Fibrosing colonopathy

Background

  • Disease that occurs in patients with cystic fibrosis treated with enteric coated pancreatic enzymes, particularly with high doses[1]
  • More common in children, but reported in adults
  • Characterized by progressive submucosal fibrosis, particularly of the proximal colon

Clinical Features

Differential Diagnosis

Pediatric Abdominal Pain

0–3 Months Old

3 mo–3 y old

3 y old–adolescence


Evaluation

  • Evaluate for alternative diagnoses
  • Imaging may show[3]:
    • long or short segment colon stricture, typically right-sided
    • +/- affected colon shortened/retracted
    • thickening of bowel wall on US prior to stricture formation[4]

Management[5]

  • Anti-inflammatory therapy (e.g. prednisone pulses)
  • Definitive treatment is balloon dilation or surgical resection of structured portion of colon

Disposition

See Also

External Links

References

  1. Fitzsimmons SC, Burkhart GA, Borowitz D, et al. High-dose pancreatic-enzyme supplements and fibrosing colonopathy in children with cystic fibrosis. N Engl J Med. 1997;336(18):1283-9.
  2. Rosenstein BJ, Langbaum TS (May 1983). "Incidence of distal intestinal obstruction syndrome in cystic fibrosis". Journal of Pediatric Gastroenterology and Nutrition. 2 (2): 299–301
  3. https://radiopaedia.org/articles/fibrosing-colonopathy-1?lang=us
  4. https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy
  5. https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy

Authors:

Claire