AJR:179, October 2002 1053 Original Report OBJECTIVE. The purpose of our study was to describe the spectrum of radiologic and pathologic features of Caroli’s disease. CONCLUSION. Caroli’s disease and its complications have overlapping radiologic ap- pearances that reflect the underlying pathology of fibrosis, ductal dilatation, cholangitis, stone formation, and malignancy. aroli’s disease, or communicating cavernous ectasia of the intrahe- patic bile ducts, is an autosomal re- cessive disorder and is among the ductal plate malformations that occur at different levels in the developing biliary tree, leading to several clinicopathologic entities [1]. The disease re- sults from the arrest of or a derangement in the normal embryologic remodeling of ducts and causes varying degrees of destructive inflam- mation and segmental dilatation. If the large intrahepatic bile ducts are affected, the result is Caroli’s disease, whereas abnormal develop- ment of the small interlobular bile ducts results in congenital hepatic fibrosis. If all levels of the biliary tree are involved, features of both congenital hepatic fibrosis and Caroli’s disease are present. This condition has been termed “Caroli’s syndrome.” Since the original description by Caroli et al. in 1958 [2], a number of case reports and small series have appeared in the radiology literature describing the cholangiographic [3, 4], sonographic [5, 6], CT [7, 8], and MR im- aging [9] features of the disease. We under- took this study to reevaluate the radiologic features of Caroli’s disease, with an empha- sis on reporting the spectrum of the features of the disease and the correlation with gross and microscopic pathologic findings. To our knowledge, ours is the largest series of pa- tients with Caroli’s disease reported in the ra- diology literature to date. Materials and Methods Data on 19 patients with the diagnosis of Caroli’s disease were entered into the archives of our institu- tion from September 1981 to July 2000. Two pa- tients were excluded from the study because of the lack of confirmatory histopathologic findings. Our final study population consisted of 17 patients. Histopathology records were available for all pa- tients and were reviewed by a hepatobiliary patholo- gist. The diagnosis of Caroli’s disease or Caroli’s syndrome was reconfirmed for all patients. In addi- tion, histopathologic specimens were evaluated for the presence of other conditions associated with ductal plate malformations, including cirrhosis, cho- langitis, and malignancy. We reviewed the clinical histories to find each pa- tient’s age, sex, and presenting signs and symptoms. Because Caroli’s disease has a known pattern of in- heritance, we also evaluated the race of each patient to establish which population among the patients in our study was affected most frequently by the dis- ease. Radiologic studies of the hepatobiliary system were available for all patients. Cholangiography was performed in 14 patients (percutaneous transhepatic cholangiography in eight, endoscopic retrograde cholangiopancreatography (ERCP) in five, and MR cholangiopancreatography in one). Cross-sectional imaging studies were available in 14 patients and in- cluded 12 CT scans (one unenhanced, three IV con- trast-enhanced, and eight enhanced and unenhanced studies); eight sonograms; and three MR imaging studies. Fourteen patients had both cross-sectional imaging studies and cholangiograms. The three pa- tients without cross-sectional imaging studies had undergone direct cholangiography (either percutane- ous transhepatic cholangiography or ERCP). Be- Angela D. Levy 1,2 Charles A. Rohrmann, Jr. 1,3 Linda A. Murakata 4 Gael J. Lonergan 1,2 Received February 12, 2002; accepted after revision March 25, 2002. The opinions and assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Departments of the Army, Navy, Air Force, or Defense. 1 Department of Radiologic Pathology, Armed Forces Institute of Pathology, M-121, 6825 16th St., N.W., Washington, DC 20306-6000. Address correspondence to A. D. Levy. 2 Department of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Rd., Bethesda, MD 20814. 3 Department of Radiology, University of Washington, 1959 N.E. Pacific, Seattle, WA 98195-7115. 4 Department of Hepatic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000. AJR 2002;179:1053–1057 0361–803X/02/1794–1053 © American Roentgen Ray Society C Caroli’s Disease: Radiologic Spectrum with Pathologic Correlation Downloaded from www.ajronline.org by 171.243.67.90 on 05/30/23 from IP address 171.243.67.90. Copyright ARRS. For personal use only; all rights reserved
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