A Case Report of Agenesis of the Right Lobe of the Liver with Hepatolithiasis

Abstract

Agenesis of the right lobe of the liver is an extremely uncommon congenital anomaly. We present a case report of a 60-year-old Japanese female patient. She was admitted with the complaint of right hypochondralgia. On admission she had slight jaundice. PTC revealed that there was no right hepatic duct and that the left duct was constricted at the hepatic hilus with intrahepatic dilation and contained stones. CT showed aplasia of the right lobe with compensatory hypertrophy of the left lobe and an ectopic position of the gallbladder. The right hepatic branch of the hepatic artery was not seen on an angiogram. The preoperative diagnosis was hepatolithiasis with agenesis of the right lobe of the liver. On laparotomy, the right lobe of the liver was found to be absent, and the left hepatic duct was bent around the hepatic hilus with many black stones peripheral to it. The operation included cholecystectomy with choledocholithotomy and T-tube drainage as well as left hepaticotomy with left hepaticojejunostomy. Twenty-five cases of right hepatic lobe agenesis have been found since 1956. Our patient is the second reported in Japan. This case was a bit different and probably the first case in the world in which the absence of the lobe has been complicated by hepatolithiasis and black stones, and these black stones are thought to be caused by the anomaly.