THE AMERICAN JOURNAL OF GASTROEN7EROLGGY

Copyright 0 1998 by Am. Coil. of Gastroenterology Published by Elsevier Science Inc.

Vol. 93, No. 1. 1998 ISSN 0002-9270/98/$19.00

PII SOOO2-9270(97)CKtO33-6

Caroli’s Disease: A Magnetic Resonance -Cholangiopancreatography Diagnosis

Tarik Asselah, M.D., Olivier Ernst, M.D., Geraldine Sergent, M.D., Claude L’hermin6, M.D., and Jean-Claude Paris, M.D.

Departments qf Hepatogastroenterology and Radiology, Hdpital Huriez, Centre Hospitalier Universitaire de Lilie, Lille, Cedex. France

Magnetic resonance cholangiopancreatography (MRCP) has received much attention in the recent literature as a

noninvasive alternative to endoscopic retrograde cholan-

giography, primarily for hiliary calculus disease, but

also for the less common indication of evaluation of

biliary anomalies. We present a case of Caroli’s disease

in which the diagnosis can be clearly inferred by MRCP.

The findings of MRCP and endoscopic retrograde

cholangiopancreatography are similar. This new proce-

dure could be a noninvasive alternative to direct cholan-

giography and perhaps will become the first-choice im-

aging technique for diagnosing Caroli’s disease. (Am J

Gastroenterol 1998;93:109-110.0 1998 by Am. Coll. of

Gastroenterology)

INTRODUCTION

In 1958, Jacques Caroli described communicating cav-

ernous ectasia of the biliary tree as an uncommon cause of

chronic hepatobiliary disease (1). This condition is charac-

terized by multiple segmental communicating dilatations-of

intrahepatic bile ducts affecting all or part of the liver.

Direct cholangiography, obtained either by endoscopic ret-

rograde cholangiopancreatography (ERCP) or percutaneous

transhepatic cholangiography (PTC). is the accepted method

for an accurate diagnosis (2). However, these two methods

are invasive with serious complications (sepsis, bile leak.

bleeding, and death) (3, 4). Sepsis, the most common of

these complications, occurs especially when obstruction ex-

ists. Magnetic resonance cholangiopancreatography (MRCP)

is a new imaging technique that has already proved its

efficiency as a noninvasive alternative to direct cholangiog-

raphy in biliary obstruction (5). We present here a case of

Caroli’s disease, with an adequate cholangiogram (obtained

by ERCP) who also underwent MRCP; therefore, the two

techniques may be compared.

Received Ma?, 8, 1997; accepted Aug. 20, 19%‘.

CASE REPORT

A 22-year-old woman was admitted to our hospital be-

cause of hematemesis. Past history was not significant, and no family history of cirrhosis was known. Physical exami-

nation revealed an enlarged spleen. There were no signs indicating liver dysfunction. Hemoglobin level was 7 g/dL, and liver tests were normal. Esophageal varices were easily

visualized by endoscopy. Abdominal computed tomography (CT) showed cystic images thinly distributed in the liver.

ERCP was performed, which showed multiple saccular and fusiform dilatation of the intrahepatic duct (Fig. 1) consis-

tent with Caroli’s disease. A magnetic resonance image examination was performed on a 1.5-T system (Vision,

Siemens, Erlangen, Germany). We used the HASTE se- quence with the following parameters: effective TE 66 ms,

number of excitations 1, matrix 128 X 256, slice thickness 4 mm, acquisition time 15 s for 13 slices. Single slices depicted multiple cystic images in connection with the bil- iary tree, and Maximum Intensity Projection showed the same pattern in the entire liver (Fig. 2). The main bile duct

appeared normal. To control portal hypertension, a spleno-renal shunt was

performed. Liver biopsy showed fibrosis consistent with

congenital hepatic fibrosis. No further episodes of bleeding have occurred since.

DISCUSSION

Caroli’s disease is characterized by multifocal dilatations

of segmental bile ducts. Caroli described two types: the rare isolated variety (type I) characterized by recurrent episodes of cholangitis; and the more frequently occurring (type 2), seen in our patient, associated with congenital hepatic fi- brosis, and consequently with portal hypertension (1, 6).

Previous reports of Caroli’s disease emphasize the diffi- culty of diagnosis, and several imaging modalities have been proposed, such as sonography, CT, CT after intrave- nous injection of biliary contrast, or hepatobiliary scintig- raphy (7). Demonstration of communication between sac- culi and bile ducts is important in distinguishing Caroli’s disease from polycystic liver disease and multiple abscesses. Communication may be evident on CT or sonograms, but

110 ASSELAH et al. AJG - Vol. 93, No. 1. 1998

FIG. 1. A 22-year-old woman with Caroli’s disease. ERCP shows dilatations of the peripheral intrahepatic biliary radicles characteristic of Caroli’s disease.

cholangiography is definitive. Therefore, direct cholangiog-

raphy is considered the method of choice for an accurate diagnosis of Caroli’s disease (2). However, serious compli- cations (sepsis, bile leak, bleeding, and death) may occur with both ERCP and PTC, with an overall incidence of

approximately 3% (3,4). In Caroli’s disease, because of the high risk of bacterial cholangitis, these invasive procedures must be performed only when communication of the cysts

with the biliary tract cannot be approached by a noninvasive method. MRCP provides “cholangiographic” images of the biliary system comparable to ERCP and PTC and requires neither the use of contrast agents nor any biliary intervention

(5). To the best of our knowledge, only one case of Caroli’s disease has been studied by MRCP, but direct cholangio-

graphy was not communicated, so comparison was not possible (8). In our case, magnetic resonance cholangiogram and direct cholangiogram can be analyzed and the findings are similar. MRCP could be a noninvasive alternative to direct cholangiography and perhaps will become the first-

choice imaging technique for diagnosing Caroli’s disease.

Reprint requests and correspondence: Olivier Ernst. M.D., Service de Radiologie EST, Centre Hospitalier Universitaire de Lille. F-59037 Lille. Cedex. France.

REFERENCES

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Caroli J, Soupault R, Kossakowski J. et al. La dilatation polykystique congenitale des voies biliaires intra-htpatiques: Essai de classification. Sem. Hop. Paris 1958;34:488-95. Missavage AE, Sugawa C. Caroli’s disease: Role of endoscopic retro- grade cholangiopancreatography. Am J Gastroenterol 1983:78:815-7. Bilbao MK, Dotter CT. Lee TG. et al. Complication of retrograde cholangiopancreatography (ERCP): A study of 10.000 cases. Gastroen- terology 1976;70:314-20. Ariyama J. Percutaneous transhepatic cholangiography. In: Margulis AR, Burenne HJ. eds. Alimentary tract radiology. St. Louis: Mosby, 1988:2229-41. Hall-Craggs M, Allen C. Owens C. et al. MR cholangiography: Clinical evaluation in 40 cases. Radiology 1993: 189:423-7. Kerr DNS, Harrison CV. Sherlock S. et al. Congenital hepatic fibrosis. Q.J. Med 1961;30:91-117. Miller WJ, Sechtin AG, Campbell WL. et al. imaging findings in Caroli’s disease. AJR 199_5;165:333-7. Pavone P, Laghi A. Catalano C. et al. Caroli’s disease: Evaluation with MR cholangiopancreatography. AJR 1996; 166:216-7.

FIG. 2. MRCP (Maximum Intensity Projection) depicts numerous cystic images in connection with the biliary tree and normal appearance of the main bile duct.