O2195: Early outcomes of the Ileoanal Pullthrough for Total Colonic Hirschsprung Disease: A Single Center Experience

Background: Total Colonic Hirschsprung Disease (TCHD) is rare and there is no consensus on its optimal surgical approach. Few studies report the outcomes for ileoanal Swenson-like pullthrough (IAPT) for TCHD. We aimed to improve counseling of families specifically for post operative expectations regarding stooling and complications.

Methods: A single center retrospective review was conducted of all patients who underwent an IAPT between 1/2020-12/2023. We evaluated 30-day complications, unplanned ED visits, readmissions, stooling patterns, and the need for medications/interventions post-operatively. An IAPT was performed only if the child was clinically well, not requiring supplemental enteral or parenteral nutrition, and was able to produce thickened stools.

Results: 25 patients (64% male) underwent an IAPT at a median age of 1.6 years (range 0.6 – 15.6) with a median follow up of 16 months (range 1-39). 1 patient had an ED visit and 4 were readmitted within 30 days, for fever (1), urinary tract infection (1), ileus (1), and Hirschsprung-associated enterocolitis (HAEC) (1). 3 additional patients were admitted for HAEC within one year. Overall, 16 patients (64%) underwent anal botulinum toxin injection, 4 (16%) were treated for SIBO, and 3 (12%) for proctalgia fugax.

At one month, 12 patients (48%) had at least 14 stools/day, 8 (32%) had 8-14 stools/day, and 5 (20%) had 1-7 stools/day (Figure 1). 14 patients (56%) were using loperamide, 18 (72%) were on fiber supplementation, and all were on a constipating diet. 12 patients (48%) had a perianal rash, and the rest reported no rash

14 (56%) patients had at least one year of follow up, with a median follow-up of 2.1 years (range 1.3-3.32). For this group of patients, 2 (14%) had at least 14 stools/day, 3 (21%) had 8-14 stools/day, and 8 (57%) had 1-7 stools/day. (Figure 1). 7 (50%) were using loperamide, 9 (64%) were taking fiber, and all were on a constipating diet. No patients had active perianal rashes, 6 (43%) had healing rashes, and 8 (57%) had no rash. 1 patient underwent an ileostomy creation due to a high stooling frequency which affected their quality of life.

Conclusion: Patients with TCHD who undergo an IAPT have low perioperative complication rates. A majority have a manageable number of bowel movements at least one year after their procedure, with minimal or no perianal rash. 16% of patients experience HAEC and may require botulinum toxin treatments for non-relaxing sphincters. These outcomes can help to manage expectations for family members and guide surgical decision-making.