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Caroli disease is a relatively rare genetic disease, also known as congenital intrahepatic cystic cholangiectasis, which is mainly manifested as non-obstructive segmental dilation of large, intrahepatic bile ducts, which is manifested as cysts in imaging and histopathological examination. The pathogenesis of Caroli disease is still unclear, and it is mainly believed to be related to PKHD1 gene mutation. Mutations in this gene often lead to autosomal recessive polycystic kidney disease (ARPKD), so Caroli disease is commonly associated with polycystic kidney disease. Caroli disease usually develops during adolescence and is characterized by recurrent cholangitis, which is diagnosed mainly by imaging. This article reviews the progress of diagnosis and treatment of Caroli disease by reading related literature.
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Objective:To study the safety and feasibility of laparoscopic surgery for treatment of hepaticojejunostomy strictures after cholangiectasis surgery in children.Methods:The clinical data of 12 children was retrospectively analysed. There were 5 males and 7 females, aged 4 (range 0.45 to 9.00) years old, who developed hepaticojejunostomy strictures after cholangiectasis surgery and underwent reoperative treatment at Anhui Provincial Children's Hospital from January 2013 to January 2021. These patients were divided into the laparoscopic surgery group ( n=5) and the open surgery group ( n=7) based on the mode of reoperation. The children were followed-up by outpatient review and the relevant clinical data of the children in the 2 groups was analyzed. Results:The reoperations were completed successfully in the 2 groups. The maximum preoperative dilated common hepatic duct diameter was significantly larger in the laparoscopic group (1.26±0.23) cm than the open group (0.64±0.19) cm ( P<0.05). The alkaline phosphatase and glutamyltransferase levels in the laparoscopic surgery group were significantly lower before the operation (all P<0.05), and the total bilirubin, direct bilirubin, alkaline phosphatase, and glutamyltransferase levels were significantly lower in the laparotomy group than before the operations (all P<0.05). In the laparoscopic group, the time of the reoperations, postoperative hospital stay, and blood loss were 268(117, 340) min, (9.0±2.9) d and (14.0±5.5) ml, respectively, while those in the open group were 180(150, 205) min, (9.7±3.4) d and (13.3±2.6) ml, respectively. There were no significant differences between the two groups (all P>0.05). On follow-up, all children were well except for one child who showed mild elevation levels of alanine aminotransferase and aspartate aminotransferase. Conclusion:Laparoscopic surgery for hepaticojejunostomy strictures after cholangiectasis surgery in children was safe and feasible. Its curative effect was no less than that of open surgery.
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ObjectiveTo review the etiology and classification of cholangiectasia. MethodThe clinical data of 1098 patients with cholangiectasia treated from January 2000 to December 2009 were retrospectively analyzed. Results For the 1098 patients, 69 patients (6.3％) had congenital choledochal cyst, and 1029 patients (93.7％) had secondary cholangiectasia which were secondary to 22 diseases, The top 5 of the etiological diseases were bile duct stones (366 patients, 33.3 ％), pancreatic head carcinoma (137 patients, 12.5％), peri-ampullary carcinoma (122 patients, 11.1％), cholangiocarcinoma (68 patients,6.2％),and chronic pancreatitis or pancreatic head cyst (62 patients,5.6 ％). ConclusionsCholangiectasia can be divided into two major categories (congenital and secondary). Congenital choledochal cyst accounted for 6.0％, secondary cholangiectasia accounted for 94 ％.The most common etiologies were bile duct stones, pancreatic head carcinoma and peri-ampullar carcinoma.
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Objective To probe the significance of ultrasonographic characteristics of cholangiectasis in differential diagnosis of the diseases of bile duct obstruction (DBDO) .Methods A total of 164 patients with DBDO were divided into 4 groups:bile duct calculi (n=52) ,cholangiocarcinoma (n=56) ,cystic dilatation of biliary duct (n=32) and bile duct papilloma (n=24) .The ultrasonographic characteristics of cholangiectasis were classified as regular type,abnormal type,cystic dilated type and bile lake type.The average value of the largest diameter of the bile duct (DBD) and the percentage of each type of the cholangiectasis of each group were compared.Results The accuracy of ultrasound for the diagnosis of 164 DBDO was 98.17% (161/164) .DBD of both bile ducts of calculi and cholangiocarcinoma were different from that of cystic dilatation of biliary duct and bile duct papilloma,respectively (P＜0.01) ,as well as between cystic dilatation of biliary duct and bile duct papilloma (P＜0.01) .The percentage of regular type of cholangiectasis in bile duct calculi (65.38%,34/52) ,abnormal type in cholangiocarcinoma (83.93%,47/56) ,cystic dilated type in cystic dilatation of biliary duct (81.25%,26/32) and bile lake type in bile duct papilloma (83.33%,20/24) was different from those of others,respectively (P＜0.01) .Conclusion Different ultrasonographic characteristics of cholangiectasis has important clinical significance in the differential diagnosis of DBDO.
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Objective To study the imaging value in diagnosis of congenital cholangiectasis.Methods The clinical and imaging (CT and ultrasonics) data of 6 patients with congenital cholangiectasis were restrospectively analyzed with literature review.Results According to Todani's classifications of cholangiectasis,there were type I in one case,CT showed cystic hypodense shadow with thin and smooth wall;type IV in 4 cases,CT showed cystic or fusiform extension of intra-and extra-hepatic bile ducts;type V in one case,CT showed cystic extension of intra-hepatic bile ducts,and the central spot enhancement could be seen on contrast-enhanced CT scan.6 cases underwent ultrasonic examinations,ultrasound showed extension of intra-and extra-hepatic bile ducts in 6 cases,choledochal cyst in one.4 cases suspected with congenital cholangiectasis,and misdiagnosed in one.In company with cholecystitis and cholelithiasis in one,biliary carcinoma in one and cirrhosis in one.Conclusion CT and US are of important value in diagnosis of congenital cholangiectasis.