Fibrosing colonopathy
Background
Average inner diameters and ranges of different sections of the large intestine.[1]
- Disease that occurs in patients with cystic fibrosis treated with enteric coated pancreatic enzymes, particularly with high doses[2]
- More common in children, but reported in adults
- Characterized by progressive submucosal fibrosis, particularly of the proximal colon
Clinical Features
- Abdominal pain/distension, vomiting, constipation, failure to thrive
- +/- bloody diarrhea
- May present similarly to distal bowel obstruction[3]
Differential Diagnosis
Pediatric Abdominal Pain
0–3 Months Old
- Emergent
- Nonemergent
3 mo–3 y old
- Emergent
- Nonemergent
3 y old–adolescence
- Emergent
- Nonemergent
Evaluation
- Evaluate for alternative diagnoses
- Imaging may show[4]:
- long or short segment colon stricture, typically right-sided
- +/- affected colon shortened/retracted
- thickening of bowel wall on US prior to stricture formation[5]
Management[6]
- 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
- ↑ Nguyen H, Loustaunau C, Facista A, Ramsey L, Hassounah N, Taylor H, et al. (July 2010). "Deficient Pms2, ERCC1, Ku86, CcOI in field defects during progression to colon cancer". Journal of Visualized Experiments (41). doi:10.3791/1931. PMC 3149991. PMID 20689513.
- ↑ 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.
- ↑ 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
- ↑ https://radiopaedia.org/articles/fibrosing-colonopathy-1?lang=us
- ↑ https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy
- ↑ https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy