Abstract

Secondary biliary cirrhosis represents a particular form of cirrhosis developed secondary to repeated inflammation produced by the obstruction (partial or total) or narrowing of the extrahepatic bile ducts, leading to periportal fibrosis. Biliary cysts are cystic dilations involving the biliary tree at single or multiple segments of the extrahepatic and intrahepatic bile ducts. Although the curative treat­ment is represented by surgery, patients require a long-term follow-up, because there is a permanent risk of de­velop­ing cholangitis and, in time, even malignancies. We re­port the case of a 13-year-old patient with a history of choledochal cyst operated on at the age of 3 years old and with multiple obstructive gallstones operated on two months before presenting to our service for full as­ses­sment and the establishment of supportive treatment. In ad­di­tion, the histopathological examination after surgery re­vealed histopathological liver changes suggestive of chro­nic cholestatic hepatitis with pre-cirrhotic features. The laboratory findings revealed elevated transaminase levels, cholestasis and hyperbilirubinemia, with negative an­ti­bodies for Ebstein-Barr virus, cytomegalovirus, he­pa­ti­tis C and hepatitis B viruses. Ultrasound examination re­vealed a liver with a micronodular structure, and the tran­sient elastography revealed a rigidity equivalent to stage F4 (METAVIR). We initiated supportive treatment with antibiotics, choleretics and vitamins, with nutritional sup­port and monthly evaluations. The evolution has been pro­gres­sively favorable, with transaminase levels almost in the normal range and cholestasis gradually reducing, with a spectacular return of the weight curve.

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