1/7. 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.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
2/7. Caroli's disease and congenital hepatic fibrosis associated with polycystic kidney disease. A case presenting with acute focal bacterial nephritis.Congenital cystic dilatation of the intrahepatic biliary ducts (Caroli's disease), until recently, has been infrequently recognized. It is often associated with autosomal recessive polycystic kidney disease (ARPKD) and congenital hepatic fibrosis (CHF). We hereby report a case with Caroli's disease, polycystic kidney disease (PKD), and CHF: This 24-year-old female patient initially presented with acute bacterial nephritis (ABN). Renal ultrasonography revealed bilateral enlarged kidneys with multiple cysts. Because her parents showed no renal cyst on ultrasonographic examination, she received further studies. Abdominal ultrasonography showed cystic dilatation of the biliary tree. Computed tomography (CT) with meglumine lotroxinate (biliscopin) infusion study and hepatobiliary scintigraphy confirmed the diagnosis of Caroli's disease. Liver biopsy revealed CHF: The radiographic and scintigraphic pictures are hereby illustrated and CT with biliscopin infusion study is emphasized. We conclude that if radiologic evidence of renal cystic lesions is absent in the parents of patients with PKD, the coexistence of Caroli's disease and CHF should be considered. The clinical pictures of ABN in this patient are also discussed. As far as we know, this is the first reported case of ABN in a patient with PKD and Caroli's disease, and it showed good response to antibiotic therapy.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
3/7. An atypical course of Caroli's disease in a renal transplant patient--case report and review of the literature.This is a rare case of Caroli's disease, diagnosed following renal transplantation in a patient with autosomal recessive polycystic kidneys. Despite advanced cystic transformation of the biliary tree with striking architectural changes, there was no evidence of portal hypertension or hepatic fibrosis. Moreover, the patient did not suffer a single episode of cholangitis, a most interesting feature of this case. Her clinical course was punctuated by repeated episodes of gastrointestinal and urinary tract infections with resistant organisms; but fortunately, she had no evidence of septicemia. Recurrent salmonella gastroenteritis indicated a chronic carrier state with the dilated bile ducts possibly acting as a potential reservoir. This has significant implications considering the immune suppression associated with renal transplantation. In general, Caroli's disease is rare. Therefore, a high index of suspicion for the diagnosis of Caroli's disease is warranted especially in patients with ARPKD or ADPKD. Once confirmed, affected patients with end-stage renal disease such as our patient, should ideally undergo combined liver-kidney transplantation.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
4/7. Caroli's disease.Carolis disease is a rare communicating segmental or diffuse dilatation of the intrahepatic biliary tree. cholangitis, liver cirrhosis and cholangiocarcinoma are its potential complications. A case of Carolis disease in a boy of 6 years with bilobal involvement presenting with intermittent abdominal pain, fever and hepatomegaly is reported here.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
5/7. Noninvasive diagnosis of Caroli syndrome associated with congenital hepatic fibrosis using hepatobiliary scintigraphy.A patient who was studied for portal hypertension had an enlarged liver and multiple hepatic cysts on abdominal CT scan. He underwent hepatobiliary scintigraphy using Tc-99m mebrofenin which documented communication of the cysts with the main biliary tree and allowed a noninvasive diagnosis of Caroli syndrome associated with congenital hepatic fibrosis.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
6/7. liver transplantation in patients with Caroli's disease and recurrent cholangitis.Caroli's disease is an uncommon congenital disorder of the intrahepatic biliary tree. It is characterized by multiple and segmental dilatations of the bile ducts. The clinical course of Caroli's disease is often complicated by recurrent episodes of bacterial cholangitis that seriously impair the patient's quality of life. Despite wide spectrum antimicrobial agents, medical treatment of cholangitis is frequently unsuccessful in patients with Caroli's disease due to the persistence of bacteria in dilatated bile ducts. Other therapies, including internal or external biliary drainages and various surgical or endoscopic procedures, have been used in the treatment of Caroli's disease, with poor results. There are no previous reports in the literature of liver transplantation for recurrent cholangitis in patients with Caroli's disease. We present two such cases, in which cholangitis is resolved.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
7/7. Spontaneous rupture of a bile duct and its endoscopic management in a patient with Caroli's syndrome.Caroli's syndrome is a condition of cystic dilation of intrahepatic bile ducts that communicate with the extrahepatic biliary tree. patients with Caroli's syndrome are prone to develop several complications. These include bacterial cholangitis, biliary sludge, calculi, and cholangiocarcinoma. We describe an adult patient with Caroli's syndrome in whom spontaneous rupture of a bile duct developed with consequent biliary peritonitis, which was successfully managed with endoscopic stent placement.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |