Gallengangs-Zyste

A 50-year-old man presented with jaundice and epigastric pain. He complained of tenderness in the right upper quadrant of the abdomen. Laboratory studies showed AST of 39 U/L (normal 7–40 U/L), ALT 93 U/L (normal 5–45 U/L), GGT 79 U/L (normal 15–70 U/L), and bilirubin 119 μmol/L (normal <19μmol/l).

The pancreatic duct was not dilated. Remains of concrements were found after cleaning the common bile duct, suggesting choledocholithiasis. After ERCP the patient was free of symptoms and laboratory studies began to normalize.

An MRCP showed a large cystic abnormality in the common bile duct with a total length of 4 cm and a maximum diameter of 2.7 cm (C = choledochal cyst, GB = gallbladder). The cyst started at the entrance of the cystic duct in the common bile duct and ended at the level of the head of the pancreas. The intrahepatic duct and the pancreatic duct appeared to be normal.

The patient underwent a choledochal resection with Roux-en-Y hepaticojejunostomy. In the resected specimen a dilated common bile duct was found with fibrotic changes in the wall consistent with a choledochal cyst type I1. There were no signs of active inflammation or malignancy. Most likely the complaints were caused by choledocholithiasis with an associated choledochal cyst.

The choledochal cyst is a relatively uncommon disease of the biliary tree. About 65% of all choledochal cysts are diagnosed in the first decade of life. Presentation in adulthood is uncommon and often triggered by conditions of the choledochal cyst, such as gallstones, pancreatitis, or cholangitis. When a choledochal cyst is suspected, MRI should be performed. Alonso-Lej classified the choledochal cysts into three types.2 This classification has been modified by Todani and coauthors, based on radiographic findings and is generally used.1
There is a well-established association between choledochal cyst and biliary malignancy, especially cholangiocarcinoma. The incidence ranges between 2.5% and 28%. The cause of these malignancies is uncertain, but the survival rates are very poor, regardless of treatment. In view of the high risk of malignancy and the high rate of complications after internal drainage procedures, primary excision with Roux-en-Y hepaticojejunostomy is the treatment of choice for patients with type I choledochal cysts.

References
1 T. Todani, Y. Watanabe and A. Toki et al., Classification of congenital biliary cystic disease: special reference to type lc and lva cysts with primary ductal stricture, J Hepatobiliary Pancreat Surg 10 (2003), pp. 340–344.
2 F. Alonso-Lej, W.B. Rever Jr and D.J. Pessagno, Congenital choledochal cyst, with a report of 2, and an analysis of 94, cases, Int Abstr Surg 108 (1959), pp. 1–30.

Journal of the American College of Surgeons Volume 205, Issue 1, July 2007, Pages 178-179