Biliary papillomatosis: clinical, cholangiographic and cholangioscopic findings

Biliary papillomatosis (BP) is a very rare disease and its clinical features and outcome are not well known. The aims of this study were to describe the characteristic cholangiographic findings and to define the role of cholangioscopic examination in the diagnosis and treatment of this disorder.

Nine patients (six men and three women, mean age 57 years) were diagnosed as BP among 5361 cases of endoscopic retrograde cholangiography (ERC) from 1990 to 1997 in our institution. The cholangiographic and cholangioscopic findings as well as clinical features were retrospectively analyzed.

ERC findings showed multiple small, round-to-ovoid filling defects in the bile duct and ductal wall irregularity in all the patients. Seven out of nine patients underwent percutaneous transhepatic cholangioscopic (PTC) examination. Additional small papillary lesions in the intrahepatic bile duct were detected by cholangioscopic examination in four patients whose ERC findings only revealed the extrahepatic lesions. Two of these four patients underwent curative resection. In these two patients, the initial surgical plan was changed from Whipple's operation to hepatico-pancratico-duodenectomy after preoperative cholangioscopic examination due to the detection of new lesions in the intrahepatic bile duct.

ERC findings of BP were highly characteristic. When BP is suspected by conventional imaging including ERC, preoperative percutaneous transhepatic cholangioscopic examination is, however, strongly recommended. This procedure may be beneficial to precisely determine the ductal extension of the disease, hence to decide whether or not hepatic resection is needed as well as to confirm the histology.