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Malformation Anorectal

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Review Article
Current Concepts in the Management of Anorectal
Carlos A. Reck‑Burneo
Department of Pediatric Surgery, Medical University of Vienna, Vienna, Austria
Anorectal malformations (ARMs) occur in approximately 1 of every 5000 newborns and management still differs widely among practitioners.
In this review, we address some of the currently accepted concepts in management. Mismanagement can have devastating consequences such
as fecal incontinence, urinary incontinence, and sexual dysfunction. We briefly review common peculiarities of the most common cases the
and initial management of the newborn. I intend to present a brief overview without going into details but to motivate interest and further
reading in this topic. Further, the recommendation is made that case referral to a center with the expertise and ideally a high volume case load,
which provides the highest benefits for the patient.
Keywords: Anorectal malformations, congenital illnesses, incontinence
Anorectal malformations (ARMs) occur in approximately 1
of every 5000 newborns.[1] That represents around 1.5 million
babies born each year worldwide with this condition. This
number is an extrapolation that probably underrepresents the
actual incidence.
The most common sequelae, when not treated properly,
are faecal incontinence, urinary incontinence and sexual
disfucntion.[2] As a consequence, social isolation can lead to
depression and mental health issues. ARMs, unfortunately,
rank highly amongst mistreated and/or undertreated congenital
illnesses. Here, we present an overview and essential
management pearls.
Newborn Baby with Anorectal Malformation
In the first days of life and after neonatal stabilising interventions
are in place, it is essential to rule out comorbidities. Screening
for the whole spectrum of the vertebral defects, anal atresia,
cardiac defects, tracheoesophageal fistula, renal anomalies and
limb abnormalities association (VACTERL) is paramount and
should not be delayed.[2,4‑6]
After careful perineal examination, a prone cross table
X‑ray after the 24 h of life can help to establish the type of
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malformation first by showing air in the rectum.[7] No decision
as to the correct management should be performed before the
first 24 h of life as the colonic intraluminal pressure would
not suffice to expel meconium through a small fistula.[1] An
abdominal ultrasound should be performed to evaluate for
hydronephrosis and in a female for hydrocolpos [Figure 1].[6,8,9]
Sacrospinal defects occur in 30% of patients, and even though
this does not merit critical evaluation, it will have a significant
impact on establishing functional prognosis. The quality of
the sacrum and the presence of a tethered cord or presacral
mass are essential and in combination with the original
diagnosis will help us evaluate the potential for continence.
Therefore, a sacral X‑ray and evaluation of the spinal cord
either by ultrasound or a magnetic resonance imaging are
necessary.[1,10] Finally, the decision needs to be made towards
the necessity of a colostomy.
Depending on the type of malformation, a colostomy will
allow decompression of the intestine, protection of a repaired
Address for correspondence: Dr. Carlos A. Reck‑Burneo,
Department of Pediatric Surgery, Medical University of Vienna, Währinger
Gürtel 18‑20, 1190 Vienna, Austria.
E‑mail: [email protected]
This is an open access journal, and articles are distributed under the terms of the Creative
Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to
remix, tweak, and build upon the work non-commercially, as long as appropriate credit
is given and the new creations are licensed under the identical terms.
For reprints contact: [email protected]
How to cite this article: Reck‑Burneo CA. Current concepts in the
management of anorectal malformations. Hamdan Med J 2018;11:100-4.
© 2018 Hamdan Medical Journal | Published by Wolters Kluwer - Medknow
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Reck-Burneo: Basic concepts in the management of ARM’s
Figure 1: Correct way to examine an anorectal malformation in females
Figure 2: Positioning of the child for the correction of an anorectal
Figure 3: 3D reconstruction of a cloacal malformation
Figure 4: Cloacal malformation
perineum, renal protection from recurrent urinary tract
infections and allow performing a distal colostogram to
establish the appropiate diagnosis.
Children born in centres where no dedicated paediatric
surgical department exists, or if; the surgeons do not have
enough experience treating ARMs; it is recommended to
perform a colostomy and refer the child to a specialised
centre. The colostomy is still the safest way to manage
children with ARM and should always be used in cases of
doubt. Furthermore, in cases where the rectal air bubble
seems to be above the coccyx, a colostomy will allow for
a distal colostogram. When perfroming a colsotomy please
consider that this surgical procedure also carries high
morbidity and also has peculiarities described elsewhere.[11]
Moreover, in developing countries, colostomy care can be
challenging and this can lead to skin affections and irritation.
The accepted consensus is to do a colostomy with separated
stomas in the first mobile portion of the descending colon
as to avoid prolapse and to prevent stool from entering the
urinary tract.[12]
Hamdan Medical Journal ¦ Volume 11 ¦ Issue 3 ¦ July-September 2018
Anorectal Malformation Types and Clinical
In 2005, the Krickenbeck classification was introduced as
a more precise tool to classify and diagnose ARM, thus
allowing better prognostic and therapeutic prediction of the
underlying problem.[13] [Table 1] The details of each diagnosis
are beyond the scope of this review, and for a more detailed
explanation, we suggest reading a paediatric surgical textbook
[Figure 2]. I do want to emphasise some key concepts and
will detail some of those here.
Rectovestibular fistula
The most common female anorectal malformation is a
fistula located in the vestibule, outside the hymen and often
erroneously labelled as a rectovaginal fistula.[14] In experienced
hands, this defect can be operated primarily and does not
require a colostomy.[15,16] Decompression and emptying of
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Reck-Burneo: Basic concepts in the management of ARM’s
Table 1: Krickenbeck classification of anorectal malformations
Major clinical groups
Perineal (cutaneous) fistula
Rectourethral fistula
Prostatic bulbar
Rectovesical fistula
Vestibular fistula
ARM with no fistula
Anal stenosis
ARM: Anorectal malformations
Rare variants
Pouch colon atresia/stenosis
Rectal atresia/stenosis
Rectovaginal fistula
H‑type fistula others
meconium are through the fistula, and in some cases, dilations
can be considered to bridge to definitive surgery.[1] As a rule
of thumb, if there are three perineal orifices in a female, the
diagnosis is a perineal fistula or a vestibular fistula. In both
cases, primary repair is to be considered, and the long‑term
prognosis is good given a normal sacrum and a normal spinal
cord with no pre‑sacral mass.[9,17,18]
A single perineal orifice in the place a normal urethra
would be located, is by definition a cloaca, given the
absence of an anus Figures 2 and 4. [19] This is the third
most common female anorectal malformation and in many
cases misdiagnosed.[20,21] The variations and classification
are out of the scope of this review, and further reading
is recommended. [22] In a cloaca, a colostomy should be
performed. Urological abnormalities are frequent with
this abnormality and urological emergencies need to be
addressed.[8] Thus, a full urological evaluation is necessary
after birth or as soon as possible. If an hydrocolpos is
present, it needs to be permanently drained at the time of the
colostomy creation. The surgical care of the cloaca should
be referred to a specialised centre with experience treating
it. Its complexity can range from mild cases with a short
common channel to complex cases that have a long common
channel.[23] The treatment option varies depending on the
length of the urethra and the common channel. Management
includes total urogenital mobilisation for milder cases with
a short channel and a complete dissection of the vagina
from the urethra in cases with a long channel. In many
cases, a colonic interposition is required to make up for a
missing or short vagina.[24] Extensive post‑operative care
and long‑term management are essential on top of a good
technical operation by an experienced surgeon.[24,25]
Perineal fistula
This is the lowest of all defects in males and it can mislead
the surgeon into believing that correction is simple. The
anterior wall is usually fused to the urethra, and urethral
injury is possible.[1] When technically well performed, good
continence can be expected. This defect can be repaired by
primary repair in experienced hands. If not, the fistula can
be dilated to allow for appropriate meconium emptying and
the repair delayed for 2–3 months. It is essential to allow
complete bowel emptying through the fistula as otherwise
a megarectum will complicate the procedure and worsen
the prognosis.[26] The most common complication as in
most ARM will be constipation and post‑operative bowel
management, especially in the initial years will ensure an
excellent prognosis later.[27]
Rectourethral fistula
This is the most common anorectal malformation in males.
The rectum can connect to the urethra in any part of its
trajectory, but it is most commonly connected to the bulbous
urethra and in worst cases to the prostate.[9] For this type of
malformation, an initial colostomy is recommended to prevent
contamination of the urinary tract and to allow a proper
diagnosis in the form of a distal colostogram is essential.
In cases of a prostatic fistula, an abdominal or laparoscopic
approach may be necessary preceding the posterior sagittal
anorectoplasty.[28] The potential for continence will also be
given in part by the baseline diagnosis on top of the quality
of the sacrum and spinal cord. Prostatic fistulas often show
an anteriorly located muscle complex and in many cases have
a decreased buttock crease which has a poor prognosis for
continence. In many of these cases, bowe management to
achieve social incontinence is often required for the whole
life of the individium.[29]
Bladder neck fistula
This is the defect with the worst prognosis in males.
A colostomy should always be performed when suspected. In
this group, laparoscopy is helpful as a pelvic approach may
not allow to reach the rectum. Laparoscopy will also facilitate
separation of the rectum from the bladder. Even though no
common wall exists between the bladder and the rectum, both
structures can be tightly attached and separation is difficult,
especially when the attachment is prolonged beyond the
fistulous tract. In some cases where the entry of the fistula is
perpendicular to the bladder, laparoscopy is especially suited
and the separation easy to perform. The outcome of bladder
neck fistulas is commonly faecal incontinence, and when the
surgery is poorly performed, there is a high risk of urinary
Anal dilations
Recommendations are changing with regard to anal dilations
with studies currently looking at the exact indication for them.
previous management stated to start dilations at 2–4 weeks
after the procedure, and this still applies to most cases. Parents
have to learn to do it by themselves. In some centres, weekly
dilations in the hospital are preferred to daily parental dilations
allowing for sedation and better pain therapy to prevent trauma.
Functional sequela
The most common sequela is constipation, and it is
utterly vital to aggressively treat it to avoid a mega
rectosigmoid. [31] Undertreated constipation will lead to
Hamdan Medical Journal ¦ Volume 11 ¦ Issue 3 ¦ July-September 2018
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Reck-Burneo: Basic concepts in the management of ARM’s
incontinence and overflow pseudoincontinence. This needs
to be aggresively treate to prevent persisten incontinence.
Urinary incontinence is also frequent especially in cloacas
and bladder neck fistulas. An abdominal X‑ray can help to
diagnose impaction and then stimulant laxatives should be
given titrating to the best dose‑effect amount.[32]
Patients that by 3 years of age are not continent or clean require
aggressive bowel management. In this systematic programme,
parents and patients are instructed on how to keep the colon
empty with either laxatives or an enema programme.[31]
In this review, we have addressed some of the current
concepts in the management of ARM. New ideas that have
gained traction and are currently being explored are the
long‑term outcomes with an emphasis on quality of life.
New concepts such as the health literacy, quality of life
and psychosocial aspects of ARM are still early in research
and will contribute to the management of this chronic
ailment.[33‑35] Great strides are made in the surgical and
operative aspects but other areas require prompt attention and
care. Centralisation is now commonly accepted as the road to
better outcomes in rare diseases. It improves care and with
ith the quality of life in patients with ARM’s. Only a centre
with enough volume can offer enough experience to the
surgical trainee and attending and will have enough volume
to allow a multidisciplinary care and most of all appropriate
follow‑up and correct bowel management. A more oftenly
seen challenge is transition to adult care as adult surgeons
barely know about the lifelong implications that ARM can
have. Research opportunities in this field are extensive and
will hopefully be addressed by a new generation of paediatric
surgeons in this field.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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Hamdan Medical Journal ¦ Volume 11 ¦ Issue 3 ¦ July-September 2018
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