HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Figures Only Full Text Full Text (PDF) CME Test (opens in a new window) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Brancatelli, G. Articles by Lagalla, R. Search for Related Content PubMed PubMed Citation Articles by Brancatelli, G. Articles by Lagalla, R. Related Collections Magnetic Resonance Imaging Computed Tomography Gastrointestinal Radiology

Fibropolycystic Liver Disease: CT and MR Imaging Findings¹

¹ From the Department of Radiology, University of Palermo, Italy (G.B., R.L.); the Department of Radiology, University of Pittsburgh Medical Center, Rm 4660, CHP, MT, 200 Lothrop St, Pittsburgh, PA 15213 (M.P.F.); the Department of Radiology, Beaujon Hospital, Clichy, France (V.V., M.P.V.); and the Department of Radiology, University of Rome "La Sapienza," Italy (D.M.). Recipient of a Cum Laude award for an education exhibit at the 2003 RSNA Scientific Assembly. Received May 26, 2004; revision requested June 30 and received August 12; accepted August 18. All authors have no financial relationships to disclose. Address correspondence to M.P.F. (e-mail: federlemp{at}upmc.edu).

Fibropolycystic liver disease encompasses a spectrum of related lesions of the liver and biliary tract that are caused by abnormal embryologic development of the ductal plates. These lesions (congenital hepatic fibrosis, biliary hamartomas, autosomal dominant polycystic disease, Caroli disease, choledochal cysts) can be clinically silent or can cause signs and symptoms such as cholangitis, portal hypertension, gastrointestinal bleeding, infections, and space-occupying masses. The different types of fibropolycystic liver disease demonstrate characteristic findings at computed tomography (CT) and magnetic resonance (MR) imaging. Patients with congenital hepatic fibrosis typically have imaging evidence of liver morphologic abnormalities, varices, splenomegaly, renal lesions, and other associated ductal plate abnormalities. Biliary hamartomas usually manifest as multiple cysts that are nearly uniform in size and measure up to 15 mm in diameter. Autosomal dominant polycystic disease typically manifests as an enlarged and diffusely cystic liver. In Caroli disease, cystic or fusiform dilatation of the intrahepatic ducts is seen, as well as the "central dot sign," which corresponds to a portal vein branch protruding into the lumen of a dilated bile duct. Choledochal cyst manifests as a fusiform or cystic dilatation of the extrahepatic bile duct. Awareness of these CT and MR imaging features is essential in detecting and differentiating between various fibropolycystic liver diseases and can assist in proper management.

© RSNA, 2005

This article has been cited by other articles:

				D. E. Morgan, M. E. Lockhart, C. L. Canon, M. P. Holcombe, and J. S. Bynon Polycystic Liver Disease: Multimodality Imaging for Complications and Transplant Evaluation RadioGraphics, November 1, 2006; 26(6): 1655 - 1668. [Abstract] [Full Text] [PDF]