Anorectal Malformations


imperforate anus
Descriptive text is not available for this image
An imperforate anus in a male neonate.

What is an anorectal malformation?
An anorectal malformation (ARM) is commonly referred to as imperforate anus. A normal anal opening is nonexistent and the colon empties anteriorly either onto the perineum or towards the vagina in a female or into the urinary system in a male. There are also instances of atresia where the rectum is blind ending with no connection or external opening. The distance away from the normal anal opening correlates with the degree of associated abnormalities.

How has the repair of anorectal malformations evolved?

Repair of a child born with an imperforate anus has evolved from attempts to simply perforate the anal opening to diversion in a variety of ways, either through the perineum, abdominal wall or sacrum. Attempts at perineal repairs were described before the 1900s and evolved into an abdominoperineal resection [1]. The early 1980s marked a significant turning point for operative repair when Drs. de Vries and Peña introduced the posterior sagittal approach which is the predominant repair done today for fistulas reachable from the perineum [2]. Subsequent modifications to this technique included the incorporation of laparoscopy for a more minimally invasive approach [3][4]. Recently, magnetic resonance imaging and advances in laparoscopic techniques have been utilized in an attempt to place the distal colon directly in the middle of the sphincter complex with minimal dissection of the perineum [5].

What are the greatest challenges for practicing pediatric surgeons?

In the United States, the long-term management of these patients’ constipation and incontinence is challenging. They commonly present with soiling, obstipation and in need of an aggressive bowel regimen. It is clear that collaboration with nursing is key. In addition, strong support from gastroenterology, urology and gynecology is beneficial in the collaborative management of these patients.

For surgeons practicing abroad, challenges with delayed presentation, inadequate facilities, and limited clinical resources are a major concern. Long-term patient follow-up and management can be challenging given the distances patients and their families have to travel for care [6][7]. There is often a gap between the initial reconstruction and the actual use of that anatomy for continence. Therefore, any technical matters from the initial surgery that can affect long-term potential function (stricture, prolapse, and mislocation of the anus) manifest later and it is hard for the surgeon to adapt their technique for future cases. This may explain the high incidence of complications and required reoperations [8].

Content in this module is referenced in SCOREAnorectal Malformation overview

video link The story of the PSARP with Dr. Alberto Pena

There's more to see -- the rest of this topic is available only to subscribers.

Last updated: June 18, 2021