Hirschsprung-associated inflammatory bowel disease (HD-IBD): A multicenter study | Retrospective study | Between 2000 - 2021 | 17 institutions | Patients diagnosed with IBD after pullthrough surgery | 55 patients | 50% long segment disease (n=28) | 68% Hirschsprung-associated enterocolitis (HAEC) (n=36) | 18% had Trisomy 21 (n=10) | Of all patients | 63% (n=34) IBD was diagnosed > age 5 | 69% (n = 38) IBD presentation: colonic or small bowel inflammation resembling IBD | 18% (n = 10) Unexplained or persistent fistula | 13% (n = 7) Unexplained HAEC > age 5 or unresponsive to standard treatment | 1/3 of patients required a surgical procedure for IBD | Conclusion: Long segment disease, HAEC after pull through surgery & trisomy 21 may represent risk factors for HD-IBD. Biological agents are considered the most effective medical treatment. | https://doi.org/10.1016/j.jpedsurg.2023.01.018 | @EmTombash | @StayCurrentMD | Source: Sutthatarn P et al. | Division of General and Thoracic Surgery, The Hospital for Sick Children, Toronto, ON, Canada | Cincinnati Children's | Journal of Pediatric Surgery
Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group
Infographic · Jul 2023 · 1 min read
In brief
In brief
Multicenter retrospective study (2000-2021) identified risk factors for post-pullthrough IBD in Hirschsprung patients: 50% had long-segment disease, 68% developed HAEC post-operatively, and 18% had Trisomy 21. Biological agents showed 80% effectiveness, though one-third required surgical intervention for IBD management.
- 63% of HD-IBD cases present after age 5; consider IBD workup in older children with persistent HAEC or unexplained fistulae post-pullthrough
- Long-segment HD, prior HAEC, and Trisomy 21 are potential risk factors for developing inflammatory bowel disease after Hirschsprung surgery
- Biological agents showed 80% effectiveness for HD-IBD treatment, significantly outperforming other medical therapies in this cohort
- 18% of HD-IBD patients presented with unexplained persistent fistulae; one-third ultimately required surgical intervention for IBD management
Written by the GCMD Library team from the infographic.
The infographic uses a teal, pink, and yellow color scheme with a hospital icon on the left. Key statistics are displayed in large colored percentages with accompanying text. A stylized intestine illustration appears in the bottom right. The layout flows from study design at top, through patient demographics in the middle, to conclusions at bottom.
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.