Caroli's disease: its diagnosis with non-invasive methods

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Abstract
More than 50 cases of communicating cavernous ectasia of the bile duct have been reported. Diagnosis of this disease has been mainly by invasive methods such as percutaneous transhepatic or operative cholangiography. We were able to obtain a correct diagnosis pre-operatively using ultrasonography, 99Tcm-HIDA hepatobiliary scintigraphy and computed tomography. We wish to emphasize the usefulness of these non-invasive methods. A 16-year-old girl was referred to the Shinshu University Hospital in September 1979 for low-grade fever, right hypochondralgia, anaemia and cyanosis with pain in the right toes. Systemic lupus erythematosus was diagnosed because of the presence of LE-cells and antinuclear antibodies. In November her serum alkaline phosphatase increased to 1500 mlU and her serum transaminases increased slightly (S–GOT61 units, S–GPT 54 units). An isotopic technetium-99m sulphur colloid scan revealed faint round defects extending over both lobes of the liver. Intercostal and transverse grey-scale ultrasonograms were obtained with a Sonolayer SAC–12A digital scan converter using a 3.5 MHz transducer. Two cystic sonolucent structures were found anterior to the intrahepatic portal vein (Fig. 1). The gall bladder was found to be normal by another subcostal scan and no cystic lesions of the kidney were revealed. A hepatobiliary scintigram with 99Tcm-HIDA 70 min after injection revealed retardation of excretion and two saccular dilatations of both intrahepatic bile ducts (Fig. 2). However, there was no dilatation of the common bile duct.