Association of small intestinal atresia and Hirschsprung disease in a multi-institutional colorectal registry.
Document Type
Article
Publication Date
8-2026
Identifier
DOI: 10.1016/j.jpedsurg.2026.163228
Abstract
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 incidence of total colonic HSCR. A high index of suspicion is critical for timely diagnosis and management of this under-recognized population.
Journal Title
Journal of pediatric surgery
Volume
61
Issue
8
First Page
163228
Last Page
163228
MeSH Keywords
Female; Humans; Infant; Infant, Newborn; Male; Abnormalities, Multiple; Hirschsprung Disease; Ileum; Incidence; Intestinal Atresia; Intestine, Small; Jejunum; Postoperative Complications; Registries; Retrospective Studies; Treatment Outcome
PubMed ID
42251886
Keywords
Hirschsprung disease; Jejunoileal atresia; Small intestinal atresia
Recommended Citation
Read Ivaturi M, Crady RC, Hussaini SF, et al. Association of small intestinal atresia and Hirschsprung disease in a multi-institutional colorectal registry. J Pediatr Surg. 2026;61(8):163228. doi:10.1016/j.jpedsurg.2026.163228
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