Diffuse microcystic pancreatic enlargement in a cystic fibrosis patient causing severe gastrointestinal symptoms and successfully treated by total pancreatectomy

Dewar, Ann L. and Connett, Gary J. (1998) Diffuse microcystic pancreatic enlargement in a cystic fibrosis patient causing severe gastrointestinal symptoms and successfully treated by total pancreatectomy. Journal of Pediatric Gastroenterology and Nutrition, 26 (4), 454-457. (doi:10.1097/00005176-199804000-00017).

Record type: Article

Abstract

Excerpt
The typical morphologic changes within the pancreas of patients with cystic fibrosis are inter- and intralobular fibrosis and destruction of the pancreatic parenchyma. The small ducts become obstructed by secretions and cellular debris, leading to formation of microcysts. Eventually, the pancreas becomes shrunken and fibrosed, with fatty infiltration of the parenchyma(1-3). On rare occasions, it contains epithelia-lined macrocysts and calcification (4). The pancreatic changes in cystic fibrosis rarely cause abdominal pain except in small numbers of patients who suffer acute pancreatitis. In most of these cases, patients have sufficient pancreatic function(5,6). We report a child with pancreatic insufficiency with cystic fibrosis, in whom severe gastrointestinal symptoms developed, associated with a previously undescribed complication of the pancreas.

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