We present a case of a young woman, who underwent surgery for congenital bile duct cyst in adulthood. Due to location of the cysts it was impossible to completely remove it.
The cyst was partially resected and it was performed cyst-jejunal anastomosis (CJA) to drain of bile. After operation the patient was 4 years without any problems.
After this interval appeared pain in the right upper quadrant and subicterus with laboratory evidence of cholestasis. The patient suffered from recurrent cholangitis due to which a number of diagnostic tests and interventions were performed.
Used imaging metods did not demonstrate an unequivocal cause of problems, therefore subsequently surgical revision was performed and the in-traoperative ultrasonography demonstrated tumor in anastomosis. The finding was inoperable due to inappropriate location.
In the following period liver and lymphnode metastases appeared and patient died five months after diagnosis of malignancy. This relatively rare case illustrates an increased risk of malignant transformation of congenital malformations of the biliary tract.
There are very few documented metachronous cancer after resection of the biliary tract in the literature.