Outcomes Following Fecal Diversion for Intractable Hirschsprung Associated Enterocolitis: A Study From the Pediatric Colorectal and Pelvic Learning Consortium.

Abstract

Background: Hirschsprung associated enterocolitis (HAEC) is a challenging problem in a subset of children with Hirschsprung disease (HD). In refractory cases, fecal diversion may be required. The aim of this study was to characterize patients who require fecal diversion for HAEC management and examine their long-term outcomes.

Methods: A retrospective review of prospectively collected data within the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry was performed. All children with a history of a corrective procedure for HD and documented post-operative HAEC were included. Our primary outcome was diversion to manage HAEC after pull-through and secondary outcomes included incidence of recurrent HAEC, fecal continence following ostomy closure, and bowel management needs at last follow-up.

Results: 951 patients were identified with a history of HD and 852 had undergone pull-through. 339/852 (39.8 %) had documented HAEC and 75/339 (22.1 %) required an ostomy, 54 (72 %) of which underwent reversal at a median of 239.5 days. After reversal, 10/54 (18.5 %) required repeat diversion for recurrent HAEC. 30/75 (40 %) had their pull-through revised. Median age at last follow-up was 5.3 [3.4, 9] years. At time of last follow-up, 40.3 % were toilet-trained for stool and 42.9 % required long term enema-based therapy.

Conclusion: Fecal diversion for refractory HAEC was common in our study. Slightly more than half of the patients underwent successful closure of their stoma during over 4.5 years of follow-up, while less than half were toilet-trained for stool at their last visit. These children may represent a unique cohort that warrants further investigation.

Level of evidence: Level VI.