Clinicopathological features of Caroli's disease: An analysis of 5 cases

Objective To investigate the clinical features,pathological features,differential diagnosis,and prognosis of Caroli's disease( CD). Methods A retrospective analysis was performed for the clinicopathological data of 5 parents with CD who were treated in Beijing Tsinghua Chang Gung Hospital from January 2014 to May 2019,and a literature review was also performed. Results There were 3 male and2 female patients,with a mean age of 20 years. All of them attended the hospital due to the presence of intermittent upper abdominal pain,and among these patients,4 had pyrexia,2 had splenomegaly and portal hypertension,1 had gallstones,4 had bilateral renal cysts,and 4 had abnormal hepatic function. Imaging examination showed varying degrees of cystic dilatation of the intrahepatic bile ducts which communicated with the biliary tree,and fibrous bands were observed in these cysts. Pathological examination showed intrahepatic bile duct dilatation with fibrous tissue proliferation and chronic inflammatory cell infiltration on the bile duct wall,and fibrovascular bundles were observed in the bile duct cavity. In addition,fibrosis and proliferation of the portal area and abnormal small bile ducts were observed in type Ⅱ CD. Conclusion CD is often seen in children and adolescents,and its clinical manifestations lack specificity. A confirmed diagnosis of CD relies on imaging and pathological examinations. Histopathological features of this disease include cystic dilatation of the intrahepatic bile ducts communicating with the biliary tree and the bands consisting of fibrous tissue and vessels in these cysts.