Most patients are asymptomatic. When patients do present with symptoms the most common symptom is abdominal pain. Other symptoms include fever, weight loss, and jaundice.[1]
Biliary duct hamartomas are defects resulting from the failure of embryonic bile duct involution that affect the small interlobular bile ducts. Patients with polycystic kidney disease and polycystic liver are far more likely to have them.[1]
Histopathology of a bile duct hamartoma, high magnification, H&E stain. It shows typical features of bile duct hamartoma: Small to medium sized, irregularly shaped bile ducts lined by bland cuboidal epithelium (may also be flattened). Prominent intervening collagenous stroma. Bile ducts containing eosinophilic debris (may also contain inspissated bile)
von Meyenburg Complex in ultrasound. Numerous little cysts with ringdown artefacts.
AtCT scans, bile duct hamartomas appear as small, well-defined hypo- or isoattenuating masses with little or no enhancement after contrast administration.[3]AtMRI, they appear hypointense on T1-weighted images, iso- or slightly hyperintense on T2-weighted images, and hypointense after administration of gadolinium based contrast-agent.[3]
^Desmet, VJ (Jan 1998). "Ludwig symposium on biliary disorders--part I. Pathogenesis of ductal plate abnormalities". Mayo Clin Proc. 73 (1): 80–9. doi:10.4065/73.1.80. PMID9443684.
^ abHorton, KM; Bluemke, DA; Hruban, RH; Soyer, P; Fishman, EK (Mar–Apr 1999). "CT and MR imaging of benign hepatic and biliary tumors". Radiographics. 19 (2): 431–51. doi:10.1148/radiographics.19.2.g99mr04431. PMID10194789.
