Among complications of choledochal cysts, malignant transformation is most concerning and management of choledochal cyst now includes complete cyst excision, whenever possible. In cases of choledochal cyst associated with pancreaticobiliary maljunction like our case, cholecystectomy along with the resection of dilatated bile duct and the biliary diversion are generally performed. However, incomplete cyst excision can result in malignant transformation within the remnant cyst. We present the case of cholangiocarcinoma arising from remnant intrapancreatic cyst 15 years after choledochal cyst excision in a patient with Todani type 1 choledochal cyst associated with pancreaticobiliary maljunction. We learn from the case that a careful long-term follow-up is needed in patients with choledochal cysts if residual cyst is remained after excision.
Bile Ducts ; Cholangiocarcinoma* ; Cholecystectomy ; Choledochal Cyst* ; Follow-Up Studies ; Humans ; Pancreatic Cyst

Choledochal cyst has only rarely been encountered in association with pregnancy. The clinical manifestations are nonspecific and variable that makes it difficult to differentiate from physiologic changes in pregnancy. Consequently, diagnosis is often delayed until patients present with life-threatening complications. During pregnancy, symptoms of choledochal cyst may be developed by hormonal changes and the enlarged uterus. Because of the risk of fetal mortality and maternal morbidity, definitive surgical treatment should be delayed and step-by-step management should be carefully implemented to avoid complication until delivery. Herein, we report a case of enlarged, symptomatic choledochal cyst that developed in a 26-year-old pregnant woman. The temporal relationship between pregnancy and symptom development, as well as the biliary sludge formation in the enlarged cyst, suggest that the choledochal cyst was influenced by pregnancy. In order to buy time for fetal maturation, endoscopic ultrasonography-guided choledochoduodenostomy was performed for biliary decompression as a bridge to surgical excision.
Adult ; Bile ; Choledochal Cyst* ; Choledochostomy* ; Decompression ; Diagnosis ; Endosonography ; Female ; Fetal Mortality ; Humans ; Pregnancy* ; Pregnant Women ; Uterus