Fibrosing colonopathy is a recently described (1993) condition with colonic fibrosis seen in patients with cystic fibrosis. This appears to be related to the use of certain high strength pancreatin preparations. Pancreatin is an enzyme mixture (amylase, protease, lipase and others) derived from porcine pancreas that enables more extensive digestion of food.


Findings in patients with fibrosing colonopathy:

(1) failure to thrive

(2) persistent malabsorption despite normal enzyme supplementation

(3) persistent abdominal pain with obstruction or distension

(4) bloody diarrhea

(5) chylous ascites

(6) thickening of the bowel wall with a normal external diameter but luminal narrowing

(7) reduced peristalsis

(8) foreshortening of the colon, with loss of haustrations ("lead pipe" colon)

(9) dilatation of the terminal ileum

(10) prominent band of fibrous tissue in the lamina propria and submucosa, with occasional chronic inflammatory cells

(11) history of taking high strength pancreatin preparation for several months (usually 12-15) before onset


Differential diagnosis:

(1) intestinal obstruction associated with cystic fibrosis, causing the distal intestinal obstructive syndrome (DIOS): discomfort and distension, constipation, weight loss, and failure to thrive

(2) stricture following surgery


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