Current Concepts in The Management of Anorectal Ma
Current Concepts in The Management of Anorectal Ma
27]
Review Article
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.
Figure 1: Correct way to examine an anorectal malformation in females Figure 2: Positioning of the child for the correction of an anorectal
malformation
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