Type IV choledochal cyst with polycystic kidney disease: a case report
- PDF / 1,133,259 Bytes
- 5 Pages / 595.276 x 790.866 pts Page_size
- 11 Downloads / 170 Views
CASE REPORT
Open Access
Type IV choledochal cyst with polycystic kidney disease: a case report Yuxin He, Zhuwen Yu and Weichang Chen*
Abstract Background: Choledochal cysts are divided into 5 types. Physicians believe that Caroli disease (which refers to type V biliary cysts) is a special type of biliary cyst caused by a mutation in the PKHD1 gene and is associated with autosomal recessive polycystic kidney disease (ARPKD). There is currently no clear association between other types of choledochal cysts and polycystic kidney disease. Case presentation: We report a 65-year-old male patient with jaundice, decreased appetite, and itchy skin. His biochemistry test results indicated obstructive jaundice disease. Cross-sectional imaging showed a type IVA choledochal cyst accompanied by autosomal dominant polycystic kidney disease (ADPKD). Due to economic difficulties, the patient achieved percutaneous transhepatic cholangial drainage (PTCD) instead of surgery. Conclusion: To our knowledge, this is the second case report of the coexistence of type IVA choledochal cysts and ADPKD. We conclude that it is vital to be aware that the above condition is a possibility. This case report will aid earlier diagnosis and management and possibly prevent further damage to liver and kidney function. Keywords: Choledochal cyst, Polycystic kidney disease, Caroli disease
Background Choledochal cysts are cystic dilations that may occur in any part of the bile duct between the liver and the duodenum. Alonso-lej first classified choledochal cysts in 1959 [1], and Todani expanded the classification system in 1977 by dividing choledochal cysts into 5 types [2]. Type I cysts are subclassified into 3 types. Type IA cysts are marked cystic dilations of the entire extrahepatic biliary tree that do not involve the intrahepatic ducts. Type IB cysts are focal, segmental dilations of the extrahepatic bile duct. Type IC cysts are smooth fusiform dilations of the entire extrahepatic bile duct. Type II cysts are discrete diverticula of the extrahepatic duct with narrow stalk connections to the common bile duct. Type III cysts are choledochoceles that are located in the duodenal wall. Type IVA cysts are multiple intrahepatic and * Correspondence: [email protected] Department of Gastroenterology, The First Affiliated Hospital of Soochow University, Soochow University, 188 Shizi Road, Suzhou, Jiangsu Province, China
extrahepatic dilations. Intrahepatic duct dilation can be cystic, fusiform, or irregular. Type IVB cysts are multiple dilatations that are confined only to the extrahepatic bile duct. Type V cysts, which are also called Caroli disease, show single or multiple dilatations of the intrahepatic bile duct. The aetiology of choledochal cysts is complex. At present, there are several assumed causes, including genetic factors, abnormal pancreaticobiliary confluence (pancreaticobiliary maljunction, PBM), gastrointestinal neuroendocrine factors, and abnormal proliferation of the bile duct epithelium. Caroli disease is an autosomal recessive genetic dis
Data Loading...
