Clinical features and imaging findings of portal biliopathy

Kim, Tae Oh; Kim, Gwang Ha; Hwang, Sang Yeon; Lee, Sang Yong; Heo, Jeong; Kang, Dae Hwan; Song, Geun Am; Cho, Mong; Kim, Suk; Lee, Jun Woo

Abstract

BACKGROUND: The term 'portal biliopathy' has been used to describe abnormalities of the extrahepatic and intrahepatic bile ducts in patients with portal hypertension, especially those with extrahepatic portal vein obstruction. The aim of this study was to delineate the clinical features and imaging findings of portal biliopathy.
METHODS
Clinical and imaging data of 15 patients who had portal biliopathy from April 2001 to March 2005 were reviewed. Two radiologists working in concensus analyzed the imaging finings and each radiologist and gastroenterologist made a comparison between MRCP and ERCP.
RESULTS
Of the 15 patients with portal biliopathy, there were 7 men and 8 women and their mean age was 56.4 years (range, 24 to 78 years). Most of them were unknown origin (11/15, 73%) and 4 patients combined with recurrent pyogenic cholangitis. 4 patients presented with obscure right abdominal pain, 3 had mild fever with chilling and others had no symptoms. Extrahepatic portal vein obstruction was replaced by cavernous transformation in all patients. 3 different types of biliary abnormalities were 3 pseudocholangiocarcinoma type, 8 varicoid type and 4 mixed type. Choledocholithiasis occuring 4 patients (2 CBD stone, 1 GB stone and 1 CBD with IHD stone). Correlation betwen MRCP and ERCP (or PTC) was identical and exact diagnosis was possible. During EST or cholecystectomy, no significant bleeding was found.
CONCLUSIONS
On portal hypertension combined with biliary obstruction, portal biliopathy should be suspected as a possible diagnosis. Knowledge of these clinical features and imaging findings should facilitate accurate diagnosis of portal biliopathy.