Fact-sheet: Multiple bile duct hamartomas
Updated on 10/07/2017 at 6:33 AM
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Definition
Hamartomatous biliary lesions of congenital origin (ductal plate malformations).
Small peribiliary clusters of mildly dilated bile ducts surrounded by fibrous stroma.
Clinical features
Asymptomatic
Laboratory findings
Normal
Ultrasound
Hyperechoic in most cases. Occasionally hypoechoic or anechoic.
CT
Multiple well-defined hypodense lesions, each smaller than 1 cm.
MRI
Markedly hypointense on T1 and markedly hyperintense on T2.
Starry sky/comet-tail appearance, with no communication with the biliary tree on MR cholangiography sequences.
Management
Exceptional risk of malignant transformation into cholangiocarcinoma, not warranting treatment.
Differential diagnosis
Simple biliary cyst: hypoechoic.
Metastases: hypoechoic, with delayed enhancement on contrast-enhanced MRI.