Journal of pediatric surgery · 2026
Beyond the pull-through: A multi-institutional study of long-term surgical and functional outcomes in total colonic Hirschsprung disease - A Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) study.
СтатьяМногоцентровое исследованиеНазвание и аннотация: Перевод в очереди
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Ahmad Hira, Joachim Alyssa, Urrutia Gonzalez Andreina Y, Shaul Donald, Austin Kelly, Badillo Andrea, Calkins Casey M, Crady Rachel C, Durham Megan M, Frischer Jason, Gayer Christopher, Grabowski Julia E, Harris Jamie C, Hong Charles R, Kawaguchi Akemi L, Langer Jacob C, Nandivada Prathima, Rana Ankur, Reeder Ron W, Rentea Rebecca M, Rollins Michael D, Rosen Nelson, Saadai Payam, Smith Caitlin A, Speck K Elizabeth, Wood Richard J
Аннотация
PURPOSE: Total colonic Hirschsprung disease (TCHD), a severe Hirschsprung disease variant (∼5-10% of cases), is defined by aganglionosis of the entire colon. This study evaluates short-term surgical outcomes and long-term functional results in children with TCHD.
METHODS: A cross-sectional retrospective cohort study was conducted on patients with TCHD with at least 4 years of follow-up enrolled at the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) institutions from 2017 to July 2025. Demographics, surgical details, and functional outcomes-including bowel management, toilet training, and stooling accidents-were collected.
RESULTS: Of 75 patients meeting inclusion criteria, 63 underwent a pull-through procedure, with 32 occurring at a PCPLC site; demographics and associated diagnoses did not influence whether patients received pull-through. Among PCPLC patients, the most common procedures were Duhamel (n = 12, 37.5%), Yancey-Soave (n = 8, 25.0%), and Swenson (n = 8, 25.0%), with 59.4% performed laparoscopic-assisted. Four patients required redo pull-through (12.5%), 22 patients (68.8%) had 1 or more additional operations, and 1 (3.1%) had an anastomotic leak. Among non-diverted patients, rates of toilet training ranged from 47.2% at ages 4-7 to 92.9% at 12+ years, although up to 38.5-55.0% of patients continued to have stooling accidents. Common bowel management plans included botulinum toxin injection, fiber, and stool diversion.
CONCLUSIONS: Pull-through procedures of patients with TCHD yielded generally favorable early and mid-term outcomes, with most patients achieving toilet training by 8-11 years. However, a subset required re-diversion or redo pull-through, and many continued to experience stooling accidents, highlighting ongoing functional challenges despite specialized colorectal care.
METHODS: A cross-sectional retrospective cohort study was conducted on patients with TCHD with at least 4 years of follow-up enrolled at the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) institutions from 2017 to July 2025. Demographics, surgical details, and functional outcomes-including bowel management, toilet training, and stooling accidents-were collected.
RESULTS: Of 75 patients meeting inclusion criteria, 63 underwent a pull-through procedure, with 32 occurring at a PCPLC site; demographics and associated diagnoses did not influence whether patients received pull-through. Among PCPLC patients, the most common procedures were Duhamel (n = 12, 37.5%), Yancey-Soave (n = 8, 25.0%), and Swenson (n = 8, 25.0%), with 59.4% performed laparoscopic-assisted. Four patients required redo pull-through (12.5%), 22 patients (68.8%) had 1 or more additional operations, and 1 (3.1%) had an anastomotic leak. Among non-diverted patients, rates of toilet training ranged from 47.2% at ages 4-7 to 92.9% at 12+ years, although up to 38.5-55.0% of patients continued to have stooling accidents. Common bowel management plans included botulinum toxin injection, fiber, and stool diversion.
CONCLUSIONS: Pull-through procedures of patients with TCHD yielded generally favorable early and mid-term outcomes, with most patients achieving toilet training by 8-11 years. However, a subset required re-diversion or redo pull-through, and many continued to experience stooling accidents, highlighting ongoing functional challenges despite specialized colorectal care.