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Inflammatory bowel disease manifesting after surgical treatment for Hirschsprung disease

David N Levin1, Margaret A Marcon, Risto J Rintala

  • 1Division of General and Thoracic Surgery, Hospital for Sick Children, Toronto, Canada.

Journal of Pediatric Gastroenterology and Nutrition
|February 24, 2012
PubMed

Insights

Children who underwent surgery for Hirschsprung disease may develop inflammatory bowel disease (IBD) years later. Long-term monitoring for IBD is crucial in these patients.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Genetics

Background:

  • Hirschsprung disease is a congenital condition affecting the large intestine.
  • Surgical correction is the standard treatment for Hirschsprung disease.
  • Long-term complications following surgery are not fully understood.

Observation:

  • A cohort of eight children developed chronic inflammatory bowel disease (IBD) between 4 and 21 years post-surgery for Hirschsprung disease.
  • Three patients had trisomy 21, and six experienced chronic or recurrent enterocolitis.
  • Four patients had a family history of IBD.

Findings:

  • Clinical manifestations included chronic diarrhea, hematochezia, abscess, and fistula formation.
  • Three patients required surgical intervention for complications like fistula, stricture, and obstruction.
  • Five patients were managed medically.

Implications:

  • Early recognition of IBD is vital in the long-term care of patients with Hirschsprung disease.
  • Patients with a history of chronic enterocolitis post-Hirschsprung surgery should be evaluated for IBD.
  • This finding highlights a potential link between Hirschsprung disease surgery and the later development of IBD.

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