| Caroli's disease (CD) is a rare congenital disorder characterized by cystic dilatation of the intrahepatic bile ducts. This report describes a patient with cholangiocarcinoma arising in the setting of monolobar CD. In spite of detailed investigations including biliary enteric bypass and endoscopic retrograde cholangiography, the diagnosis of mucinous cholangiocarcinoma (CCA) was not made for almost one year. The presentation, diagnosis and treatment of monolobar CD and the association between monolobar CD and biliary tract cancer are discussed. Hepatic resection is the treatment of choice for monolobar CD. ( info) |
2/66. Septic shock after liver transplantation for Caroli's disease: clinical improvement after treatment with C1-esterase inhibitor. The extent of complement and contact activation is related to outcome in sepsis. A low functional index of their main blocker C1-esterase inhibitor (C1-INH) is considered as a relative deficiency of C1-INH and might contribute to the development of fatal complications in the intensive care unit. The first results of therapeutic intervention with C1-INH concentrate in septic shock are promising. We report on our experience of C1-INH concentrate administration in a young woman with Caroli's disease as ultimate rescue therapy for septic shock with capillary leakage syndrome after combined liver and kidney transplantation. No focus of infection was detectable and thus surgical intervention was not indicated. Antibiotic therapy at that time included vancomycin, tobramycin, meropenem and fluconazol. Hemodynamic stabilization occurred within hours after administration of C1-INH concentrate. Simultaneously a reduction in vasopressor medication was possible and negative fluid balance was achieved. ( info) |
3/66. Clonal chromosomal abnormalities in congenital bile duct dilatation (Caroli's disease). BACKGROUND: Caroli's disease is a rare congenital disorder characterised by cystic dilatation of the intrahepatic bile ducts and an increased risk of cholangiocellular carcinoma. The cause is unknown, but occasional familial clustering suggests that some cases are inherited, in particular when occurring in association with polycystic kidney disease and germline PKD1 gene mutations. To date, no gene responsible for familial isolated Caroli's disease has been identified, and no genetic investigations of liver tissue from patients with Caroli's disease have been reported. PATIENT/METHOD: A liver biopsy specimen from a patient with isolated Caroli's disease, without any signs of cholangiocellular carcinoma, was short term cultured and cytogenetically investigated after G banding with Wright's stain. RESULT: cytogenetic analysis disclosed the karyotype 45-47,XX,der(3)t(3;8)(p23;q13), 2mar[cp6]/46,XX[18]. CONCLUSIONS: The finding of an unbalanced translocation between chromosomes 3 and 8 suggests that loss of distal 3p and/or gain of 8q is of pathogenetic importance in Caroli's disease. Alternatively, structural rearrangements of genes located in 3p23 and 8q13 may be of the essence. These chromosomal breakpoints may also pinpoint the location of genes involved in inherited forms of Caroli's disease not associated with polycystic kidney disease. ( info) |
4/66. Radionuclide scintigraphy in Caroli's disease. Congenital cystic dilatation of the intrahepatic bile ducts involving the major intrahepatic radicles was first described by Caroli and hence named as Caroli's disease. We present here a case in which the only symptom was intermittent pain in the abdomen for last one-and-a-half-year and a radionuclide scan done effectively, not only diagnosed the disease but also the associated cholangitis. ( info) |
5/66. Caroli's disease associated with a gastric diverticulum. Caroli's disease or communicating ectasia of the intrahepatic biliary tree is a rare disease with unknown aetiology. The coexistence of this along with the uncommon condition of a gastric diverticulum has never been reported before. A deficiency in the fibromuscular matrix of both the bile ducts and the gastric wall may explain why these two pathologies may coexist in a single patient. ( info) |
| We describe a case of Caroli syndrome (Caroli's disease and congenital hepatic fibrosis) in a 10-year-old boy with bilobar involvement and numerous renal cysts. liver transplantation offers the only hope for such patients. ( info) |
7/66. Regression of antenatally diagnosed localized Caroli's disease. The authors report on an infant who had a multiloculated cystic lesion located in segment IV of the liver, consistent with Caroli's disease diagnosed, by routine prenatal ultrasound at 25 weeks' gestation, and confirmed by hepatobiliary HIDA scan and computed tomography soon after birth. Because there was no sign of biliary obstruction, the patient was observed initially, with gradual regression of the cysts noted by serial sonograms. Caroli's disease in older children and adults often is associated with recurrent cholangitis and cirrhosis, mandating resection when the disease is unilobar. However, the natural history of Caroli's disease diagnosed in utero is unclear, and a period of observation appears warranted in the asymptomatic patient. ( info) |
8/66. Cholangiocellular carcinoma associated with segmental Caroli's disease. This case report describes the association of cholangiocarcinoma arising in a liver segment with Caroli's disease. We demonstrate the diagnostic difficulties in recognizing the pathogenetic background and outline the indication for surgical treatment. ( info) |
9/66. Improved accuracy in the diagnosis of intrahepatic bile duct ectasia in Caroli's disease by combination of ultrasound and endoscopic retrograde cholangiography. Caroli's disease is characterized by dilatation of the intrahepatic bile ducts. cholangitis, liver cirrhosis and development of a cholangiocarcinoma are possible complications. For optimal therapy, a correct diagnosis of the extent of the disease is mandatory. The present report demonstrates that the combination of endoscopic retrograde cholangiography and ultrasound may lead to a more reliable diagnosis of the extent of Caroli's disease. It is therefore essential to perform ultrasound in all these patients. ( info) |
10/66. Gallstone ileus in Caroli's disease. This report describes a patient with a type II Caroli' s disease who developed a gallstone ileus. Previous hepaticojejunostomy operations were thought to have facilitated the migration of stones into the bowel and consequent gallstone ileus. This complication, which was strongly suspected from the clinical history, was diagnosed by ultrasound examination despite the absence of aberrantly located stones on plain abdominal x-ray. Ultrasound is useful in the diagnostic workup of patients with bowel obstruction to confirm obstruction itself, to assess its level, and possibly to identify the cause. ( info) |