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Current Concepts in The Management of Anorectal Ma

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27]

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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For reprints contact: [email protected]
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.

100 © 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
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.
Urinary incontinence is also frequent especially in cloacas 8. Levitt MA, Bischoff A, Peña A. Pitfalls and challenges of cloaca
and bladder neck fistulas. An abdominal X‑ray can help to repair: How to reduce the need for reoperations. J Pediatr Surg
diagnose impaction and then stimulant laxatives should be 2011;46:1250‑5.
given titrating to the best dose‑effect amount.[32] 9. Peña A. Anorectal malformations. Semin Pediatr Surg 1995;4:35‑47.
10. Bui CJ, Tubbs RS, Oakes WJ. Tethered cord syndrome in children:
Patients that by 3 years of age are not continent or clean require A review. Neurosurg Focus 2007;23:E2.
11. Bischoff A, Levitt MA, Lawal TA, Peña A. Colostomy closure: How to
aggressive bowel management. In this systematic programme,
avoid complications. Pediatr Surg Int 2010;26:1087‑92.
parents and patients are instructed on how to keep the colon 12. Bischoff A, Levitt MA, Peña A. Update on the management of anorectal
empty with either laxatives or an enema programme.[31] malformations. Pediatr Surg Int 2013;29:899‑904.
13. Holschneider A, Hutson J, Peña A, Beket E, Chatterjee S, Coran A, et al.
Preliminary report on the international conference for the development
Conclusion of standards for the treatment of anorectal malformations. J Pediatr Surg
2005;40:1521‑6.
In this review, we have addressed some of the current
14. Levitt MA, Bischoff A, Breech L, Peña A. Rectovestibular
concepts in the management of ARM. New ideas that have fistula – Rarely recognized associated gynecologic anomalies. J Pediatr
gained traction and are currently being explored are the Surg 2009;44:1261‑7.
long‑term outcomes with an emphasis on quality of life. 15. Moore TC. Advantages of performing the sagittal anoplasty operation
for imperforate anus at birth. J Pediatr Surg 1990;25:276‑7.
New concepts such as the health literacy, quality of life
16. Menon P, Rao KL. Primary anorectoplasty in females with common
and psychosocial aspects of ARM are still early in research anorectal malformations without colostomy. J Pediatr Surg
and will contribute to the management of this chronic 2007;42:1103‑6.
ailment.[33‑35] Great strides are made in the surgical and 17. Arnoldi R, Macchini F, Gentilino V, Farris G, Morandi A, Brisighelli G,
operative aspects but other areas require prompt attention and et al. Anorectal malformations with good prognosis: Variables affecting
the functional outcome. J Pediatr Surg 2014;49:1232‑6.
care. Centralisation is now commonly accepted as the road to 18. Kyrklund K, Pakarinen MP, Koivusalo A, Rintala RJ. Bowel functional
better outcomes in rare diseases. It improves care and with outcomes in females with perineal or vestibular fistula treated with
ith the quality of life in patients with ARM’s. Only a centre anterior sagittal anorectoplasty: Controlled results into adulthood. Dis
with enough volume can offer enough experience to the Colon Rectum 2015;58:97‑103.
19. Levitt MA, Peña A. Cloacal malformations: Lessons learned from
surgical trainee and attending and will have enough volume 490 cases. Semin Pediatr Surg 2010;19:128‑38.
to allow a multidisciplinary care and most of all appropriate 20. Levitt MA, Peña A. Anorectal malformations. Orphanet J Rare Dis
follow‑up and correct bowel management. A more oftenly 2007;2:33.
seen challenge is transition to adult care as adult surgeons 21. Rosen NG, Hong AR, Soffer SZ, Rodriguez G, Peña A. Rectovaginal
fistula: A common diagnostic error with significant consequences in
barely know about the lifelong implications that ARM can girls with anorectal malformations. J Pediatr Surg 2002;37:961‑5.
have. Research opportunities in this field are extensive and 22. Peña A. Cloaca – Historical aspects and terminology. Semin Pediatr
will hopefully be addressed by a new generation of paediatric Surg 2016;25:62‑5.
surgeons in this field. 23. Rintala RJ. Congenital cloaca: Long‑term follow‑up results with
emphasis on outcomes beyond childhood. Semin Pediatr Surg
Financial support and sponsorship 2016;25:112‑6.
24. Wood RJ, Reck‑Burneo CA, Dajusta D, Ching C, Jayanthi R, Bates DG,
Nil. et al. Cloaca reconstruction: A new algorithm which considers the role
of urethral length in determining surgical planning. J Pediatr Surg 2017.
Conflicts of interest pii: S0022‑3468(17)30644‑9.
There are no conflicts of interest. 25. Vilanova‑Sanchez A, Reck CA, McCracken KA, Lane VA, Gasior AC,
Wood RJ, et al. Gynecologic anatomic abnormalities following anorectal
malformations repair. J Pediatr Surg 2018;53:698‑703.
References 26. Kyrklund K, Pakarinen MP, Taskinen S, Rintala RJ. Bowel function
1. Wood RJ, Levitt MA. Anorectal malformations. Clin Colon Rectal Surg and lower urinary tract symptoms in males with low anorectal
2018;31:61‑70. malformations: An update of controlled, long‑term outcomes. Int J
2. Shaul DB, Harrison EA. Classification of anorectal Colorectal Dis 2015;30:221‑8.
malformations – Initial approach, diagnostic tests, and colostomy. 27. Rintala RJ. Congenital anorectal malformations: Anything new? J
Semin Pediatr Surg 1997;6:187‑95. Pediatr Gastroenterol Nutr 2009;48 Suppl 2:S79‑82.
3. Minneci PC, Kabre RS, Mak GZ, Halleran DR, Cooper JN, Afrazi A, 28. Shawyer AC, Livingston MH, Cook DJ, Braga LH. Laparoscopic
et al. Screening practices and associated anomalies in infants with versus open repair of recto‑bladderneck and recto‑prostatic anorectal
anorectal malformations: Results from the Midwest pediatric surgery malformations: A systematic review and meta‑analysis. Pediatr Surg Int
consortium. J Pediatr Surg 2018;53:1163‑7. 2015;31:17‑30.
4. Lane VA, Ambeba E, Chisolm DJ, Lodwick D, Levitt MA, Wood RJ, 29. Kyrklund K, Pakarinen MP, Koivusalo A, Rintala RJ. Long‑term
et al. Low vertebral ano‑rectal cardiac tracheo‑esophageal renal limb bowel functional outcomes in rectourethral fistula treated with PSARP:
screening rates in children with anorectal malformations. J Surg Res Controlled results after 4‑29 years of follow‑up: A single‑institution,
2016;203:398‑406. cross‑sectional study. J Pediatr Surg 2014;49:1635‑42.
5. Totonelli G, Catania VD, Morini F, Fusaro F, Mosiello G, Iacobelli BD, 30. Hassett S, Snell S, Hughes‑Thomas A, Holmes K 10‑year outcome of
et al. VACTERL association in anorectal malformation: Effect on the children born with anorectal malformation, treated by posterior sagittal
outcome. Pediatr Surg Int 2015;31:805‑8. anorectoplasty, assessed according to the Krickenbeck classification.
6. Belman AB, King LR. Urinary tract abnormalities associated with J Pediatr Surg 2009;44:399‑403.
imperforate anus. J Urol 1972;108:823‑4. 31. Bischoff A, Levitt MA, Peña A. Bowel management for the treatment of

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[Downloaded free from http://www.hamdanjournal.org on Wednesday, January 2, 2019, IP: 10.232.74.27]

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

pediatric fecal incontinence. Pediatr Surg Int 2009;25:1027‑42. 2017;52:1997‑2000.


32. Vilanova‑Sanchez A, Gasior AC, Toocheck N, Weaver L, Wood RJ, 34. Grano C, Bucci S, Aminoff D, Lucidi F, Violani C. Quality of life in
Reck CA, et al. Are senna based laxatives safe when used as long term children and adolescents with anorectal malformation. Pediatr Surg Int
treatment for constipation in children? J Pediatr Surg 2018;53:722‑7. 2013;29:925‑30.
33. Dingemans AJ, Reck CA, Vilanova‑Sanchez A, Gonzalez DO, 35. Clermidi P, Podevin G, Crétolle C, Sarnacki S, Hardouin JB.
Gasior AC, Weaver LJ, et al. Does clinic visit education within a The challenge of measuring quality of life in children with
multidisciplinary center improve health literacy scores in caregivers Hirschsprung’s disease or anorectal malformation. J Pediatr Surg
of children with complex colorectal conditions? J Pediatr Surg 2013;48:2118‑27.

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