Objective To investigate the diagnosis and treatment of congenital choledochal cysts(CCC) in children. MethodsThe manifestation, auxiliary examination, mode of operation and effect of 42 patients with CCC in children from Jan., 1980 to June, 1999 were analyzed retrospectively.Results The patients with the triad of jaundice, an abdominal mass, and pain was 38.1% among the 42 patients. B-ultrasonic diagnosis was made with a correct diagnostic rate of 95.2%. The effective rate of internal drainage was significantly lower than that of resection of the cyst(χ2=19.36, P＜0.001) while the reoperation rate and incidence of carcinoma of internal drainage were higher than those of resection of the cyst(χ2=11.59, P＜0.001 and χ2=4.97, P＜0.05). Conclusion B-ultrasonic diagnosis is recommended as the first examination method. Internal drainage should be abandoned. Resection of the cyst with Roux-Y hepaticojejunostomy is recommended as the treatment of choice on extrahepatic cholangiectasis. Liver transplantation is a reasonable choice to treat the diffuse intrahepatic cholangiectasis.
Objective To investigate the value of magnetic resonance (MR) imaging for diagnosing typeⅠ congenital choledochocele and its complications. Methods The MR imaging data of 13 cases with proved typeⅠ congenital choledochocele associated with complications were retrospectively reviewed and compared with operative findings. MR imaging sequences included axial T2W and T1W plain scan, true-FISP coronal images, 2D-MRCP, and Gd-enhanced T1W images. Results All patients had cystic dilatation of the common bile ducts to various degrees. In 6 patients complicated with stone and infection, the bile duct showed uniform wall thickening with marked enhancement, and calculus were depicted within the duct lumen with dilatation of the proximal biliary duct. In 7 cases complicated with carcinoma of biliary duct, a polypoid soft tissue mass or nodule was seen inside the ductal lumen in 3 cases, or the duct wall was irregularly thickened in 4 patients. Six cases received curative operation, but one patient with extensive local infiltration, vascular encasement and lymphadenopathy had only palliative treatment. MR imaging observations were verified by surgery findings in all 13 patients.Conclusion MR imaging is very valuable not only in diagnosing typeⅠ congenital choledochocele, but also in revealing its complications.
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.
Choledochal cysts are characterized by single or multiple cystic dilatations of the intrahepatic and / or extrahepatic biliary ducts. The typical presentation of this condition is non-specific. Clinicians must have a high clinical suspicion of choledochal cysts while investigating patients with jaundice, abdominal pain, and abdominal mass. There are multiple classifications for choledochal cysts . The Todani classification system is the most widely used in clinical practice. Based on clinical practice and thinking, we established a new “three regions and five types” classification system on the basis of Todani classification to guide clinical work, but further verification is needed. Surgery is the mainstay of treatment for choledochal cysts and the approach depends on the cyst type and the extent of hepatobiliary pathology. The principles of treatment include complete excision of the cyst and restoration of biliary-intestinal continuity. In view of the risk of biliary malignancy continues to be high after surgery, long-term follow-up is strongly recommended.