Liver Transplantation for Caroli Disease
نویسندگان
چکیده
Caroli disease is a rare congenital disorder characterized by multifocal, segmental dilatation of intrahepatic bile ducts. Patients with Caroli disease who have recurrent bouts of biliary infection, particularly those who also have complications related to portal hypertension may require liver transplantation. In liver transplant ward of Shiraz University of Medical Science we had 4 patients with Caroli disease who were transplanted. Herein, we describe the demographic characteristics and post-transplant course of the patients. These patients presented with liver failure, recurrent cholangitis and portal hypertension sequelae unresponsive to medical treatment. The mean age of patients was 24.5 (range: 18-36) years, the mean MELD score was 17.5 (range: 11-23), three patients were female; one was male. All of the patients had good post-transplantation course except for one patient who developed post-operative biliary stricture for whom biliary reconstruction was done.
منابع مشابه
Severe Hepatopulmonary Syndrome in a Child with Caroli Syndrome
Hepatopulmonary Syndrome (HPS) is a potential complication of chronic liver disease and is more commonly seen in the adult population. Caroli Syndrome is a rare inherited disorder characterized by intrahepatic ductal dilation and liver fibrosis that leads to portal hypertension. In children with liver disease, HPS should be considered in the differential diagnosis of prolonged, otherwise unexpl...
متن کاملLaparoscopic hepatectomy for the treatment of Caroli's disease: a case report
Caroli disease is a rare congenital disorder characterized by nonobstructive dilatation of intrahepatic ducts. In cases with symptomatic intrahepatic manifestations, treatment should correspond to the type with hepatic resection for localized disease and transplantation for diffuse forms. If possible, complete resection of the cysts can cure the symptoms and avoid the risk of malignancy. A 66-y...
متن کاملRecurrence of fatty liver disease following liver transplantation for NAFLD-related cirrhosis: Current status and challenges
Non-alcoholic fatty liver disease (NAFLD) is emerging as a major health problem worldwide. NAFLD is a continuum of disease ranging from mild liver steatosis to severe steatohepatitis, which will ultimately lead to end-stage liver disease with high morbidity and mortality rates. This disorder is considered as a silent liver disease. The metabolic syndrome and its components are accounted as the ...
متن کاملLeft-hemihepatectomy as a method of treatment of locally limited Caroli disease.
The study introduces a case of a 51-year old patient with Caroli's disease of left liver lobe. In 2011 the patient was admitted to Clinic of General, Vascular and Transplantation Surgery. She was after first in her life incident of an acute pancreatitis and subsequent ERCP procedure with left hepatic biliary tract drainage. The lady was qualified to left-hemihepatoctomy, which was successfully ...
متن کامل