Hepatic Cholangiohamartomas (Von Meyenburg Complexes)

Abstract

Von Meyenburg complexes (multiple bile-duct microhamartomas) are formed by numerous groups of small bile-ducts comprised in a fibrous stroma. Together with other bile-duct hamartomas, such as cholangioadenomas and cholangiocystadenomas (often included under the heading of von Meyenburg complexes), they are closely related pathogenetically to polycystic disease of the liver. The frequent association of other developmental anomalies, both of the liver (hemangiomas) and of other organs (namely cystic disease of the kidney), confirms the malformative origin. The possible malignant transformation of von Meyenburg complexes has recently been reported. Seven cases of hepatic cholangiohamartomas are reported: three multiple bile-duct microhamartomas (1 associated with cystic disease of the kidney), two cholangioadenomas (1 associated with hepatic hemangioma) and two biliary cystadenomas.