Abstract
The patient was a woman in her 20s who had undergone a diversion operation three years ago for congenital biliary dilatation (Todani IV-A type) and pancreaticobiliary maljunction (new Komi classification type Ia). Imaging examination during a follow-up visit revealed a neoplastic lesion in the dilated right hepatic duct, and the patient was referred to our hospital for further evaluation. The lesion was visible by small-bowel endoscopy, and examination of a biopsy specimen revealed the diagnosis of adenocarcinoma. Therefore, based on the diagnosis of perihilar cholangiocarcinoma, right hepatectomy with caudate lobectomy and biliary tract reconstruction was performed following portal embolization. The resected specimen showed two protruding lesions, both of which were diagnosed as adenocarcinoma having a papillary structure. As is generally known, congenital biliary dilatation is associated with a high risk of development of cholangiocarcinoma, and a diversion operation is considered as the standard treatment. In recent years, however, there have been several case reports of cholangiocarcinoma developing after a diversion operation. Therefore, it is essential to further study the optimal operative procedure for congenital biliary dilatation involving dilatation of the intrahepatic bile duct.
