Download or read book CONGENITAL FIBROCYSTIC LIVER DISEASE. Imaging Features and Differential Diagnosis written by Elena Inchausti and published by . This book was released on 2017 with total page pages. Available in PDF, EPUB and Kindle. Book excerpt: LEARNING OBJETIVES:To understand the diseases that come from the abnormal embryological development of the ductal plate, known as fibropolicystic liver diseases.To illustrate the imaging findings of congenital fibrocystic liver diseases.To be able to differentiate each entity from other differential diagnosis. BACK GROUND:Ductal plate malformations, known as fibrocystic liver diseases, are a group of congenital disorders resulting from the abnormal embryogenesis of the biliary ductal system. These entities include congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, and cholecochal cyst. The hepatic lesions can be associated with renal anomalies, suggesting the expression of a common underlying genetic abnormality.IMAGING FINDINGS:The different types of fibrocystic liver disease demonstrate characteristic findings at ultrasound, computed tomography and magnetic resonance imaging. We present different cases of each entity including.-tCongenital hepatic fibrosis.-tBiliary hamartomas.-tAutosomal dominant polycystic disease.-tCaroli disease.-tCholedochal cysts.We also present other diseases that can mimic these entities, including early viral alcoholic cirrhosis, hepatic lymphoma or metastasis, when it comes to congenital hepatic fibrosis. In the case of Caroli disease differential diagnosis with the sclerosing colangitis must be done. CONCLUSION:Accurate diagnosis of fibrocystic liver diseases is important to indicate an appropriate clinical management, as they can be clinically silent or can cause signs and symptoms such as portal hypertension, infections or space-occupying masses.