Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group
Topic overview
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.
Key takeaways
- 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%).
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