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.

Creator(s)

• Hira Ahmad
• Alyssa Joachim
• Andreina Y. Urrutia Gonzalez
• Donald Shaul
• Kelly Austin
• Andrea Badillo
• Casey M. Calkins
• Rachel C. Crady
• Megan M. Durham
• Jason Frischer
• Christopher Gayer
• Julia E. Grabowski
• Jamie C. Harris
• Charles R. Hong
• Akemi L. Kawaguchi
• Jacob C. Langer
• Prathima Nandivada
• Ankur Rana
• Ron W. Reeder
• Rebecca M. Rentea, Children's Mercy HospitalFollow
• Michael D. Rollins
• Nelson Rosen
• Payam Saadai
• Caitlin A. Smith
• K Elizabeth Speck
• Richard J. Wood

Document Type

Article

Publication Date

8-2026

Identifier

DOI: 10.1016/j.jpedsurg.2026.163232

Abstract

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.

Journal Title

Journal of pediatric surgery

Volume

61

Issue

8

First Page

163232

Last Page

163232

MeSH Keywords

Child; Child, Preschool; Female; Humans; Infant; Male; Colorectal Surgical Procedures; Cross-Sectional Studies; Hirschsprung Disease; Postoperative Complications; Retrospective Studies; Toilet Training; Treatment Outcome; United States

PubMed ID

42251888

Keywords

Aganglionosis; Botulinum toxin; Constipation; Fecal incontinence; Fiber; Hirschsprung disease

Recommended Citation

Ahmad H, Joachim A, Urrutia Gonzalez AY, et al. 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. J Pediatr Surg. 2026;61(8):163232. doi:10.1016/j.jpedsurg.2026.163232