Article1 min read·Published Jun 2026

Association of small intestinal atresia and Hirschsprung disease in a multi-institutional colorectal registry

pubmed.ncbi.nlm.nih.gov

Nelson G. Rosen

Purpose: Unlike colonic atresia, concomitant jejuno-ileal atresia (JIA) and Hirschsprung disease (HSCR) is an under-recognized association. We used a multi-institutional patient registry to describe the incidence, clinical characteristics, management, and outcomes of patients with both conditions. Methods: We performed a retrospective review of patients with HSCR in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. Additional data was collected from participating sites regarding atresia management and outcomes. Results: Of 1459 eligible patients with HSCR, 15 (1.0%) also had JIA. Median age at initial surgical consult and atresia diagnosis was 1 day (IQR 0.5-3.0 and 0.0-3.0, respectively). Median age at HSCR diagnosis was 58.5 days (IQR 16-147.5) compared to a median of 7 days (IQR 3.0-60.0) for patients with HSCR alone. Most patients had ileal atresia (N = 10, 66.7%), and total colonic HSCR (N = 9, 60.0%), although HSCR segment length was not consistently related to atresia location. After primary atresia surgery, 46.7% (N = 7) of patients had a complication within 30 days. At one year, 53.3% (N = 8) of patients required ongoing nutritional support, 55.6% (N = 5) had short bowel syndrome, 80.0% (N = 12) had at least one readmission, and 100% a reoperation. Twelve patients (80.0%) have undergone pull-through. Conclusion: Patients with both small intestinal atresia and HSCR demonstrated a higher likelihood of delayed HSCR diagnosis and an increased inci

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Article · Jun 2026 · 1 min read

In brief

Multi-institutional registry study identifies rare co-occurrence of jejuno-ileal atresia and Hirschsprung disease in 1% of HSCR patients, with delayed HSCR diagnosis (median 58.5 vs 7 days) and high morbidity including 55.6% developing short bowel syndrome and 100% requiring reoperation within one year.

Written by the GCMD Library team from the article.

Nelson G. Rosen

Statements in this article

Concomitant jejuno-ileal atresia and Hirschsprung disease occurs in 1.0% of patients with HSCR (15 of 1459 patients in the registry).
Research
Median age at initial surgical consult for patients with both JIA and HSCR was 1 day (IQR 0.5-3.0 days).
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Median age at atresia diagnosis in patients with both conditions was 1 day (IQR 0.0-3.0 days).
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Median age at HSCR diagnosis in patients with concomitant JIA was 58.5 days (IQR 16-147.5 days), compared to 7 days (IQR 3.0-60.0 days) for patients with HSCR alone.
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Ileal atresia was the most common atresia location, occurring in 66.7% of patients (10 of 15).
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Total colonic HSCR was present in 60.0% of patients (9 of 15) with concomitant JIA.
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HSCR segment length was not consistently related to atresia location.
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After primary atresia surgery, 46.7% of patients (7 of 15) had a complication within 30 days.
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At one year, 53.3% of patients (8 of 15) required ongoing nutritional support.
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At one year, 55.6% of patients (5 of 9) had short bowel syndrome.
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At one year, 80.0% of patients (12 of 15) had at least one readmission.
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At one year, 100% of patients required at least one reoperation.
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Twelve patients (80.0%) have undergone pull-through surgery for HSCR.
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