BACKGROUND/AIMS: Biliary obstruction secondary to portal cavernoma is a rare and little-known entity. From 1985 to 1994, we observed eight cases of portal cavernoma compressing the biliary tract. We report here the features of biliary involvement in these cases of portal cavernoma including the circumstances of diagnosis, biliary tract morphology, liver pathology and the efficiency of various treatments. METHODS AND RESULTS: The causes of portal vein obstruction were portal vein thrombosis in one case, peritonitis in another, omphalitis in two cases, portal vein catheterization in one case and unknown in two cases. The portal cavernoma was revealed through esophageal varices ruptures in four patients. The mean time from portal cavernoma diagnosis to biliary involvement was 8 years (range 0-21). Six patients had acute cholangitis, one of whom revealed portal vein obstruction. All the patients had abnormal liver function tests. Imaging techniques (transparietal abdominal ultrasonography and abdominal computed tomography scan [n = 8], endosonography [n = 5] and endoscopic retrograde cholangiography [n = 7]) showed in all cases an extraluminal obstacle, laminating the common bile duct. Pathologic examination of the liver showed secondary biliary cirrhosis in one patient, periportal and perisinusoidal fibrosis in another and no abnormalities in three other cases. Three patients were treated by endoscopic sphincterotomy but cholangitis persisted in two cases, leading to death in one. One patient who underwent a splenorenal shunt was symptom-free 60 months after surgery. Balloon endoscopic dilatation of the common bile duct, performed in one case, led to normalization of liver biological tests. The patients with abnormal liver pathology were treated by propranolol and ursodesoxycholic acid. No complication had occurred after 6 and 24 months of follow-up. CONCLUSIONS: In conclusion, eliminating biliary obstruction seems to be essential in providing the best change of survival for patients when biliary obstruction becomes symptomatic. In cases of abnormal liver pathology, associating propranolol with ursodesoxycholic acid would seem to be useful.
BACKGROUND/AIMS: Biliary obstruction secondary to portal cavernoma is a rare and little-known entity. From 1985 to 1994, we observed eight cases of portal cavernoma compressing the biliary tract. We report here the features of biliary involvement in these cases of portal cavernoma including the circumstances of diagnosis, biliary tract morphology, liver pathology and the efficiency of various treatments. METHODS AND RESULTS: The causes of portal vein obstruction were portal vein thrombosis in one case, peritonitis in another, omphalitis in two cases, portal vein catheterization in one case and unknown in two cases. The portal cavernoma was revealed through esophageal varices ruptures in four patients. The mean time from portal cavernoma diagnosis to biliary involvement was 8 years (range 0-21). Six patients had acute cholangitis, one of whom revealed portal vein obstruction. All the patients had abnormal liver function tests. Imaging techniques (transparietal abdominal ultrasonography and abdominal computed tomography scan [n = 8], endosonography [n = 5] and endoscopic retrograde cholangiography [n = 7]) showed in all cases an extraluminal obstacle, laminating the common bile duct. Pathologic examination of the liver showed secondary biliary cirrhosis in one patient, periportal and perisinusoidal fibrosis in another and no abnormalities in three other cases. Three patients were treated by endoscopic sphincterotomy but cholangitis persisted in two cases, leading to death in one. One patient who underwent a splenorenal shunt was symptom-free 60 months after surgery. Balloon endoscopic dilatation of the common bile duct, performed in one case, led to normalization of liver biological tests. The patients with abnormal liver pathology were treated by propranolol and ursodesoxycholic acid. No complication had occurred after 6 and 24 months of follow-up. CONCLUSIONS: In conclusion, eliminating biliary obstruction seems to be essential in providing the best change of survival for patients when biliary obstruction becomes symptomatic. In cases of abnormal liver pathology, associating propranolol with ursodesoxycholic acid would seem to be useful.