A Comparison of Patients Undergoing Redo Hirschsprung Surgery at a Colorectal Referral Center

Background: Patients undergo redo surgery for a variety of reasons for Hirschsprung disease (HD). We sought to characterize these reasons and compare the redo patient population to those who did not require reoperation.

Methods: A retrospective study of HD patients referred to our tertiary referral colorectal program from January 2010-December 2019 was conducted. Patients with total colonic HD were excluded from analysis. Fisher’s exact and Mann-Whitney U tests were used for statistical analyses.

Results: 192 patients were seen at our institution for HD. Median age of diagnosis was 9 days (IQR: 3, 125). 114 (62.2% of 183) patients had primary pull-through procedures without diversion. By type of pull-through, there were 80 Soave, 43 Swenson, 5 Duhamel, 3 myomectomy and 1 mucosectomy. Transition zone was identified for 61% (117/192) of patients: 2.1% rectum, 18.8% rectosigmoid, 25.5% sigmoid, 6.8% descending colon, 2.1% splenic flexure, 2.6% transverse colon, 1.6% hepatic flexure, 1.6% ascending colon. 41.1% of patients had ≥1 episode of enterocolitis. 51.6% (n=99) of patients were seen by our bowel management program: 49 patients were started on enemas, 29 on laxatives, 16 tried both. 35 (18.2%) of patients eventually had an antegrade flush operation. 68.8% patients had at least one biopsy and 59.4% had a contrast study after initial pull-through surgery. 31.7% (61) patients had redo surgeries. Reasons for redo included retained transition zone (35.0%), soave cuff (26.2%), stricture (22.9%), recurrent enterocolitis (21.3%), colonic dysfunction (9.8%), iatrogenic injury (8.2%), fistula (4.9%), kink (1.6%), and Duhamel spur (1.6%), with some patients having more than one reason for redo. Table 1 shows comparison between groups.

Conclusion: Patients requiring redo Hirschsprung surgery are more likely to have had recurrent enterocolitis, rectal biopsies, contrast studies and eventually antegrade flush operations performed. Intact dentate line and primary pull-through did not decrease the chance of requiring reoperation.