Surgical management of biliary tree cysts

Abstract

Background: Biliary tree cysts represent a genetic and pathological dilatation, focal or extended, of hepatobiliary system. Incidence in western populations is around 1/100.000-1/150.000 and is more frequent in asiatic areas (1/1.000). Todani classification is based on site and morphology of cysts. Acute cholangitis is the main clinical presentation, cancer incidence is around 2,5-16%. Material and Methods: From 2008 to 2018 we observed and surgically treated 8 cases. 1: 63 y.o. male presented with abdominal pain and dilatation of left biliary tree at CT scan. He was treated with left hepatectomy. 2: 72 y.o. woman presented with epigastric pain and fever. At CT scan colliquative lesion of left liver: she underwent left hepatectomy. Histological findings revealed Caroli disease. 3: 53 y.o. man hospedalized for colelitiasis and dilatation of S3 biliary tree. He underwent S3 resection and colecistectomy. 4: 68 y.o. woman admitted for abdominal pain and dilatation of S2-S3 biliary tree. She underwent S2 and S3 resection and cholecistectomy. 5: 66 y.o. woman presented with abdominal pain and weight loss. At CT scan left liver was disomogenous and with dilatation of biliary tree. She was treated with left hepatectomy. Histological findings confirmed Caroli disease associated to cholangiocarcinoma. 6: 30 y.o. woman resented with abdominal pain. CT scan showed Todani disease type II-III: She underwent laparoscopic resection of extrahepatic biliary tree with bilio-digestive anastomosis. Results: 30-days complications was 12,5%. 90-days mortality was 0%. Two years follow-up: 7 patients are alive and in good conditions, patient 5 died for disease progression after 12 months from surgery. Conclusions: In case of unilobar and symptomatic disease surgical resection is treatment of choice. Liver transplant is indicated in bilobar disease. Extrahepatic disease is treated with resection of biliary tree and reconstruction. It’s important to treat these rare diseases in order to avoid risk of malignant developement.