Trust Guideline for the Management of Anorectal Malformations in

Neonates and Infants

A Clinical Guideline recommended for use
Neonatal Intensive Care Unit and Paediatric
For Use in:
Wards at Jenny Lind Children’s Hospital
Neonatal and Paediatric medical, surgical and
By:
nursing staff
Neonates and infants admitted to NICU or
For:
Paediatric Wards
Division responsible for document: Women and Children’s Services
Anorectal malformation, Imperforate anus,
Key words:
colostomy
Joshua Cave Paediatric Surgical Registrar, Billie
Name and job title of document
Dean CNS, Mr Richard England, Consultant
author:
Paediatric Surgeon (NNUH)
Name and job title of document Mr AB Mathur, Service Director Paediatric
author’s Line Manager: Surgery, Women’s and Children’s Division
All Neonatologists, Paediatric / Neonatal
Surgeons and Paed Gastroenterologists
(Dr R Roy, Dr P Clarke, Dr M Dyke, Dr D Booth,
Supported by: Dr P Muthukumar, Dr F Walston, Dr H O’Reilly,
Mr M S Kulkarni, Mr A B Mathur, Mr A Minocha,
Mr A Ram, Mr R Anbarasan, Dr M A Morris, Dr
G Briars) (NNUH)
Clinical Guidelines Assessment Panel
Assessed and approved by the: If approved by committee or Governance Lead
Chair’s Action; CGSAP tick here 
Date of approval: 10/11/2021
Ratified by or reported as approved
Clinical Safety and Effectiveness Sub-Board
to (if applicable):
To be reviewed before:
This document remains current after this 10/11/2024
date but will be under review
To be reviewed by: Mr Richard England
Reference and / or Trust Docs ID No: 10836
Version No: 3
Compliance links: (is there any NICE
None
related to guidance)
If Yes - does the strategy/policy N/A

Guideline for: The Management of Anorectal Malformations in Neonates and Infants

Author/s: Mr Richard England Author/s title: Consultant Paediatric Surgeon
Approved by: CGAP Date approved: 10/11/2021 Review date: 10/11/2024
Available via Trust Docs Version: 3 Trust Docs ID: 10836 Page 1 of 19
deviate from the recommendations of
NICE? If so why?

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.

Guideline for: The Management of Anorectal Malformations in Neonates and Infants

Author/s: Mr Richard England Author/s title: Consultant Paediatric Surgeon
Approved by: CGAP Date approved: 10/11/2021 Review date: 10/11/2024
Available via Trust Docs Version: 3 Trust Docs ID: 10836 Page 2 of 19
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants

Version and Document Control:

Version Date of
Change Description Author
Number Update
11/11/202 Amended following audit, flowchart
3 Mr Richard England
1 amended

This is a Controlled Document

Printed copies of this document may not be up to date. Please check the hospital intranet
for the latest version and destroy all previous versions.
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants

Quick reference guide

 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
Objectives

Ensure best peri-natal practice in the diagnosis, investigation and management of a

baby born with an anorectal malformation (ARM).

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. The recommendations are derived from standard national
practice in paediatric surgery, published literature, previous regional audits and expert
opinion.

Definitions of Terms Used

Anorectal malformation: A wide range of congenital abnormalities 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: 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. An anoplasty is a less complicated form of PSARP.
Other variations of this operation are beyond the remit of this document but this can be
used as a general term for ward staff.

Fistula: Abnormal Connection between the rectum and another structure such as
bladder/urethra/vestibule.

Imperforate anus: Historical term which describes the absence of an anal opening in most
ARM.

Muscle complex: Description of anal sphincter muscle group which normally surrounds
the anus. In ARM the anus is either not formed or is outside this complex. Definitive
surgery aims to reconstruct the anus in the centre of this muscle complex to give the best
chance of future continence.

Perineum: Tissue between scrotum or vulva and anus

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 and in rare cases can be fatal.

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 which will typically occur electively after discharge from hospital.
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
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 and a
chest x-ray performed with a tube placed as far down as possible. This is to rule out
oesophageal atresia which may be associated with an ARM.

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. Patency can be gently assessed by the passage of a rectal thermometer. External
appearances may appear normal in the presence of an anal or rectal membrane or
atresia.

If the anus appears absent then an abnormal opening or 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 by
firm parting of the labia.
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
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 extremely distended and the baby will be very dehydrated.
This scenario is avoidable and potentially life threatening. All cases of an anorectal
malformation should be diagnosed within 24 hours of birth (see audit section).

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, clotting, Group
and save and blood glucose taken as well as blood cultures if delayed presentation or risk
factors for sepsis.

In addition electrolytes, urea and creatinine may be useful if taken at more than 24hrs of
age.

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 hourly. 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
Trustdocs Id: 9998

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.
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants

The diagnosis and an outline of likely management over the short term should be
discussed with parents as soon as possible and documented on the parent
communication sheets in the notes.

Surgical management in the new-born 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. If practicable the attending surgical team, ahead of any operative
intervention, should discuss with a member of the paediatric colorectal team regarding the
need for a stoma and appropriate stoma formation as well as plan for ongoing
management.

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 as risk of infection and wound breakdown is lower. The decision whether
this is possible is made by the paediatric colorectal surgeon and depends on the condition
of the baby in the first 24 hours of life.

In some cases it may be appropriate to dilate perineal or vestibular anomalies regularly

with hegar dilators. The need for this will be determined and commenced by the
paediatric surgeon with support by a surgical nurse specialist.

Most of the other anomalies require a colostomy and the urgency of this will be
determined by clinical assessment of the baby by the paediatric surgeon. The colostomy
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.

All ARM babies will need review by surgical nurse specialist who will assist in establishing
stoma/perianal care/dilatations and follow up ahead of definitive surgery.

It is essential that a paediatric surgeon has a thorough discussion with parents as to the
type of abnormality, immediate management and expected clinical course including a
discussion about plans for definitive surgery and expected long term issues. This should
be documented in the communication section of the patient notes.

Post-operative period
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
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 surgical nurse specialist should be asked to review and discuss stoma care with the
parents. Further stoma care training will be provided by NICU nurses competent to
provide teaching.

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.

Anorectal Malformations are complex conditions that require intensive medical contact in
infancy and carries long term consequences, it may well also be associated with other
serious malformations. It has been shown this can affect childhood behaviour,
educational attainment and quality of life. It is therefore important that parents are offered
psychological support for the journey ahead. A referral after surgery to clinical psychology
should be encouraged.

Screening for associated anomalies (VACTERL Screen)

All babies with an anorectal 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 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.
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
A proforma (Appendix 1) detailing the exact test for each system and the appropriate
management has been produced and should be completed for each baby with an ARM.

VACTERL proforma completion is an auditable criteria.

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, for
example – management of cardiac, renal or spinal anomalies may be all need attention in
the neonatal period.

A recent audit of departmental use of the proforma has shown that this is poorly
completed and in particular documentation of referrals was only complete 29% of the
time. This exposes patients to risks of missed malformations and delay in appropriate
management.

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 clinical 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.

Screening tests should not be deferred to outpatient follow-up or for completion at local
district hospitals as this leads to delay, missing opportunities for age dependent
investigations and poor communication over test results.

Planning for discharge and follow-up arrangements

Specific follow-up arrangements depend on the individual patient and their form of
anorectal malformation.

The Paediatric Surgeon involved will arrange to see the baby in clinic and the paediatric
surgical registrar will dictate a surgical summary discharge letter. The surgical section of
Badger discharge letter should also be completed.

The surgical nurse specialist will arrange to follow up in telephone clinic and 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.

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

neonatologist in outpatients 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 Trustdocs Id: 1237
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants

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.

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 surgical nurse specialist
5) Documentation of at least one discussion with parents concerning the diagnosis
and subsequent plan of treatment.
6) Involvement of clinical psychologist with interest in neonatal surgery
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
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. Version 2 of this document was audited in May 2021 and redrafted following
publication of the audit results.

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

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

7. Lorraine Ludman, Gut feelings: A psychologists 20 year journey with paediatric

surgeons. JRSM 2003; 96:87-91

8. Management of Newborn babies at increased risk of developing neonatal infection

Trustdocs Id: 9998

9. The Follow-up of Infants on NICU Trustdocs Id: 1237

 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants

Associated Documents: Appendix 1 VACTERL Screening Proforma

Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
Appendix 2

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

females.

a) Recto-perineal fistula

b) Rectourethral Bulba fistula

 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
c) Rectourethral Prostatic fistula

d) Rectovesical fistula
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants

e) Rectovestibular fistula (female)

f) Persistent cloaca

Lead
Responsible Frequency of Lead Responsible for Reporti
Monitoring Tool /
for monitoring developing action arrange
Element to be monitored) Method of
monitoring plan & acting on
monitoring
recommendations

R England Audit 3 years R England Paediat

Diagnosis of Anorectal neonata
Malformations within 24hrs meeting
of birth
 Trust Guideline for the Management of Anorectal Malformations in
Neonates and Infants
Documentation of clinical Paediat
examination by surgeon at R England Audit 3 years R England neonata
diagnosis meeting
To be Paediat
Separate Audit determined neonata
Use of the VACTERL
R England (PAEDS SURG-14- following R England meeting
screening proforma
15-01) prospective Paediat
audit. group.
Involvement of stoma
Paediat
nurse if colostomy
R England Audit 3 years R England neonata
formation has been
meeting
performed.
Documentation of at least
one discussion with Paediat
parents concerning the R England Audit 3 years R England neonata
diagnosis and subsequent meeting
plan of treatment.