J Pediatr. 2021 Jun;233:98-104.e2. doi: 10.1016/j.jpeds.2021.01.060. Epub 2021 Feb 2.

Charles N Bernstein ¹, M Ellen Kuenzig ², Stephanie Coward ³, Zoann Nugent ⁴, Ahmed Nasr ⁵, Wael El-Matary ⁶, Harminder Singh ⁴, Gilaad G Kaplan ³, Eric I Benchimol ⁷

Author information

• ¹University of Manitoba IBD Clinical and Research Center, Winnipeg, Manitoba, Canada; Department of Internal Medicine, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada. Electronic address: Charles.bernstein@umanitoba.ca.
• ²Children's Hospital of Eastern Ontario (CHEO) Inflammatory Bowel Disease Center, Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ontario, Canada; CHEO Research Institute, Ottawa, Ontario, Canada; ICES uOttawa, Ottawa, Ontario, Canada.
• ³Department of Medicine and Community Health Sciences, University of Calgary, Calgary, Alberta, Canada.
• ⁴University of Manitoba IBD Clinical and Research Center, Winnipeg, Manitoba, Canada; Department of Internal Medicine, Max Rady College of Medicine, Rady Faculty of Health Sciences, University of Manitoba, Winnipeg, Manitoba, Canada; CancerCare Manitoba, Winnipeg, Manitoba, Canada.
• ⁵Department of Pediatric Surgery, Children's Hospital of Eastern Ontario and Department of Surgery, University of Ottawa, Ottawa, Ontario, Canada.
• ⁶Department of Pediatrics and Child Health, University of Manitoba, Winnipeg, Manitoba, Canada; Manitoba and Children's Hospital Research Institute of Manitoba, Winnipeg, Manitoba, Canada.
• ⁷Children's Hospital of Eastern Ontario (CHEO) Inflammatory Bowel Disease Center, Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ontario, Canada; CHEO Research Institute, Ottawa, Ontario, Canada; ICES uOttawa, Ottawa, Ontario, Canada; Department of Pediatrics and School of Epidemiology and Public Health, University of Ottawa, Ottawa, Ontario, Canada.

Abstract

Objective: To determine the frequency with which inflammatory bowel disease (IBD) is diagnosed in persons with Hirschsprung disease in population-based datasets from 3 Canadian provinces.

Study design: In study I, Ontario data were used to assess the incidence of IBD in a birth cohort of children with Hirschsprung disease relative to children without Hirschsprung disease. In study II, a case-control design was used in Alberta and Manitoba to determine the frequency of previously diagnosed Hirschsprung disease in persons with IBD, compared with the frequency of Hirschsprung disease in matched controls. Validated algorithms for Hirschsprung disease and IBD were applied to each provincial health registry.

Results: In study I, of the 716 children diagnosed with Hirschsprung disease in Ontario since 1991, 18 (2.5%) ultimately developed IBD (168.8 per 100 000 person-years), compared with 7109 of 3 377 394 children without Hirschsprung disease (0.2%, 14.2 per 100 000 person-years). The percentage of males with post-Hirschsprung disease IBD was 77.8%. The incidence rate ratio was 11.9 (95% CI, 7.5-18.8). In study II, the OR of having had Hirschsprung disease before a diagnosis of IBD compared with controls was 74.9 (95% CI, 17.1-328.7) in Alberta and 23.8 (95% CI, 4.6-123) in Manitoba. Crohn's disease was more common after Hirschsprung disease than ulcerative colitis.

Conclusions: IBD can emerge in more than 2% of patients with Hirschsprung disease and, like Hirschsprung disease itself, is more common in males. IBD is much more common after a diagnosis of Hirschsprung disease than in the general population.