(5) Biliary hamartomas, also referred to as von Meyenburg complex
, are common, benign lesions characterized by the presence of numerous, small cystic lesions in the liver parenchyma ranging from 2 mm to 15 mm (Figure 2).
The bile duct adenoma: a lesion distinct from Meyenburg complex. Arch Pathol Lab Med.
(30) By contrast, bile duct hamartomas (von Meyenburg complexes) are usually multiple, are cystic, contain bile, and are lined by flattened uniform "biliary" type epithelial cells.
Also known as von Meyenburg complexes, bile duct hamartomas arise from ductal plate malformations that involve the small interlobular bile ducts.
Bile duct hamartomas (Von Meyenburg complexes): Value of MR imaging in diagnosis.
This leads to different congenital bile duct disorders, such as Caroli disease and syndrome, autosomal recessive polycystic kidney disease, autosomal dominant polycystic kidney disease, congenital hepatic fibrosis, and bile duct hamartomas (BDHs, also known as von Meyenburg complexes
). The clinical presentation and outcome of these congenital diseases show great variation; some patients die as neonates or in early infancy (eg, perinatal, neonatal, and infantile forms of autosomal recessive polycystic kidney disease), and others (eg, BDH) present as clinically asymptomatic and usually incidental findings at laparotomy or autopsy. While approximately 7% of patients with Caroli disease ultimately develop a cholangiocarcinoma, BDH has only rarely been found in association with it.