Adenocarcinoma in a choledochal cyst during pregnancy: a case report and guidelines for management

Choledochal cysts, although more common in females than males, have only rarely been encountered in association with pregnancy. We report a patient with a large choledochal cyst that was excised at the time of cesarean section. Pathologically, the cyst wall was found to contain a focus of adenocarcinoma. The development of carcinoma is a recognized risk in patients with choledochal cysts that mandates cyst excision, rather than bypass, whenever possible. The literature regarding choledochal cysts in pregnancy is reviewed. Pregnant patients with choledochal cysts require careful management so as to avoid cyst rupture; and cesarean section is preferable to normal vaginal delivery in most cases. Although the preferred management of a choledochal cyst is excision and Roux-en-Y reconstruction, this may have to be deferred until after delivery, depending on gestation age, because of the risk of fetal mortality and maternal morbidity that is associated with this procedure.