Diagnostic suggestion and surgical consideration for Hirschsprung’s disease associated with high anorectal malformation

Abstract

Object: The objective of this study were to highlight the finding of vasa recta (tortuous) on the colonic wall as a diagnostic clue for suspecting an associated Hirschsprung’s disease (HD) and to draw attention to the importance of preserving the aganglionic rectum plus a retrorectal pull through in these cases.

Background: The association of HD with anorectal malformation (ARM) is both diagnostically and surgically challenging.

Patients and methods: Records of cases with ARM treated over 15 years were examined. Among these, five children with an associated HD were analyzed with regard to their clinical, radiological, surgical, histopathological findings, and outcome. Relevant literature was also reviewed.

Results: HD was present in 1.26% cases of ARM. All were men with high-type ARM. Two groups were identified. In group 1 (two patients), associated HD was suspected after completion of all stages of ARM repair. They reported prolonged postoperative constipation, abdominal distention, and enterocolitis. Moreover, they endured additional surgeries for HD. In group 2 (three patients), HD was suspected at the time of initial colostomy for ARM in two patients on visualizing tortuous (cork screw) vasa recta on the sigmoid colon surface in a region similar to the transition zone in HD. Biopsy from the site confirmed HD. In the third patient, these vessels were visualized on the colostomy loop at the time of laparoscopic pull through for ARM. Preservation of the aganglionic rectum (to function as a fecal reservoir) as well as a retrorectal pull through was done in both groups.

Conclusion: Finding prominent corkscrew vessels on the colonic surface could serve as a clinical clue for the presence of HD in cases of ARM. Moreover, preserving the aganglionic rectum and performing a Duhamel pull through helps provide acceptable continence.

Keywords: anorectal malformation, Hirschsprung’s disease, rectal reservoir, tortuous vasa recta