Enterocolitis and Bowel Function in Children with Hirschsprung Disease and Trisomy 21

Abstract Background/Purpose. The purpose of this study was to study the effect of Trisomy 21 (T21) on enterocolitis rates and bowel function among children with Hirschsprung Disease (HD). Methods A retrospective cohort study of patients with HD treated at our tertiary children's hospital (2000–2015) and a cohort of patients with HD treated in our pediatric colorectal center (CRC) (2011–2015) was performed. Results 26/207 (13%) patients with HD had T21. 70 (41%) with HD alone were diagnosed with enterocolitis episodes compared to 9 (38%) with HD + T21 (p = 0.71). 55/207 patients were managed in the CRC. 11/55 patients (20%) had HD + T21. 25 (58%) with HD had one or more enterocolitis episodes compared to 4 (36%) with HD + T21 (p = 0.20). Number of hospitalizations for enterocolitis was similar between all groups. Toilet training was assessed in 32 CRC patients (25 HD, 7 HD + T21). One child with HD + T21 was toilet trained by age 4 years versus 12 with HD (p = 0.20). Laxative or enema therapy was required for constipation management in 57% HD versus 64% HD + T21. Conclusion Enterocolitis rates in children with HD + T21 did not differ from rates in children with HD alone. The majority of patients with CRC follow-up had constipation requiring laxative or enema therapy, which demonstrates the need for consistent post-operative follow-up). Retrospective Study – Level II.