Fibrosing colonopathy: Difference between revisions
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==Management<ref>https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy</ref>== | ==Management<ref>https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy</ref>== | ||
*Anti-inflammatory therapy (e.g. [[prednisone]] pulses) | *Anti-inflammatory therapy (e.g. [[prednisone]] pulses) | ||
*Definitive treatment is balloon dilation or surgical resection of structured portion of colon | |||
==Disposition== | ==Disposition== | ||
Revision as of 15:50, 2 September 2019
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
- Abdominal pain/distension, vomiting, constipation, failure to thrive
- +/- bloody diarrhea
- May present similarly to distal bowel obstruction[2]
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[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
- ↑ 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