Portal hypertension in cystic fibrosis

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Early diagnosis and prompt therapy aimed at minimizing and combating pulmonary and gastrointestinal complications have resulted in prolonged survival of children with cystic fibrosis. 1 The widespread exocrine glandular dysfunction seen in the disease has been found to involve the mucous secretions of the bilary tract in many. As a result of recurrent plugging of the smaller biliary ducts, focal biliary obstruction, cholangiolitis and biliary cirrhosis may ensue, 2 and in those surviving beyond the first few years of life, such hepatic involvement may become a prominent part of the disease complex. It has been estimated that more than 20 per cent of cystic fibrosis patients beyond adolescence have biliary cirrhosis of a degree sufficient to be clinically manifest. 1 Bleeding esophageal varices and/or hypersplenism may complicate portal hypertension and require surgical therapy for successful management. These children, unlike those with extrahepatic portal venous obstruction and normal hepatic function, act much more like the adult cirrhotic with bleeding esophageal varices. Exsanguinating hemorrhage is much more likely to occur, and the absorption of digested blood in the gastrointestinal tract may precipitate hepatic decompensation in an already impaired liver. At the Children's Hospital Medical Center, where well over 2,000 children with cystic fibrosis have been followed, 17 patients with cystic fibrosis and portal hypertension have been treated with portosystemic venous shunts for control of bleeding esophageal varices or varices and hypersplenism, or both, and serve as the basis for this report.