Managing adults with previously treated anorectal malformations

Abstract

Background: Although rare, it is not unheard of to encounter patients whose anorectal malformations have been ignored until adulthood in resource-limited environments. However, it is extremely uncommon to be faced with patients whose early primary management was directed toward a permanent stoma. In cases where the proximal rectum is all but sacrificed, the management for definitive repair and reconstitution of intestinal continuity is a challenge. Little information exists in the literature. Methods: The management of two adult female patients born with rectovestibular fistulas in a resource-limited environment is reviewed. Their management was complicated by inadequate repair, followed by resection of the distal intestine, and creation of a permanent end stoma. Both patients were seeking definitive repair of the inadequately treated anorectal malformation and reconstitution of intestinal integrity. The existing literature is reviewed. Results: Both patients are doing well three years after the final surgical interventions. Neither patient has fecal incontinence. One initially had issues with constipation, but has successfully overcome the problem using dietary measures. Neither has issues with the genitourinary system. Review of the literature revealed numerous articles describing the delayed presentation of patients with anorectal malformations, but none describing this specific group of patients in whom the initial management specifically targeted a permanent stoma. Conclusion: Adults with inadequately repaired rectovestibular fistulas whose initial surgical management specifically targets resection of the proximal rectum and creation of a permanent end colostomy are rare. Their management is a challenge, especially in resource-limited environments. When the anorectal malformation is of a favourable type, appropriate investigation coupled with meticulous surgical planning of the definitive repair may yield excellent outcomes.