars) (NNUH)
Assessed and Clinical Guidelines Assessment Panel

approved by the: 19th November 2014
Clinical Standards Group (NNUH)

Reported as approved:
Effectiveness Sub-Board (NNUH)
Document issued: November 2014
To be reviewed before: November 2017
To be reviewed by: Mr Richard England
Document supersedes: None
Document Reg. No: CA6039
This guideline has been approved by the Trust's Clinical Guidelines Assessment Panel as an aid to the
diagnosis and management of relevant patients and clinical circumstances. Not every patient or situation fits
neatly into a standard guideline scenario and the guideline must be interpreted and applied in practice in the
light of prevailing clinical circumstances, the diagnostic and treatment options available and the professional
judgement, knowledge and expertise of relevant clinicians. It is advised that the rationale for any departure from
relevant guidance should be documented in the patient's case notes.
The Trust's guidelines are made publicly available as part of the collective endeavour to continuously improve
the quality of healthcare through sharing medical experience and knowledge. The Trust accepts no
responsibility for any misunderstanding or misapplication of this document.
Author/s: Mr Richard England Date of issue: November 2014

Valid until: November 2017 Guideline Ref No: CA6039 v1.1
Document: ARM Guideline 2014
Copy of complete document available from: Trust Intranet Page 1 of 19
Quick reference guide
Antenatal Examination Delayed

Diagnosis at Birth Diagnosis
Resuscitation
Diagnosis confirmed
Initial management
Nil By Mouth, IVI, NGT
Assessment by Paediatric
Surgeon
Primary Colostomy
Anoplasty VACTERL
Screening
see page 8
Stoma
Nurse
Dilatations
(Arranged by
Paediatric
surgeon)
Further
Surgery
Planned

Objective/s
Ensure best practice in the diagnosis, investigation and management of a baby born
with an anorectal malformation (ARM) in the newborn period.
Rationale
This guideline has been written to ensure that all babies born with an anorectal
malformation are diagnosed in a timely manner and managed appropriately to avoid
complications. This guideline will also help medical and nursing staff explain the
management clearly to parents. Most of the recommendations are derived from
standard national practice in paediatric surgery, published literature and findings of a
recent regional audit which involved this unit demonstrating areas where improvements
could be made.
Definitions of Terms Used
Anorectal malformation: Term used to encompass the wide range of defects involving
the development of the rectum and anus.
Colostomy: Opening of the bowel (usually sigmoid colon) onto the skin to divert faeces
away from the perineum.
Definitive Surgery: This term is used to explain that an operation to reconstruct a new
anal opening is required in most cases. This is often called a PSARP (“P-Sarp” or
“Sarp”) operation which stands for Posterior Sagittal Anorectoplasty. Variations of this
operation are beyond the remit of this document but this can be used as a general term
for ward staff.
Fistula: Connection between the rectum and another structure which is not a normal
anal canal.
Imperforate anus: Term used to describe the situation where there is no anal opening in
the correct position.
Muscle complex: Term used to describe the sphincter muscle which is separated from
the fistula or anal canal in babies with an ARM. The definitive surgery will reposition the
muscle complex around the new anus.
Perineum: Area of the skin between the legs which should normally include the urinary,
genital, and anal openings.
Introduction
Malformation of the anal opening often leads to bowel obstruction in the newborn and
requires complex surgery to correct. The process of managing this condition starts with
recognition and correct diagnosis. Delayed recognition can lead to complications and
distress for the baby and parents.
There are a large number of different anorectal malformations that can also affect the
urinary and genital tracts. A number of specialised investigations may be needed at
different stages of the management before definitive surgery can be undertaken.
If the type of malformation will not allow safe or adequate passage of faeces the baby
will need a colostomy. The neonatal and surgical team will be able to prepare the baby
and family for this and train the parents in the management of the colostomy while
waiting for the definitive surgery.
It is important to ensure the baby is otherwise developing well, putting on weight and
feeding well. Routine neonatal checks and tests should continue as normal despite the
presence of a complex malformation.
Diagnosis
Antenatal Management
Congenital causes of bowel obstruction may occasionally be noticed on antenatal scans

as distended bowel loops. A specific diagnosis is not possible from antenatal scans but
review by an obstetrician with an interest in fetomaternal medicine should be obtained.
Careful assessment of the heart and kidneys on antenatal ultrasound should also be
made. Discussion with a paediatric surgeon with a view to counselling would be
appropriate.
An antenatal alert should be circulated to the neonatal team and paediatric surgeons.
Mode of delivery should be determined by obstetric principles.
A plan for a paediatrician to attend the delivery should be in place.
Postnatal Baby Check
If there has been an antenatal concern about dilated bowel, then a paediatrician should
attend the delivery. Neonatal life support principles take priority.
Attention should be made to the potential presence of cardiac anomalies.
An examination of the abdomen and perineum should be carefully performed.
If there is a concern about bowel obstruction whether anorectal or at a higher level, then
the baby should not be fed. Intravenous access and IV fluids should be started. A size
8-10Fr (term baby) nasogastric tube should be passed, position confirmed and left on
free drainage. If an 8 Fr tube cannot be passed the surgical team should be notified.

Even if there were no antenatal concerns regarding bowel obstruction on antenatal

scans the abdomen and anus should be inspected by a paediatrician or experienced
midwife before discharge.
The routine baby check should include careful examination of the anus and any
meconium present in the area should be wiped away.
Features such as the position of the anus, puckering of the anal rim by circumferential
muscle, size of the anal opening by inspection and any reflex contractions should be
noted.
If the anus appears absent then a fistula further forward (anterior) towards the genital
region should be looked for.
Any meconium on the perineum should be noted and recorded even if the orifice of
origin is not known.
In boys the presence of a normal penis and descent of the testes should be assessed.
In girls, examination for a urethral opening and vagina should be performed by careful
but firm parting of the labia.
The examination findings should be carefully documented.
Delayed presentation
There are continued reports in the literature of delayed presentation of anorectal

malformations where imperforate anus has not been noticed before discharge and the
diagnosis is delayed until abdominal distension, poor feeding or vomiting alert the
mother or healthcare staff. These are late signs in anorectal malformations and by this
time (2-3 days of age) the bowel will be very distended and the baby will be dehydrated.
This scenario is avoidable.
The baby should be re-admitted to hospital as an emergency and seen quickly by a

paediatrician and paediatric surgeon. The mother should be instructed not to feed the
baby. Intravenous resuscitation and IV antibiotics should be given. A nasogastric tube
should be inserted, position checked and left on free drainage. Consideration for
admission to the neonatal unit should be discussed if surgery is likely.
Initial management
All babies should have a complete neonatal examination and additional anomalies
managed as appropriate.
Once the diagnosis of an anorectal malformation is made then feeds should be stopped.

Intravenous access should be obtained and blood tests for FBC, CRP, blood culture and
blood glucose taken.
In addition electrolytes, urea and creatinine may be useful if taken at more than 24hrs of
age.
A Group and Save sample should also be taken.
Initially IV fluids (appropriate fluid type and rate for age) at maintenance rates are
appropriate unless there are signs of dehydration requiring additional resuscitation
fluids.
A nasogastric tube should be placed and gastric position checked. It should be left on
free drainage and aspirated regularly. The interval between aspirations should be no
greater than 4 hrly. Significant aspirates should be replaced with IV fluids.
A decision when a long line and TPN is required can be taken jointly between the
neonatal, paediatrics gastroenterology and surgical teams, as time to achieving
adequate feeds varies according to the specific anomaly and management required.
IV antibiotics including anaerobic cover should be given. The duration of cover is

dictated by culture results and surgical management. Please refer to guidelines for
Management of Newborn babies at increased risk of developing neonatal infection IO16
All babies should have received IV or IM Vitamin K. Oral vitamin K is unreliably

absorbed in bowel obstruction.
It is useful to monitor urine output, taking note of the site of origin of the urinary stream
and whether the urine contains meconium in male infants.
All babies with an anorectal malformation should have a chest and abdominal x-ray as
soon as they are stabilised.
Mothers should be encouraged to continue expressing breast milk for storage, even
though their baby is not being fed.
Surgical management in the newborn period
The surgical management is determined by an accurate diagnosis of the type of

anorectal malformation. In most cases this can be determined by inspection of the
perineum by a paediatric surgeon.
The simplest classification of anomalies is shown in table 1. However it should be noted

that there are many variations and rare variants. The exact surgical management of
each type of ARM is beyond the scope of this document.


Table 1: Classification of Anorectal Malformations
Male Female
Perineal fistula Perineal fistula
Rectourethral (bulbar) fistula Rectovestibular fistula
Rectourethral (prostatic) fistula
Rectovesical (bladder neck) fistula Cloaca
No fistula No fistula
Rectal Atresia / Stenosis Rectal Atresia / Stenosis
Rare variant Rare variant
Appendix 1: Diagrams depicting the most common ARM types.
In general a perineal fistula might be amenable to an anoplasty which is a simpler

version of the definitive repair. This could potentially be performed without a colostomy
in the newborn period. The decision whether this is possible is made by the paediatric
surgeon and depends on the condition of the baby in the first 24 hours of life.
Most of the other anomalies require a colostomy. This allows diversion of the faeces
away from the fistula and ensures the definitive repair is performed in a clean area. It
also allows further investigations to be performed at a later date.
The timing of the colostomy is determined by the paediatric surgeon as it depends on

the degree of bowel obstruction.
Post-operative period
The paediatric surgical team will liaise with the neonatal team regarding the outline
details of the operative procedure and the post-operative plan. This will also be
documented on the operation note and should be read by both medical and nursing staff
on the neonatal unit.
The baby should have IV fluids for maintenance hydration unless they have already
been started on TPN.
Feeds can be started according to the surgeons instructions.
If a colostomy has been formed this should be kept moist and loosely covered until it
starts to work.
A stoma nurse should be asked to review and discuss stoma care with the parents.
A stoma bag can be applied once the stoma starts to work unless there are conflicting
wound care issues.

If a primary anoplasty has been performed then the surgeon will give instructions
regarding wound care, feeding and antibiotic duration.
In more complex anomalies there may be other forms of stoma or drainage tube present
and these can be explained by the surgical team and appropriate care instructed.
Screening for associated anomalies (VACTERL Screen)
All babies with ananorectal malformation should have a full clinical examination for
additional anomalies. This should be documented in the admission notes.
In particular it is recognised that ARMs can form one component of an association of

abnormalities known as VACTERL.
This stands for
V – Vertebral
A – Anorectal
C – Cardiac
TE – Tracheo-esophageal
R – Renal
L – Limb
Abnormalities in each of these body systems should be looked for specifically and if
found then an appropriate specialist referral made.
The screening of this association has been the subject of a recent multi-centre audit.
This found that the 100% standard was not met in 50% of the criteria set, here at
NNUH. Deficiencies were related to obtaining vertebral and spinal column imaging as
well as documentation of clinical findings.
A proforma detailing the exact test for each system and the appropriate management
has been produced and should be completed for each baby with an ARM.
This proforma is included as an appendix

Each baby born with an ARM should have the screening for VACTERL completed
before discharge to ensure items are not missed and timely referrals can be made.
A cardiac echo should be obtained prior to any surgery. If this is not possible then
surgery should only go ahead if there are no clinical concerns of a cardiac lesion and
the anaesthetist is happy to proceed without.
Passage of a Nasogastric tube should have been performed in the initial evaluation and
the position of the tip confirmed ideally on CXR / AXR. This effectively rules out
Oesophageal atresia / Tracheo – oesophageal fistula, but exceptions can occur – see
references.

In addition to VACTERL the presence of dysmorphism should lead to the consideration

of genetic /chromosomal studies and a referral to the genetics department at
Addenbrookes Hospital.
If there are multiple components (more than 2) of the VACTERL association present
then genetic studies should also be done and a genetic referral made. This should be
discussed with the parents and named paediatric surgeon.
Planning for discharge and follow-up arrangements
Specific follow-up arrangements depends on the individual patient and their form of
anorectal malformation.
The Paediatric Surgeon involved will arrange to see the baby in clinic.
Premature neonates or those with associated anomalies should also be reviewed by a

neonatologist to ensure they are developing normally and to provide a medical review.
Baby’s transferred from outside the Norwich area should be referred to their local
paediatrician for regular follow-up and coordination of multi-disciplinary team
involvement. Please refer to the guidelines for The Follow-up of Infants on NICU
CA2082
A neonatal outreach or children’s community nurse should be informed and follow-up

visits at home arranged.
Open access to the Children’s Assessment Unit should be arranged and the parents
informed of the contact details.
If a stoma has been formed then a community stoma nurse should visit the family at
home to assess ongoing needs, ensure stoma care supplies are available and
troubleshoot for problems such as prolapse and bag leakage. The stoma nurse and
community nurses should ensure the parents can recognise that high output from the
stoma may need medical attention.
The General Practitioner should receive a copy of the neonatal discharge letter which
should include any appropriate surgical instructions.
Summary of further management and outcome
Patients who have had a primary anoplasty may need no further surgery but will be
followed up by the paediatric surgeon. Any constipation or soiling will be monitored by
them and advice on laxative use will be given.

A patient who has received an initial colostomy will be allowed to grow and develop for a
number of months (dependent on the type of malformation they have) before definitive
surgery is planned.
They may undergo further investigations within this period to assess the malformation.
Once the definitive surgery is performed the paediatric surgeon will instruct parents if
they require anal dilatations to be performed.
Once the new anus has healed up then the colostomy can be closed. This is a further
surgical procedure and requires an admission of between a few days and 1-2 weeks in
most cases.
The outcome for anorectal malformations is very dependent on the specific abnormality.
The paediatric surgeon will discuss issues such as constipation, soiling and
incontinence with the parents. Associated abnormalities such as heart and urological
conditions may influence the overall progress of the patient.
Clinical audit standards / monitoring compliance
To ensure that care is compliant with the above standards, the following monitoring
processes will be undertaken:
Planned audit of this guideline should be carried out at regular intervals suggested 3-5
yearly intervals
Suggested criteria are as follows but can be modified according to perceived needs at
the time of audit.
1) Diagnosis of anorectal malformations within 24hrs of birth
2) Documentation of clinical examination by surgeon at diagnosis
3) Use of the VACTERL screening proforma (Note: audit of the completion of this
proforma may be subject to a separate audit process)
4) Involvement of stoma nurse if colostomy formation has been performed.
5) Documentation of at least one discussion with parents concerning the diagnosis
and subsequent plan of treatment.
The audit results will be sent to the Paediatric surgery team / Clinical Director who will
ensure that these are discussed at relevant governance meetings to review the results
and make recommendations for further action.

Summary of development and consultation process undertaken before

registration and dissemination
The authors listed above drafted the guideline. During its development it was discussed
at a multidisciplinary guideline meeting of the Paediatric Surgical Department and the
Neonatal Unit, changes suggested were discussed and incorporated.
It was subsequently circulated for comment to the Paediatric Medicine and Surgical
Departments and the Neonatal Unit (Consultants, Specialist Registrars, Advanced
Neonatal Nurse Practitioners, Sisters and Senior Staff Nurses).
In addition the draft guideline was forwarded to the fetomaternal obstetric department,
midwifery unit, paediatric radiologists and stoma nurses. Suggestions for further
improvement were incorporated; consensus was reached for non-evidence based
treatment (advised according to current expert opinion/best practice).
It was also discussed at the Neonatal Audit and Guideline meeting on 22nd October
2014
This version has been endorsed by the Clinical Guideline Assessment Committee
Distribution list/ dissemination method
Neonatal Unit / NNUH Intranet clinical guidelines
References
1. Lakhoo K. Fetal counselling for surgical conditions. Early Hum Dev 2012; 88: 9-13.
2. Peña A. Management of anorectal malformations during the newborn period. World

J Surg 1993; 17:385-92.
3. Pena A, Migotto -Krieger M, Levitt MA. Colostomy in anorectal malformations: a

procedure with serious but preventable complications. J Pediatr Surg 2006; 41:748-
756.
4. Soccorso G, England RJ, Godbole PP, Fisher RM, Marven SS. Mind the gap:
delayed diagnosis of oesophageal atresia and trachea-oesophageal fistula due to
passage of a nasogastric tube. Arch Dis Child Fetal Neonatal Ed 2012; 97:F463-4.
5. Solomon B. VACTERL / VATER Association. Orphanet J Rare Dis 2011; 6:56.
6. Turowski C, Dingemann J and Gillick J. Delayed diagnosis of imperforate anus: an

unacceptable morbidity. Pediatr Surg Int. 2010; 26:1083-1086.

Associated Documents: appendix VACTERL Screening Proforma



Appendix 2
1) Diagrams depicting the most common anorectal malformations in males and

females.
a) Recto-perineal fistula
b) Rectourethral Bulba fistula

c) Rectourethral Prostatic fistula
d) Rectovesical fistula

e) Rectovestibular fistula (female)
f) Persistent cloaca

Appendix 3
Element to be
Trust GuidelineLead Monitoring
for the Management ofFrequency
AnorectalLead Responsible forin Neonates
Malformation Reportingand Infants
Sharing and
monitored Responsible for Tool / Method of developing action arrangements disseminating
monitoring of monitoring monitoring plan & acting on lessons learned &
(For NHSLA documents (Committee or group
recommendations recommended
this must include all Level (Title needed & where monitoring
changes in practice
1 minimum requirements) name of individual results and action
as a result of
where plan progress are
monitoring
appropriate) reported to)
compliance with
this document
Diagnosis of Anorectal Paediatric Surgery

Malformations within and neonatal
R England Audit 3yrs R England
24hrs of birth Governance
meetings
Documentation of clinical Paediatric Surgery

examination by surgeon and neonatal
at diagnosis R England Audit 3yrs R England
Governance
meetings
Paediatric Surgery
To be
and neonatal
Separate Audit determined
Use of the VACTERL Governance
R England (PAEDS SURG- following R England
screening proforma meetings and
14-15-01) prospective
Regional Paediatric
audit.
Colorectal group.
Involvement of stoma
nurse if colostomy Paediatric Surgery
formation has been and neonatal
R England Audit 3yrs R England
performed. Governance
meetings
Documentation of at least
one discussion with Paediatric Surgery
parents concerning the and neonatal
R England Audit 3 yrs
diagnosis
Author/s:and
Mr Richard England Date of issue: November 2014 Governance
Valid until: November
subsequent plan of 2017 Guideline Ref No: CA6039 v1.1 meetings
treatment.

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Trust Guideline for the Management of Anorectal Malformations in

Neonates and Infants

A Clinical Guideline recommended for use

Neonatal Intensive Care Unit (NNUH)

By: Neonatal & Paediatric medical, surgical & nursing staff

For: Neonates and infants admitted to NICU

Key words: Anorectal malformation, Imperforate anus, colostomy

Written by: Mr Richard England, Consultant Paediatric Surgeon (NNUH)

All Neonatologists, Paediatric / Neonatal Surgeons & Paed

Assessed and Clinical Guidelines Assessment Panel

Clinical Standards Group (NNUH)

Document issued: November 2014

To be reviewed before: November 2017

To be reviewed by: Mr Richard England

Document supersedes: None

Document Reg. No: CA6039

Author/s: Mr Richard England Date of issue: November 2014

Antenatal Examination Delayed

Author/s: Mr Richard England Date of issue: November 2014

Definitions of Terms Used

Congenital causes of bowel obstruction may occasionally be noticed on antenatal scans

Mode of delivery should be determined by obstetric principles.

A plan for a paediatrician to attend the delivery should be in place.

Postnatal Baby Check

Author/s: Mr Richard England Date of issue: November 2014

Even if there were no antenatal concerns regarding bowel obstruction on antenatal

The examination findings should be carefully documented.

There are continued reports in the literature of delayed presentation of anorectal

The baby should be re-admitted to hospital as an emergency and seen quickly by a

Author/s: Mr Richard England Date of issue: November 2014

A Group and Save sample should also be taken.

IV antibiotics including anaerobic cover should be given. The duration of cover is

All babies should have received IV or IM Vitamin K. Oral vitamin K is unreliably

Surgical management in the newborn period

The surgical management is determined by an accurate diagnosis of the type of

The simplest classification of anomalies is shown in table 1. However it should be noted

Author/s: Mr Richard England Date of issue: November 2014

Author/s: Mr Richard England Date of issue: November 2014

Table 1: Classification of Anorectal Malformations

Appendix 1: Diagrams depicting the most common ARM types.

In general a perineal fistula might be amenable to an anoplasty which is a simpler

The timing of the colostomy is determined by the paediatric surgeon as it depends on

Feeds can be started according to the surgeons instructions.

Author/s: Mr Richard England Date of issue: November 2014

Screening for associated anomalies (VACTERL Screen)

In particular it is recognised that ARMs can form one component of an association of

This stands for

This proforma is included as an appendix

Author/s: Mr Richard England Date of issue: November 2014

In addition to VACTERL the presence of dysmorphism should lead to the consideration

Planning for discharge and follow-up arrangements

Premature neonates or those with associated anomalies should also be reviewed by a

A neonatal outreach or children’s community nurse should be informed and follow-up

Summary of further management and outcome

Author/s: Mr Richard England Date of issue: November 2014

Clinical audit standards / monitoring compliance

Author/s: Mr Richard England Date of issue: November 2014

Summary of development and consultation process undertaken before

Distribution list/ dissemination method

Neonatal Unit / NNUH Intranet clinical guidelines

2. Peña A. Management of anorectal malformations during the newborn period. World

3. Pena A, Migotto -Krieger M, Levitt MA. Colostomy in anorectal malformations: a

5. Solomon B. VACTERL / VATER Association. Orphanet J Rare Dis 2011; 6:56.

6. Turowski C, Dingemann J and Gillick J. Delayed diagnosis of imperforate anus: an