FIBROPOLYCYSTIC LIVER DISEASES
GIUSEPPE BRANCATELLI

Learning objectives
• Understand the different steps of ductal plate development.
• Review a multimodality imaging approach in those diseases caused by ductal plate malformations.
• Understand the pathologic characteristics of congenital fibrocystic hepatobiliary abnormalities and correlate them with the imaging finding. Fibropolycystic liver diseases are a unique group of entities that are thought to stem from a derangement of embryonic ductal plate development at various stages. These conditions include congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, and choledochal cysts. Hepatobiliary and renal anomalies frequently coexist in various combinations, suggesting the expression of a common underlying genetic abnormality. CT and MR imaging play a pivotal role in achieving a correct noninvasive diagnosis of these entities and may help guide patient treatment.

 

ARTIGO COMPLETO (PDF)