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

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

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 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

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

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

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

BACKGROUND: Cystic liver disease is a rare disease entity, but its surgical management can vary from partial excision to hepatic lobectomy. This study was conducted to evaluate proper surgical management of cystic liver disease. METHODS: The authors reviewed the medical records of 26 patients who underwent operative management for cystic liver disease, consisting of 15 cases of simple cyst, 7 cases of biliary cystadenoma, 1 case of biliary cystadenocarcinoma, 1 case of polycystic disease and 2 cases of Caroli's disease. RESULTS: The 26 patients had a mean age of 57, with a male to female ratio of 1:4. Patients' main symptoms were right upper quadrant pain or discomfort, and epigastric pain. Their main physical findings were palpable mass and direct tenderness, but 23% of patients were aymptomatic and 35% had no physical findings. Preoperative diagnosis was made with abdominal ultrasound and computed tomogram. Intervening septum was identified in 27% of simple cysts and 100% of biliary cystadenoma. However neither diagnostic tool has 100% diagnostic accuracy. All cysts except polycystic disease were located at either both hepatic lobes but more right lobe in simple cysts. The mean size of all cysts were 10.2cm ranging from 2-to-22cm without any difference among simple cysts, cystadenomas, and cystadenocarcinoma. Operative methods for simple cysts were partial cystectomy with marsulpialization, total cystectomy and hepatic segmentectomy in 87% of cases, of which 2 cases were performed by laparoscopic approach. However partial cystectomy was confined to unilocular simple cyst. There was no recurrence of simple cyst postoperatively. Biliary cystadenoma were managed by total cyst excision, hepatic segmentectomy or lobectomy in 86% of cases, but one cystadenoma was managed by partial cystectomy with marsulpialization had a persisting cyst. One patient with polycystic liver disease died because of overwhelming sepsis with postoperative acute renal failure. CONCLUSION: For unilocular simple cyst, partial cystectomy with marsulpialization can be an acceptable surgical procedure, even with a laparoscopic approach. But for biliary cystadenoma, the cyst should be totally removed at least to prevent recurrence and transformation to cystadenocarcinoma. Therefore, correct preoperative diagnosis is a prerequisite for appropriate selection of operative methods.
Acute Kidney Injury ; Caroli Disease ; Cystadenocarcinoma ; Cystadenoma ; Cystectomy ; Diagnosis ; Female ; Humans ; Liver Diseases* ; Liver* ; Male ; Mastectomy, Segmental ; Medical Records ; Rare Diseases ; Recurrence ; Sepsis ; Ultrasonography

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