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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