The diagnosis of cystic liver disease has made great progress with the advent of enhanced imaging techniques. At the same time, its management has gradually improved over the past few decades, providing the basis for the development of appropriate diagnostic and treatment guidelines. To this end, the European Association for the Study of the Liver has developed clinical guidelines for the diagnosis and treatment of non-infectious cystic liver disease. This guideline put forward recommendations based on an in-depth review of the relevant literature for addressing clinical issues, including the diagnosis and treament of hepatic cysts, hepatic mucocystic tumors, biliary hamartomas, polycystic liver disease, Caroli disease or Caroli syndrome, biliary hamartomas, and peribiliary cyst.
Humans ; Liver Diseases/pathology* ; Cysts/pathology* ; Caroli Disease/diagnosis* ; Liver Neoplasms/therapy* ; Hamartoma

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-year-old woman presented to Wuxi Xishan People's Hospital with recurrent intermittent upper quadrant abdominal pain. Further examinations suggested the diagnosis of Caroli disease limited to the left hepatic lobe. She underwent laparoscopic hepatectomy. Pathological examination confirmed the diagnosis of Caroli disease, and no malignancy was found. There were no immediate complications and no long-term complications after one and one-half years of follow-up. Laparoscopic hepatectomy could be a feasible, safe treatment option for localized Caroli disease.
Abdominal Pain ; Aged ; Caroli Disease* ; Cholecystectomy ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Diagnosis ; Dilatation ; Female ; Follow-Up Studies ; Hepatectomy* ; Humans ; Laparoscopy

Caroli's disease is a rare autosomal-recessive disorder caused by malformation of the ductal plate during embryonic development. Although it is present at birth, Caroli's disease is typically not diagnosed until between the second and fourth decades of life, as it was in the present patient. Here we report a rare case of Caroli's disease limited to one liver segment, which was initially misdiagnosed as an intraductal papillary neoplasm of the bile duct. The asymptomatic patient was treated with liver segmentectomy.
Adult ; Bile Duct Neoplasms/diagnosis/pathology ; Bile Ducts, Intrahepatic ; Caroli Disease/*diagnosis/pathology ; Diagnostic Errors ; Humans ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

No abstract available.
Caroli Disease/complications/*diagnosis ; Child ; Cholangiopancreatography, Magnetic Resonance ; Creatinine/blood ; Female ; Humans ; Liver/pathology ; Liver Transplantation ; Polycystic Kidney, Autosomal Recessive/complications/*diagnosis/ultrasonography ; Tomography, X-Ray Computed

Adenocarcinoma ; metabolism ; pathology ; Adenoma ; pathology ; Bile Duct Neoplasms ; metabolism ; pathology ; Bile Ducts, Intrahepatic ; CA-19-9 Antigen ; metabolism ; Cadherins ; metabolism ; Caroli Disease ; pathology ; Cholangiocarcinoma ; pathology ; Cystadenocarcinoma ; metabolism ; pathology ; Cystadenoma ; metabolism ; pathology ; Cysts ; pathology ; Diagnosis, Differential ; Hamartoma ; pathology ; Humans ; Keratin-19 ; metabolism ; Keratin-20 ; metabolism ; Keratin-7 ; metabolism ; Liver Diseases ; pathology

No abstract available.
Adult ; Bile Ducts, Intrahepatic/pathology ; Caroli Disease/complications/*diagnosis/pathology ; Gallstones/complications/*diagnosis/radiography ; Humans ; Male ; Tomography, X-Ray Computed

Caroli's syndrome is a rare autosomal recessive hereditary disease. Here a case of Caroli's syndrome associated with medullary sponge kidney was reported. The patient was a 2-years and 10 months-old boy. He presented with hepatosplenomegaly. Fever, abdominal pain or jaundice was not found. The imaging examination showed intrahepatic bile duct dilation, splenomegaly, medullary sponge kidney and nephrocalcinosis. After introduction of the case, this paper reviewed the clinical characteristics, diagnosis and treatment of Caroli's syndrome.
Caroli Disease ; diagnosis ; etiology ; therapy ; Diagnosis, Differential ; Humans

Adult ; Bile Ducts, Intrahepatic ; diagnostic imaging ; pathology ; Biopsy, Needle ; Caroli Disease ; complications ; diagnosis ; pathology ; Child ; Diagnosis, Differential ; Female ; Humans ; Liver ; diagnostic imaging ; pathology ; Liver Cirrhosis ; complications ; congenital ; diagnosis ; pathology ; Male ; Spleen ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

Caroli's disease is a rare congenital hepatobiliary disease characterized by multifocal segmental dilatation of intrahepatic bile ducts affecting all or parts of the liver. Two forms of Caroli's disease are described, the pure form and that associated with periportal fibrosis. The disease may diffusely affect the liver or be localized to one lobe or segment. Less than 20% of all reported cases of Caroli's disease are monolobar type. We report a case of simple type Caroli's disease confined to right lobe of the liver in a 22 year old man. He was admitted due to right upper abdominal pain and diagnosed by magnetic resonance cholangiopancreatography. He was treated with right hepatic lobectomy and recovered completely.
Adult ; Bile Ducts, Intrahepatic/pathology ; Caroli Disease/diagnosis/pathology/*surgery ; Common Bile Duct/surgery ; Diagnosis, Differential ; Hepatectomy ; Humans ; Liver/*pathology ; Magnetic Resonance Imaging ; Male ; Tomography, X-Ray Computed

Caroli's disease is defined as a communication between congenital cystic dilatation of the bile duct and the biliary system. Caroli's disease accompanied with autosomal dominant polycystic kidney disease is a very rare finding and the differential diagnosis between Caroli's disease with polycystic kidney disease and a polycystic liver is very important. A 67-year-old male patient was admitted to our hospital for hematochezia, and he underwent hemodialysis for chronic renal failure due to his autosomal dominant polycystic kidney disease. On colonoscopy, ulcero-infiltrative tumor was observed in the sigmoid colon. It was diagnosed as adenocarcinoma on tissue biopsy. On abdominal computerized tomography and magnetic resonance cholangiopancreatography, a number of simple cysts was observed in both kidneys and we were able to identify the communication between the dilated intrahepatic ducts and the intrahepatic cystic lesions, and so we diagnosed this patient as having Caroli's disease.
Adenocarcinoma ; Aged ; Bile Ducts ; Biliary Tract ; Biopsy ; Caroli Disease* ; Cholangiopancreatography, Magnetic Resonance ; Colon* ; Colon, Sigmoid ; Colonic Neoplasms* ; Colonoscopy ; Diagnosis, Differential ; Dilatation ; Gastrointestinal Hemorrhage ; Humans ; Kidney ; Kidney Failure, Chronic ; Liver ; Male ; Polycystic Kidney Diseases* ; Polycystic Kidney, Autosomal Dominant ; Renal Dialysis