Surgery is the only effective treatment for congenital choledochal cysts, as it allows for the resection of the cysts, the complete relief of cholangitis, and the prevention of canceration of cysts. The key elements of surgery for central choledochal cysts involve the cysts resection, bile-intestinal anastomosis, and biliopancreatic diversion. The difficulty in operating on central choledochal cysts lies in the rational decision making and effective management of cysts in the hilar and pancreatic regions. Depending on the type of central choledochal cysts with different anatomical patterns, a reasonable and feasible individualized surgical management strategy can be established to effectively avoid adverse therapeutic consequences such as postoperative biliary leakage, cholangio-intestinal anastomotic stricture, residual choledochal cysts and its carcinogenesis.
A complex mechanism of reduced number of bile duct innervating ganglion cells, smooth muscle distribution, foregut duplications, and abnormal pancreaticobiliary duct junction, which occurs during embryonic development and after birth in a genetic context, can lead to pathological congenital biliary dilatation. As a precancerous lesion of the biliary system, irrational treatment of congenital biliary dilatation will further increase the risk of malignancy in patients. By understanding the causes, pathological features, and limitations of early detection techniques of malignant tumor secondary to congenital biliary dilatation is helpful to clarify the key points in the management of congenital biliary dilatation, reduce the incidence of postoperative adverse treatment events and avoid the medical risk of secondary malignancy.