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Original Report |
1 Department of Radiologic Pathology, Armed Forces Institute of Pathology,
M-121, 6825 16th St, N.W., Washington, DC 20306-6000.
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.
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.
Abstract
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CONCLUSION. Caroli's disease and its complications have overlapping radiologic appearances that reflect the underlying pathology of fibrosis, ductal dilatation, cholangitis, stone formation, and malignancy.
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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 imaging [9] features of the disease. We undertook this study to reevaluate the radiologic features of Caroli's disease, with an emphasis 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 patients with Caroli's disease reported in the radiology literature to date.
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Histopathology records were available for all patients and were reviewed by a hepatobiliary pathologist. The diagnosis of Caroli's disease or Caroli's syndrome was reconfirmed for all patients. In addition, histopathologic specimens were evaluated for the presence of other conditions associated with ductal plate malformations, including cirrhosis, cholangitis, and malignancy.
We reviewed the clinical histories to find each patient's age, sex, and presenting signs and symptoms. Because Caroli's disease has a known pattern of inheritance, we also evaluated the race of each patient to establish which population among the patients in our study was affected most frequently by the disease. 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 included 12 CT scans (one unenhanced, three IV contrast-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 patients without cross-sectional imaging studies had undergone direct cholangiography (either percutaneous transhepatic cholangiography or ERCP). Because our patients were referred from many institutions, their studies were performed on a variety of equipment, and differing protocols were followed, so the techniques were not standardized.
All radiologic studies were reviewed for the presence or absence of biliary dilatation; if dilatation was present, its extent, character, and dimensions were assessed. The size of the extrahepatic bile ducts was measured by standard references present on the images. The presence of intrahepatic biliary dilatation was determined subjectively by consensus. Intrahepatic biliary dilatation was assessed for distribution (diffuse or segmental) and character (saccular or fusiform). Cholangiograms were evaluated for the presence of focal areas of ductal narrowing or stenosis. Secondary findings on cholangiograms—such as filling defects that could represent lithiasis or a neoplasm and strictures that resembled neoplasms—were also evaluated. Strictures aroused suspicion of possible neoplasia if they had shoulders, irregular margins, or irregular tapering. The cross-sectional imaging studies were evaluated for secondary findings such as the presence of enhancing fibrovascular bundles in dilated intrahepatic ducts ("central dot sign") [8], lithiasis, or masses that suggested a complicating malignancy or abscess.
Three radiologists reviewed all radiologic studies retrospectively, with final interpretations made by consensus. The final diagnosis for each patient was known at the time of interpretation. After the review of the radiologic studies, correlation with pathology reports and photographs of gross specimens (n = 13) was performed. Photographs of histopathologic specimens were available for all patients with malignancies, so the location of the malignancy could be directly correlated with its radiologic appearance.
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Pathologic Findings
All patients had histologic confirmation of a ductal plate malformation.
Caroli's disease was present in 12 (71%) and Caroli's syndrome (Caroli's
disease and congenital hepatic fibrosis) in five (29%)
(Fig. 1). Cirrhosis was present
in eight patients (47%). Thirteen patients (76%) had histologic evidence of
cholangitis. The gross specimen photographs of the 13 patients with such
photographs available showed saccular or fusiform ductal dilatation that
correlated with the radiologic findings for all of the patients. Periductal
fibrosis and stones were visible macroscopically in five patients.
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Four patients (24%) had complicating neoplasms, all of which were classified as cholangiocarcinomas (adenocarcinomas arising from intrahepatic bile ducts). One patient had a case of cholangiocarcinoma that was subtyped as a papillary adenocarcinoma.
Radiologic Findings
Ductal features.—An intrahepatic duct abnormality was
present in all patients, and extrahepatic duct dilatation was present in nine
(53%). In the remaining eight patients (47%), dilatation of only the
intrahepatic ducts was found. All of the patients with extrahepatic duct
dilatation exhibited fusiform enlargement of the common hepatic duct and the
common bile duct (Fig. 2).
Dilatation of the extrahepatic bile duct ranged from 10 to 30 mm in diameter
(mean, 18.3 mm) and was smooth and fusiform in all patients. No patients had
saccular dilatation of the extrahepatic bile duct.
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Intrahepatic duct dilatation was segmental in 14 patients (82%) (Fig. 2). Dilatation was slightly more common in the right lobe (n = 8) than in the left (n = 6). The three remaining patients had diffuse involvement of all intrahepatic ducts (Fig. 3). Intrahepatic biliary dilatation with saccular features was present in 13 patients (76%) (Fig. 4). Fusiform dilatation was present in four patients (24%) (Fig. 5A,5B).
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In all 14 patients with cholangiograms available, we found multiple areas of focal narrowing or stenosis in the affected biliary segments. An alternating pattern of stricture and dilatation typifies this feature (Figs. 2, 4, and 5A,5B). The presence of stones was confirmed pathologically in eight patients (47%); and in seven of the eight, the stones were visible on cholangiography (Fig. 5A,5B).
Saccular or fusiform biliary dilatation with attenuation similar to that of water was identified in all patients with an available CT scan (n = 12) except one, a patient with intraductal papillary adenocarcinoma. This patient had an enhancing soft-tissue density in the dilated ducts. Enhancing fibrovascular bundles in or along the margin of dilated ducts (the central dot sign) were present in five (36%) of the 14 patients with cross-sectional imaging studies. This finding correlated with the presence of a fibrovascular bundle in or at the side of the dilated duct at gross pathology in two of the four patients who had images with the central dot sign and who also had both cross-sectional imaging studies and a gross specimen photograph (Fig. 6A,6B). One patient had an intrahepatic abscess that appeared as a hypodense mass on CT. None of the patients had imaging or pathologic evidence of an extrahepatic complication such as a subhepatic or subdiaphragmatic abscess, pancreatitis, or pancreatic pseudocyst.
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Malignancy.—The four patients with complicating adenocarcinomas ranged in age from 26 to 77 years (mean, 47.7 years). The average time from the onset of symptoms to the development of malignancy was 5.3 years. All four patients presented with right upper quadrant pain. Two patients had infiltrating masses along the walls of the large bile ducts of the left lobe of the liver. In both patients, the masses were identified on cholangiograms as irregularly narrowed bile ducts. In all four patients, fusiform dilatation of the more proximal duct was seen; one of the patients had filling defects on cholangiography that were confirmed at pathology to represent intraductal lithiasis (Fig. 7A,7B). The third patient had a focal 3-cm circumscribed mass in the left lobe of the liver identified on CT. The fourth patient had multiple intraductal enhancing masses on CT (Fig. 8A,8B) that were identified histopathologically as papillary adenocarcinoma. No extrahepatic findings were present in the patients with malignancy.
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TopAbstractIntroductionMaterials and MethodsResultsDiscussionReferences |
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The cholangiographic features of Caroli's disease are well established as saccular or fusiform dilatation of the intrahepatic bile ducts [4]. Irregular bile duct walls, strictures, and stones may be present. In our study, we found that segmental ductal dilatation is more common (82% of our patients) than diffuse ductal dilatation. Alternating areas of stricture and dilatation were observed in all patients with cholangiograms. These features correlate with gross and histopathologic findings of periductal fibrosis, inflammation, and, in two patients, malignancy that infiltrated the ductal walls.
The association of Caroli's disease with extrahepatic bile duct dilatation is mentioned throughout the literature, as is the coexistence of Caroli's disease with choledochal cysts. The exact incidence of extrahepatic duct involvement in Caroli's disease is not known. A literature review of 46 cases found extrahepatic dilatation in 21% of patients [10]. Our series shows that this figure may be higher than previously thought: 53% of our patients had extrahepatic duct dilatation. Recognition of this feature is important because Caroli's disease should be considered in the differential diagnosis for patients with intra- and extrahepatic biliary dilatation. Repeated bouts of cholangitis, stone formation, and stone passage may explain extrahepatic duct dilatation in some patients with Caroli's disease or Caroli's syndrome.
As shown in our series, the presence of diffuse fusiform dilatation of the extrahepatic duct measuring 3 cm or less in diameter combined with the characteristic intrahepatic ductal findings may be helpful in differentiating patients with Caroli's disease from patients with a choledochal cyst and intrahepatic biliary dilatation. Although the Todani classification of choledochal cysts includes Caroli's disease as a type V choledochal cyst [11], the current understanding of the pathogenesis of Caroli's disease (autosomal recessive and often associated with renal disorders) and choledochal cysts (congenital and not associated with renal disorders) makes it unlikely that these entities are related.
Cholangiography and cross-sectional imaging may be useful in differentiating the common complications of cholangitis and stone formation from the less frequent complication of malignancy. Bloustein [12] reported the incidence of malignancy in Caroli's disease as 7%. The 24% incidence of malignancy seen in our patient population is higher than expected and may be the result of selective referral bias in our study population. Although small in number, our study population shows that a range of radiologic appearances of complicating malignancy (focal hepatic mass, intraductal mass, and biliary stricture) exists. Diagnosing malignancy can be challenging in patients with Caroli's disease because the cholangiographic and cross-sectional imaging features of the disease and its complications overlap. MR cholangiography may be useful in the evaluation of a suspected malignancy. The modality has the added benefit of revealing both intraluminal and extraluminal disease and has recently been reported as an aid in the diagnosis of malignancy in Caroli's disease [13].
The differential diagnosis of Caroli's disease includes primary sclerosing cholangitis, recurrent pyogenic cholangitis, polycystic liver disease, a choledochal cyst, biliary papillomatosis, and (occasionally) obstructive biliary dilatation. Primary sclerosing cholangitis and recurrent pyogenic cholangitis may be associated with duct dilatation, stenosis, intrahepatic calculi, and malignancy. However, the ductal dilatation in primary sclerosing cholangitis is rarely saccular and is typically more isolated and fusiform, which is not characteristic of Caroli's disease. Additionally, 70% of white patients with primary sclerosing cholangitis have coexisting inflammatory bowel disease (namely, ulcerative colitis).
Recurrent pyogenic cholangitis is the most difficult diagnosis to exclude because patients with pyogenic cholangitis present with sepsis and have intra- and extrahepatic biliary dilatation. The presence of saccular dilatation favors the diagnosis of Caroli's disease because saccular dilatation is unusual in recurrent pyogenic cholangitis. However, the occasional case of Caroli's disease that manifests as diffuse fusiform dilatation may be impossible to distinguish from recurrent pyogenic cholangitis using imaging alone.
Polycystic liver disease may initially mimic Caroli's disease. Patients with polycystic liver disease may present with both hepatic and renal cysts. However, the hepatic cysts of polycystic liver disease only rarely communicate with the bile ducts. These patients typically have intrinsically normal bile ducts.
Several inherent limitations to our study are related to the natural selection bias in a referral population. The examinations of our patients were performed at various institutions over a 19-year period with different equipment and protocols. The lack of standardization of imaging parameters and injection techniques cannot be overcome in a study population of this type and may influence final radiologic interpretations. We had the advantage, however, of being able to review the resected gross pathology specimens and pathology records to help ensure that radiologic interpretations were correct.
In conclusion, we have shown that the spectrum of radiologic appearances of Caroli's disease ranges from segmental to diffuse biliary dilatation, from saccular to fusiform dilatation, and from isolated intrahepatic involvement to intra- and extrahepatic involvement. Knowledge of this spectrum and of the features of a complicating malignancy are important in the diagnosis and treatment of patients with Caroli's disease.
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