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Acquired Segmental Megacolon

Acquired Segmental Megacolon
Cystic fibrosis (CF) is characterized by symptoms related to pulmonary dysfunction and pancreatic insufficiency. Constipation, though a frequent complaint of patients with CF, receives less attention. We report a case of acquired segmental megacolon and constipation necessitating surgical colonic resection in an adult patient with CF. The differential diagnosis and possible causes of megacolon in this setting are discussed.

Constipation can be a common problem in patients with CF. It affects one third of all CF patients and becomes much more common after age 30. The prevalence of this complaint has been reported to be 14% of patients less than 5 years old and 73% of those older than 30. Causes of constipation in patients with CF include meconium plug syndrome, Hirschsprung's disease, and fibrosing colonopathy with strictures related to the use of high-dose pancreatic enzyme supplements. Similar causes of constipation have been reported in adults with CF, though adults may also have the more common causes of constipation including functional constipation and diabetic autonomic neuropathy. Some of these CF patients may have acquired colonic megacolon related to their chronic constipation as well. We present a case of a massively dilated acquired segmental megacolon associated with rectosigmoid colonic fibrosis in a young adult with CF.

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