Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group

Part of Pediatric Colorectal and Pelvic Reconstruction 15 items

Infographic · Jul 2023 · 1 min read

In brief

Multicenter retrospective study of 55 patients who developed IBD-like symptoms after Hirschsprung pullthrough surgery. Long-segment disease, post-operative HAEC, and Trisomy 21 emerged as potential risk factors, with most diagnoses occurring after age 5. Biological agents showed 80% effectiveness as medical treatment.

HD-IBD typically presents after age 5; consider IBD workup in older children with unexplained fistulae or refractory HAEC.
Long-segment disease, post-pullthrough HAEC, and Trisomy 21 are potential risk factors for developing HD-IBD.
Biological agents showed 80% effectiveness and were the most successful medical treatment for HD-IBD.
One-third of HD-IBD patients required surgical intervention despite medical management.
HD-IBD can manifest as colonic/small bowel inflammation (69%), unexplained fistula (18%), or atypical HAEC (13%).

Written by the GCMD Library team from the infographic.

A three-column comparison layout with illustrated diagrams of surgical procedures. Each column contains a procedural illustration at the top, followed by statistical data presented in colored boxes below. The design uses a clean, clinical color palette with icons and percentage indicators to highlight key outcomes.

Pattamon Sutthatarn, Eveline Lapidus-Krol, Caitlin Smith, Ihab Halaweish, Kristy Rialon, Matthew W. Ralls, Rebecca M. Rentea, Mary B. Madonna, Candace Haddock, Ana M. Rocca, Ankush Gosain, Jason Frischer, Hannah Piper, Allan M. Goldstein, Payam Saadai, Megan M. Durham, Belinda Dickie, Mubeen Jafri, Jacob C. Langer

Background/Purpose

A small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.

Methods

Retrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale.

Results

There were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD.

Conclusion

More than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment.

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