What is the Risk of Inflammatory Bowel Disease in Patients with Hirschsprung’s Disease

Background: It is well-recognized that enterocolitis can occur in individuals with Hirschsprung’s disease, even after surgery. Case series have described cases of inflammatory bowel disease (IBD) in individuals with Hirschsprung’s which have some overlapping features with Hirschsprung disease-associated enterocolitis (HAEC). Those with IBD, however, have a constellation of clinical findings more typical of IBD and respond to treatments used in IBD.

A recent study from Canada (CN Bernstein et al. J Pediatr 2021; 233: 98-104. Increased Incidence of Inflammatory Bowel Disease After Hirschsprung Disease: A Population-based Cohort Study) provides more data regarding IBD in individuals with HD.

Key findings:

  • In the study from Ontario, 18 of 716 (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); thus, this represented a 12-fold increased risk.
  • In the study from Alberta and Manitoba, the OR of having had Hirschsprung disease before a diagnosis of IBD compared with controls was 74.9 (95% CI, 17.1-328.7) and 23.8 (95% CI, 4.6-123) respectively.
  • Crohn’s disease was more common after Hirschsprung disease than ulcerative colitis.

My take: I have personally seen IBD in HD and wondered how much increased risk that HD conferred. Now the question: what is the mechanism?

Related blog posts:

Alongside the Seven Mile Bridge in the Florida Keys:

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

This site uses Akismet to reduce spam. Learn how your comment data is processed.