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.

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.