[A case of intrahepatic lithiasis associated with intestinal malrotation--consideration of its etiology]

We report a case of intrahepatic lithiasis associated with intestinal malrotation, which showed some bile duct abnormalities, such as cystic dilatation accompanied with stenosis in its distal portion. The resected hepatic tissue was examined histopathologically. Cellular debris due to desquamation of the epithelium, hypersecretion of mucopolysaccharides from the proliferated intrahepatic glands, and periductal laminar or band-like fibrosis were present. The mixture of desquamated epithelium, mucopolysaccharides and bile juice occupied the bile duct lumen, suggesting intrahepatic immature gallstones. The timing of formation of the intrahepatic bile duct is nearly coincident with that of the rotation of the intestine. Intestinal malrotation is sometimes associated with the congenital biliary duct anomaly and therefore the present patient might were well be the case.