Fibrosing colonopathy: Difference between revisions
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==Background== | ==Background== | ||
[[File:Diameters of the large intestine.png|thumb|Average inner diameters and ranges of different sections of the large intestine.<ref> 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.</ref>]] | [[File:Diameters of the large intestine.png|thumb|Average inner diameters and ranges of different sections of the large intestine.<ref> 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.</ref>]] | ||
*Disease that occurs in patients with [[cystic fibrosis]] treated with enteric coated pancreatic enzymes, particularly with high doses<ref>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.</ref> | *Disease that occurs in patients with [[Special:MyLanguage/cystic fibrosis|cystic fibrosis]] treated with enteric coated pancreatic enzymes, particularly with high doses<ref>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.</ref> | ||
*More common in children, but reported in adults | *More common in children, but reported in adults | ||
*Characterized by progressive submucosal fibrosis, particularly of the proximal colon | *Characterized by progressive submucosal fibrosis, particularly of the proximal colon | ||
==Clinical Features== | ==Clinical Features== | ||
*[[Abdominal pain]]/distension, [[vomiting]], [[constipation]], [[failure to thrive]] | |||
*+/- bloody [[diarrhea]] | *[[Special:MyLanguage/Abdominal pain|Abdominal pain]]/distension, [[Special:MyLanguage/vomiting|vomiting]], [[Special:MyLanguage/constipation|constipation]], [[Special:MyLanguage/failure to thrive|failure to thrive]] | ||
*May present similarly to distal [[bowel obstruction]]<ref>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</ref> | *+/- bloody [[Special:MyLanguage/diarrhea|diarrhea]] | ||
*May present similarly to distal [[Special:MyLanguage/bowel obstruction|bowel obstruction]]<ref>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</ref> | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
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{{Pediatric abdominal pain DDX}} | {{Pediatric abdominal pain DDX}} | ||
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==Evaluation== | ==Evaluation== | ||
*Evaluate for alternative diagnoses | *Evaluate for alternative diagnoses | ||
*Imaging may show<ref>https://radiopaedia.org/articles/fibrosing-colonopathy-1?lang=us</ref>: | *Imaging may show<ref>https://radiopaedia.org/articles/fibrosing-colonopathy-1?lang=us</ref>: | ||
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**+/- affected colon shortened/retracted | **+/- affected colon shortened/retracted | ||
**thickening of bowel wall on US prior to stricture formation<ref>https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy</ref> | **thickening of bowel wall on US prior to stricture formation<ref>https://www.sciencedirect.com/topics/neuroscience/fibrosing-colonopathy</ref> | ||
==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. [[Special:MyLanguage/prednisone|prednisone]] pulses) | |||
*Definitive treatment is balloon dilation or surgical resection of structured portion of colon | *Definitive treatment is balloon dilation or surgical resection of structured portion of colon | ||
==Disposition== | ==Disposition== | ||
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==See Also== | ==See Also== | ||
*[[Cystic fibrosis]] | |||
*[[Special:MyLanguage/Cystic fibrosis|Cystic fibrosis]] | |||
==External Links== | ==External Links== | ||
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==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:GI]] [[Category:Pediatrics]] | [[Category:GI]] [[Category:Pediatrics]] | ||
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Latest revision as of 22:54, 4 January 2026
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