Caroli's disease is a rare congenital disorder characterized by focal or diffuse dilatation of the intrahepatic bile ducts. If asymptomatic, this condition may remain undetected for several years. We present a case of focal Caroli's disease, first suspected on sonography, with a coexisting bile duct carcinoma. Both computed tomography (CT) imaging and percutaneous transhepatic cholangiography (PTC) supported a diagnosis of Caroli's disease but did not detect the presence of an associated mass, which was histologically proved to be a bile duct carcinoma. The advantages and disadvantages of different modalities in evaluating Caroli's disease are discussed.
2. Forbes A, Murray Lyon IM: Cystic disease of the liver and biliary tract. Gut1991: 116-122.
3.
3. Nakanuma Y, Terada T: Caroli's disease in congenital hepatic fibrosis and infantile polycystic disease. Liver1982;2:346-354.
4.
4. Balsells J, Margarit C: Adenocarcinoma in Caroli's disease treated by liver transplantation. Hepatobiliary Surgery1993;7:81-86.
5.
5. Bismirth H, Malt RA: Current concepts in cancer: Carcinoma of the biliary tract. N Engl J Med1979;301:704-710.
6.
6. Koenigsberg M, Weiner, SN, Walzer A: The accuracy of sonography in the differential diagnosis of obstructive jaundice: A comparison with cholangiography. Radiology1979;133:157-165.
7.
7. Klatskin G: Adenocarcinoma of the hepatic duct at its bifurcation with the porta hepatis: An unusual tumor with distinctive clinical and pathological features. Am J Med1965;38:241-256.
8.
8. Young W, Blanc C, White SJ: Congenital biliary dilatation: A spectrum of disease detailed by ultrasound. Br J Radiol1990;63:333-337.
9.
9. Miteldstadt CA, Volberg FM, Fisher GJ. Caroli's disease: Sonographic findings. Am J Radiol1980;134:585-589.
10.
10. Lee MG, Cho KS, Auh YH, et al: Hepatic arterial color Doppler signals in Caroli's disease. Clinical Imaging1992;16:234-238.
11.
11. Choi BI, Yeon KM, Kim SH: Caroli's disease: Central dot sign in CT. Radiology1990;174:161-165.
12.
12. Yoshida, H, Itai Y, Minami M: Biliary malignancies occurring in choledochal cysts. Radiology1989;173:389-391.
13.
13. Toma P, Giogio L, Pelizza A: Sonographic pattern of Caroli's disease: Report of 5 new cases. J Clin Ultrasound1991;19:155-158.
14.
14. Savader SJ, Benenati JF, Venbrux AC et al: Choledochal cysts: Classification and cholangiographic appearance. Am J Roentgenol1991;156(2):327-331.
15.
15. Guiband L, Bret PM, Reinhold C, et al: Bile duct obstruction and choledocholithiasis: Diagnosis with MR cholangiography. Radiology1995;197:109-115.
16.
16. Outwater EK, Gordon SJ: Imaging the pancreatic and biliary ducts with MR. Radiology1994;192:19-21.
