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Colonic stricture in a boy with cystic fibrosis
1Department of Histopathology, Charing Cross and Westminster Medical School, London, UK.
Insights
Cystic fibrosis patients can develop intestinal obstruction from fibrotic strictures. A child treated with right hemicolectomy recovered, suggesting a link to enteric-coated pancreatin microspheres.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Cystic Fibrosis Research
Background:
- Cystic fibrosis (CF) is a genetic disorder affecting multiple organs, including the gastrointestinal tract.
- Gastrointestinal complications are common in CF patients, necessitating ongoing research into their causes and treatments.
- Pancreatic enzyme replacement therapy is crucial for managing maldigestion in CF.
Observation:
- A pediatric case of subacute intestinal obstruction was identified.
- The obstruction was caused by a fibrotic stricture in the ascending colon.
- This presentation is a newly recognized pathology in cystic fibrosis patients.
Findings:
- Surgical intervention with right hemicolectomy successfully resolved the obstruction.
- No recurrence of the obstruction was observed during a nine-month follow-up period.
- Emerging evidence links this fibrotic stricture to the use of enteric-coated high-strength pancreatin microspheres.
Implications:
- This finding highlights a potential new gastrointestinal complication associated with specific CF therapies.
- Further investigation is warranted to confirm the link between enteric-coated pancreatin microspheres and fibrotic strictures.
- Clinical vigilance for intestinal obstruction in CF patients using these microspheres is recommended.
Abstract:
Many problems may arise within the gastrointestinal tract of patients with cystic fibrosis. We report a new cause of subacute intestinal obstruction due to a fibrotic stricture of the ascending colon in a child with cystic fibrosis. Treatment was with a right hemicolectomy. There has been no recurrence after nine months follow-up. Recent similar cases suggest that this new pathology is linked to the use of enteric-coated high-strength pancreatin microspheres.