Fact-sheet: Multiple biliary hamartoma
Updated on 09/12/2019 at 11:30 AM
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Definition
Other name: Von Meyenburg complex disease.
Hamartomatous lesions of the bile ducts of congenital origin: correspond to remnants of the most peripheral bile ducts, obstructed and involuted within a fibrous stroma, related to a late-onset ductal plate malformation.
Exceptional risk of cholangiocarcinoma due to biliary stasis.
Possible association with:
- Caroli disease
- Congenital hepatic fibrosis
- Autosomal dominant polycystic kidney and liver disease
- Isolated polycystic liver disease.
Clinical features
Asymptomatic
Laboratory findings
No liver function test abnormalities
Ultrasound
Biliary hamartomas may be hyperechoic
MRI
Multiple cystic lesions between 2 and 10 mm, distributed throughout the hepatic parenchyma and not communicating with the bile ducts.
Exceptional presence of a small mural nodule that may enhance.
Differential diagnosis
Unlike Caroli disease:
- No communication with the bile ducts
- Administration of hepatospecific contrast agent does not alter the signal of the cysts