Journal of pediatric surgery · 2026
Association of small intestinal atresia and Hirschsprung disease in a multi-institutional colorectal registry.
СтатьяМногоцентровое исследованиеНаблюдательное исследованиеНазвание и аннотация: Перевод в очереди
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Read Ivaturi Megan, Crady Rachel C, Hussaini Syed F, Ahmad Hira, Austin Kelly, Badillo Andrea, Calisto Juan L, Calkins Casey M, Darcy David, Durham Megan M, Fuchs Julie R, Gayer Christopher, Grabowski Julia E, Harris Jamie C, Hong Charles R, Kawaguchi Akemi L, Rana Ankur, Reeder Ron W, Rentea Rebecca M, Rollins Michael D, Rosen Nelson, Saadai Payam, Smith Caitlin A, Speck Elizabeth K, Velazco Cristine S, Halaweish Ihab, Gasior Alessandra C, Criss Cory, Wood Richard J, Pruitt Liese C C
Аннотация
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.
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.