[Chronic cholestatic liver disease and grand mal seizures]

A 48-year-old woman was hospitalised because of grand-mal seizures. 3 years previously a malignant melanoma had been resected from the skin of the back. She was also known to have chronic cholestasis of unknown cause. On physical examination there were postictal signs, but no neurological abnormalities and no jaundice.

Biochemical tests demonstrated greatly increased alkaline phosphatase (576U/I). gamma-GT (1556U/I) and leucine aminopeptidase (258U/I). The transaminases were only slightly raised (GOT 113U/I, GPT 82U/I). Magnetic resonance imaging of the brain revealed a single intracerebral space-occupying lesion, compatible with a melanoma metastasis. Endoscopic retrograde cholangiopancreatography discovered a filiform, short stenosis in the choledochal duct, histologically an adenocarcinoma.

The cerebral metastasis was removed stereotactically without complications. A Whipple-type gastroduodenopancreatectomy was performed 2 months later. Histology of an intraoperative liver biopsy revealed Caroli's syndrome (focal intrahepatic biliary dilatation) with congenital hepatic fibrosis. Cholestasis persisted after the operation and was treated with ursodeoxycholic acid. The patient has now been free of symptoms for 3 years.

Caroli's syndrome should be included in the differential diagnosis of chronic cholestasis of unknown cause. The case also demonstrates the justification, under certain conditions, of aggressive treatment even when there are two different malignancies.