Related Experiment Videos
Colonic strictures in children with cystic fibrosis
J M Zerin1, J Kuhn-Fulton, S J White
1Department of Radiology, Indiana University Medical Center, Riley Hospital for Children, Indianapolis 46202-2920.
Insights
Children with cystic fibrosis can develop irreversible colonic strictures. This condition involves progressive narrowing of the colon, characterized by fibrosis and loss of normal structure.
Area of Science:
- Pediatric Gastroenterology
- Gastrointestinal Pathology
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Colonic strictures are a potential complication in children with CF.
Purpose of the Study:
- To characterize the radiographic, clinical, surgical, and histologic features of colonic strictures in pediatric CF patients.
Main Methods:
- Retrospective review of medical records from ten pediatric CF patients with colonic strictures.
- Analysis of radiographic, surgical, and histologic findings.
Main Results:
- Radiographic findings included mucosal irregularity, wall thickening, loss of haustration, and long-segment luminal narrowing.
- Histology revealed severe submucosal fibrosis and fatty infiltration, with transmural fibrosis in some cases.
- Minimal acute inflammation was observed, distinguishing it from Crohn disease.
Conclusions:
- Colonic strictures in children with cystic fibrosis result from irreversible, often progressive, luminal narrowing.
- Fibrotic changes are the primary histopathologic feature.
Purpose:
To determine the radiographic, clinical, surgical, and histologic findings in children with cystic fibrosis who develop strictures of the colon.
Materials And Methods:
Ten children (five boys, five girls; age range, 2.5-9.0 years; mean age, 5.5 years), who were treated at the practices of the authors, were retrospectively identified and their medical records reviewed.
Results:
Radiographic manifestations of the colonic disease included mucosal irregularity and spiculation with nodular thickening of the colonic wall and loss of normal colonic haustration. Luminal narrowing involved long segments of the colon. Longitudinal shortening of the colon was also a prominent feature. The decrease in caliber of the bowel ranged from mild narrowing to complete occlusion of the lumen. Histologic examination revealed severe submucosal fibrosis and fatty infiltration with transmural extension of the fibrosis to involve the serosa in some cases. Unlike in Crohn disease, however, acute inflammatory changes were minimal or absent.
Conclusion:
Colonic stricture in children with cystic fibrosis is due to irreversible and frequently progressive narrowing of the colonic lumen.