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

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

Three siblings with congenital dilatation of the intrahepatic bile ducts (Caroli's disease) are presented. Bile duct pathology was associated with congenital hepatic fibrosis and polycystic renal disease in all three patients. On color Doppler imaging (CD imaingl, multiple small color Doppler signals were observed in or near the vascular radicles within the dilated bile ducts, besides other well-known sonographic findings such as bile duct dilatations, biliary calculi. Dopper frequency spectral analysis confirmed all these color Doppler signals as arterial origin in all patients, showing pulsatile wave pattern. Although portal venous radicles are well known in conventional sonograms or computed tomotraphy(CT), continuous wave patterns were not detected in all patients. In addition to previously reported sonographic findings about Caroli's disease, color Doppler signals showing arterial wave pattern in or around the portal venous radicles within dilated duets are another helpful diagnostic criteria and these findings are easily depicted on routine sonograms with color mapping.
Bile Ducts* ; Bile Ducts, Intrahepatic ; Bile* ; Caroli Disease* ; Dilatation ; Fibrosis ; Gallstones ; Humans ; Pathology ; Polycystic Kidney Diseases ; Siblings ; Ultrasonography

No abstract available.
Adult ; Bile Duct Neoplasms/*pathology ; Bile Ducts, Intrahepatic/*pathology/radiography ; Caroli Disease/*pathology ; Cholangiocarcinoma/*pathology ; Female ; Humans ; Magnetic Resonance Imaging ; Positron-Emission Tomography ; 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 syndrome is a rare congenital disorder that involves intrahepatic bile duct ectasia and congenital hepatic fibrosis, frequently seen with concomitant autosomal recessive polycystic kidney disease (ARPKD). Literature on infants with ARPKD is rare. Here, we present a case of a two month old boy who was diagnosed with Caroli's syndrome and ARPKD.
Polycystic Kidney, Autosomal Recessive/complications/*diagnosis/pathology ; Male ; Kidney/ultrasonography ; Infant ; Humans ; Hepatic Duct, Common/pathology/ultrasonography ; Caroli Disease/complications/*diagnosis/pathology

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

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

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