Caroli's disease and syndrome: hepatic and gastrointestinal complications in adult patients

Abstract

Background: Caroli syndrome is a developmental anomaly. The most accepted theory explaining its pathogenesis relates it to malformation of the ductal plaque at different levels of the intrahepatic biliary tree. It is a combination of Caroli's disease and congenital hepatic fibrosis. Objective: To determine the main hepatic and gastrointestinal complications of Caroli's disease and syndrome. Methodology: articles from the period 2017-2022 that addressed the relationship between Caroli disease and syndrome, hepatic and gastrointestinal complications in adult patients were considered. Articles of descriptive, relational, applicative and predictive scope, published in indexed scientific journals were included. Results: Among the main complications of Caroli syndrome are cholangitis, sepsis, liver abscess, cholangiocarcinoma and portal hypertension. After cholangitis occurs, a large number of patients die within 5 to 10 years. That the syndrome could progress to cholangiocarcinoma, which occurs in 7% - 14% of patients. Death is related to liver failure or complications of portal hypertension. Conclusion: Liver transplantation is established as the only definitive treatment available to reverse the symptoms of Caroli syndrome. However, no consensus has been reached on the indication or timing of liver transplantation for patients with Caroli disease.