Spectrum of biliary abnormalities in portal cavernoma cholangiopathy (PCC) secondary to idiopathic extrahepatic portal vein obstruction (EHPVO)-a prospective magnetic resonance cholangiopancreaticography (MRCP) based study.

OBJECTIVE: To characterize biliary abnormalities seen in portal cavernoma cholangiopathy (PCC) on MR cholangiopancreaticography (MRCP) and elucidate certain salient features of the disease by collaborating our findings with those of previous studies. METHODS: We prospectively enrolled 52 patients with portal cavernoma secondary to idiopathic extrahepatic portal vein obstruction, who underwent a standard MRCP protocol. Images were analyzed for abnormalities involving the entire biliary tree. Terms used were those proposed by the Indian National Association for Study of the Liver. Angulation of the common bile duct (CBD) was measured in all patients with cholangiopathy. RESULTS: Cholangiopathy was seen in 80.7% of patients on MRCP. Extrahepatic ducts were involved in 95% of patients either alone (26%) or in combination with the intrahepatic ducts (69%). Isolated involvement of the intrahepatic ducts was seen in 4.8% of patients. Abnormalities of the extrahepatic ducts included angulation (90%), scalloping (76.2%), extrinsic impression/indentation (45.2%), stricture (14.3%) and smooth dilatation (4.8%). The mean CBD angle was 113.2 ± 19.8°. Abnormalities of the intrahepatic ducts included smooth dilatation (40%), irregularity (28%) and narrowing (9%). Cholelithiasis, choledocholithiasis and hepatolithiasis were seen in 28.6% (12) patients, 14.3% (6) patients and 11.9% (5) patients, respectively. There was a significant association between choledocholithiasis and CBD stricture, with no significant association between choledocholithiasis and cholelithiasis. A significant association was also seen between hepatolithiasis and choledocholithiasis. CONCLUSION: The spectrum of biliary abnormalities in PCC has been explored and some salient features of the disease have been elucidated, which allow a confident diagnosis of this entity. Advances in knowledge: PCC preferentially involves the extrahepatic biliary tree. Changes in the intrahepatic ducts generally occur as sequelae of involvement of the extrahepatic ducts, although isolated involvement of the intrahepatic ducts does occur. Increased angulation of the CBD and scalloping are most commonly seen. Angulation may predispose to choledocholithiasis and thus development of symptomatic cholangiopathy. Choledocholithiasis and hepatolithiasis occur as sequelae of PCC.