Diffuse form of Caroli's disease: therapeutical approach in a female patient with recurrent cholangitis

A 49-year old female was admitted to the 3rd Surgical Clinic Cluj with clinical signs of cholangitis. She had had these symptoms for 30 years and in 2007 she was diagnosed as suffering from a diffuse form of Caroli's disease. On admission, a biological syndrome of cholestasis was noticed, associated with an inflammatory syndrome and hepatocytolysis. The imaging examinations confirmed the presence of bilateral intrahepatic cysts communicating with the biliary tree and intrahepatic lithiasis. Surgery was performed with left lobectomy, cholecystectomy, lavage of the right biliary tree and single loop cholangio-jejunal Roux-en-Y anastomosis. The patient had a favorable postoperative evolution and was discharged on the 7th day. The optimal therapeutic solution for this patient would have been a liver transplantation. However, given the emergency presentation, the surgery choice was to treat the present complications, namely the structural damage in the left lobe, the microabcesses at this level, the intrahepatic lithiasis and cholangitis. Caroli's disease, due to its complications, may impose to the surgeon to choose between different therapeutical strategies before liver transplantation.