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Review Article

Current Concepts in the Management of Anorectal

Malformations
Carlos A. Reck‑Burneo
Department of Pediatric Surgery, Medical University of Vienna, Vienna, Austria

Abstract
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

Introduction malformation first by showing air in the rectum.[7] No decision

as to the correct management should be performed before the
Anorectal malformations (ARMs) occur in approximately 1
first 24 h of life as the colonic intraluminal pressure would
of every 5000 newborns.[1] That represents around 1.5 million
not suffice to expel meconium through a small fistula.[1] An
babies born each year worldwide with this condition. This
abdominal ultrasound should be performed to evaluate for
number is an extrapolation that probably underrepresents the
hydronephrosis and in a female for hydrocolpos [Figure 1].[6,8,9]
actual incidence.
Sacrospinal defects occur in 30% of patients, and even though
The most common sequelae, when not treated properly,
this does not merit critical evaluation, it will have a significant
are faecal incontinence, urinary incontinence and sexual
impact on establishing functional prognosis. The quality of
disfucntion.[2] As a consequence, social isolation can lead to
the sacrum and the presence of a tethered cord or presacral
depression and mental health issues. ARMs, unfortunately,
mass are essential and in combination with the original
rank highly amongst mistreated and/or undertreated congenital
diagnosis will help us evaluate the potential for continence.
illnesses. Here, we present an overview and essential
Therefore, a sacral X‑ray and evaluation of the spinal cord
management pearls.
either by ultrasound or a magnetic resonance imaging are
necessary.[1,10] Finally, the decision needs to be made towards
Newborn Baby with Anorectal Malformation the necessity of a colostomy.
In the first days of life and after neonatal stabilising interventions
are in place, it is essential to rule out comorbidities. Screening Colostomy
for the whole spectrum of the vertebral defects, anal atresia,
Depending on the type of malformation, a colostomy will
cardiac defects, tracheoesophageal fistula, renal anomalies and
allow decompression of the intestine, protection of a repaired
limb abnormalities association (VACTERL) is paramount and
should not be delayed.[2,4‑6] Address for correspondence: Dr. Carlos A. Reck‑Burneo,
After careful perineal examination, a prone cross table Department of Pediatric Surgery, Medical University of Vienna, Währinger
Gürtel 18‑20, 1190 Vienna, Austria.
X‑ray after the 24 h of life can help to establish the type of E‑mail: [email protected]

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DOI:
10.4103/HMJ.HMJ_66_18 How to cite this article: Reck‑Burneo CA. Current concepts in the
management of anorectal malformations. Hamdan Med J 2018;11:100-4.

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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
malformation

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 Anorectal Malformation Types and Clinical
establish the appropiate diagnosis.
Approach
Children born in centres where no dedicated paediatric In 2005, the Krickenbeck classification was introduced as
surgical department exists, or if; the surgeons do not have a more precise tool to classify and diagnose ARM, thus
enough experience treating ARMs; it is recommended to allowing better prognostic and therapeutic prediction of the
perform a colostomy and refer the child to a specialised underlying problem.[13] [Table 1] The details of each diagnosis
centre. The colostomy is still the safest way to manage are beyond the scope of this review, and for a more detailed
children with ARM and should always be used in cases of explanation, we suggest reading a paediatric surgical textbook
doubt. Furthermore, in cases where the rectal air bubble [Figure 2]. I do want to emphasise some key concepts and
seems to be above the coccyx, a colostomy will allow for will detail some of those here.
a distal colostogram. When perfroming a colsotomy please
consider that this surgical procedure also carries high
morbidity and also has peculiarities described elsewhere.[11] Female
Moreover, in developing countries, colostomy care can be Rectovestibular fistula
challenging and this can lead to skin affections and irritation. The most common female anorectal malformation is a
The accepted consensus is to do a colostomy with separated fistula located in the vestibule, outside the hymen and often
stomas in the first mobile portion of the descending colon erroneously labelled as a rectovaginal fistula.[14] In experienced
as to avoid prolapse and to prevent stool from entering the hands, this defect can be operated primarily and does not
urinary tract.[12] require a colostomy.[15,16] Decompression and emptying of

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Reck-Burneo: Basic concepts in the management of ARM’s

be dilated to allow for appropriate meconium emptying and

Table 1: Krickenbeck classification of anorectal malformations
the repair delayed for 2–3 months. It is essential to allow
Major clinical groups Rare variants complete bowel emptying through the fistula as otherwise
Perineal (cutaneous) fistula Pouch colon atresia/stenosis a megarectum will complicate the procedure and worsen
Rectourethral fistula Rectal atresia/stenosis the prognosis.[26] The most common complication as in
Prostatic bulbar Rectovaginal fistula most ARM will be constipation and post‑operative bowel
Rectovesical fistula H‑type fistula others
management, especially in the initial years will ensure an
Vestibular fistula
excellent prognosis later.[27]
Cloaca
ARM with no fistula Rectourethral fistula
Anal stenosis This is the most common anorectal malformation in males.
ARM: Anorectal malformations
The rectum can connect to the urethra in any part of its
trajectory, but it is most commonly connected to the bulbous
meconium are through the fistula, and in some cases, dilations urethra and in worst cases to the prostate.[9] For this type of
can be considered to bridge to definitive surgery.[1] As a rule malformation, an initial colostomy is recommended to prevent
of thumb, if there are three perineal orifices in a female, the contamination of the urinary tract and to allow a proper
diagnosis is a perineal fistula or a vestibular fistula. In both diagnosis in the form of a distal colostogram is essential.
cases, primary repair is to be considered, and the long‑term In cases of a prostatic fistula, an abdominal or laparoscopic
prognosis is good given a normal sacrum and a normal spinal approach may be necessary preceding the posterior sagittal
cord with no pre‑sacral mass.[9,17,18] anorectoplasty.[28] The potential for continence will also be
Cloaca given in part by the baseline diagnosis on top of the quality
A single perineal orifice in the place a normal urethra of the sacrum and spinal cord. Prostatic fistulas often show
would be located, is by definition a cloaca, given the an anteriorly located muscle complex and in many cases have
absence of an anus Figures 2 and 4. [19] This is the third a decreased buttock crease which has a poor prognosis for
most common female anorectal malformation and in many continence. In many of these cases, bowe management to
cases misdiagnosed.[20,21] The variations and classification achieve social incontinence is often required for the whole
are out of the scope of this review, and further reading life of the individium.[29]
is recommended. [22] In a cloaca, a colostomy should be Bladder neck fistula
performed. Urological abnormalities are frequent with This is the defect with the worst prognosis in males.
this abnormality and urological emergencies need to be A colostomy should always be performed when suspected. In
addressed.[8] Thus, a full urological evaluation is necessary this group, laparoscopy is helpful as a pelvic approach may
after birth or as soon as possible. If an hydrocolpos is not allow to reach the rectum. Laparoscopy will also facilitate
present, it needs to be permanently drained at the time of the
separation of the rectum from the bladder. Even though no
colostomy creation. The surgical care of the cloaca should
common wall exists between the bladder and the rectum, both
be referred to a specialised centre with experience treating
structures can be tightly attached and separation is difficult,
it. Its complexity can range from mild cases with a short
especially when the attachment is prolonged beyond the
common channel to complex cases that have a long common
fistulous tract. In some cases where the entry of the fistula is
channel.[23] The treatment option varies depending on the
perpendicular to the bladder, laparoscopy is especially suited
length of the urethra and the common channel. Management
and the separation easy to perform. The outcome of bladder
includes total urogenital mobilisation for milder cases with
neck fistulas is commonly faecal incontinence, and when the
a short channel and a complete dissection of the vagina
surgery is poorly performed, there is a high risk of urinary
from the urethra in cases with a long channel. In many
incontinence.[30]
cases, a colonic interposition is required to make up for a
missing or short vagina.[24] Extensive post‑operative care Anal dilations
and long‑term management are essential on top of a good Recommendations are changing with regard to anal dilations
technical operation by an experienced surgeon.[24,25] with studies currently looking at the exact indication for them.
previous management stated to start dilations at 2–4 weeks
Males after the procedure, and this still applies to most cases. Parents
have to learn to do it by themselves. In some centres, weekly
Perineal fistula dilations in the hospital are preferred to daily parental dilations
This is the lowest of all defects in males and it can mislead allowing for sedation and better pain therapy to prevent trauma.
the surgeon into believing that correction is simple. The
anterior wall is usually fused to the urethra, and urethral Functional sequela
injury is possible.[1] When technically well performed, good The most common sequela is constipation, and it is
continence can be expected. This defect can be repaired by utterly vital to aggressively treat it to avoid a mega
primary repair in experienced hands. If not, the fistula can rectosigmoid. [31] Undertreated constipation will lead to

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Reck-Burneo: Basic concepts in the management of ARM’s

incontinence and overflow pseudoincontinence. This needs 7. Reck‑Burneo CA, Vilanova‑Sanchez A, Wood RJ, Levitt MA,
to be aggresively treate to prevent persisten incontinence. Bates DG. Imaging in anorectal and cloacal malformations. Pediatr
Radiol 2018;48:443‑4.
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for imperforate anus at birth. J Pediatr Surg 1990;25:276‑7.
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16. Menon P, Rao KL. Primary anorectoplasty in females with common
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and will contribute to the management of this chronic 2007;42:1103‑6.
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Conflicts of interest pii: S0022‑3468(17)30644‑9.
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Wood RJ, et al. Gynecologic anatomic abnormalities following anorectal
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