Persistent obstructive symptoms after surgery for Hirschsprung's disease: development of a diagnostic and therapeutic algorithm

Abstract

Background: Although most children with Hirschsprung's disease (HD) do well after pull-through surgery, some continue to have persistent obstructive symptoms that may lead to significant morbidity. The author's goal was to develop an organized algorithm for the workup and ultimate management of these symptoms.

Methods: All children referred to the author with severe obstructive symptoms after a pull-through for HD were evaluated using an algorithm based on 5 potential etiologies, and appropriate therapeutic interventions were instituted.

Results: A total of 49 children were evaluated over 7 years. Mechanical obstruction was found using rectal examination and barium enema in 7, of which, 2 had resection of a Duhamel spur, and 5 had a repeat pull-through (after failed dilatation). Rectal biopsy results showed aganglionosis in 10, of whom, 8 underwent repeat pull-through, and 2 refused further surgery. Abnormal intestinal motility or intestinal neuronal dysplasia was found proximal to the aganglionic segment in 10 children using colonic and small bowel manometry or laparoscopic biopsies; 4 of these had additional colon resected and repeat pull-through, and 6 were treated with a bowel management routine, cecostomy, or stoma formation. Internal sphincter achalasia was felt to be the primary cause of symptoms in 14, and all were treated with intrasphincteric botulinum toxin. Eight were found not to fall into any of the above groups and were treated medically for presumed functional megacolon; 2 of these ultimately had a colostomy.

Conclusions: An organized approach to the diagnosis and management of obstructive symptoms in a child after a pull-through for HD permits accurate diagnosis and effective treatment in the majority of cases.