Characterizing the use of botulinum toxin in patients with Hirschsprung disease treated at referral institutions for pediatric colorectal surgery

Purpose: Botulinum toxin (BT) is used to treat pediatric patients with Hirschsprung disease (HD) with obstructive symptoms. We aimed to characterize use of BT in HD patients across pediatric colorectal surgery referral centers.

Methods: A multicenter retrospective study of BT use in children (0-18y) with HD was performed using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) between 2017 and 2021. Sites with <10 HD patients recorded were excluded. Patterns were evaluated using Fisher's exact, Wilcoxon rank-sum, Kruskal-Wallis, and Cochran-Armitage trend test.

Results: 494 patients at 8 centers were included. 118 (23.9%) received at least one BT injection. Among patients who required redo pullthrough procedures, 53.1% received BT compared to 22.7% of patients who only underwent one pullthrough (p<0.001). Age at pullthrough was also significantly associated (p = 0.021). A lower proportion of Hispanic patients received BT (9.6% vs. 26.3%;p = 0.006). Percentage of HD patients receiving BT varied significantly across sites (p<0.001).

Conclusions: Use of BT in patients with HD varies widely with greater use in patients who underwent redo surgery and in those who underwent pullthrough at an older age. Hispanic patients received less BT. These findings highlight the need to develop consensus guidelines and for further study on timing of injections and potential disparities in care.

Level of evidence: III.