Choledochal cyst in pregnancy: a therapeutic dilemma

Choledochal cysts occurring in pregnant women represent a diagnostic and therapeutic challenge to a broad spectrum of the medical profession. Not only is the association rare, but the clinical signs and symptoms are obscured by physiological changes that occur during pregnancy. As a result, diagnosis is often delayed until patients present with life-threatening complications.

This report describes three cases of choledochal cysts occurring during pregnancy.

Although the diagnosis was initially missed in two patients, delayed treatment was not associated with an adverse outcome. In a third patient, conservative management was complicated by rupture of the cyst which resulted in fetal loss and a protracted hospital course. Definitive cyst surgery resulted in a good long-term result in all three patients.

Although choledochal cysts rarely occur in pregnancy, clinicians need to be aware of the condition, as delayed or inappropriate therapy may be catastrophic for both mother and child. Once the diagnosis is established, patients should be referred to specialized centers where treatment can be carefully planned, bearing in mind maternal and fetal well-being, as well as the likelihood of cyst-related complications both in the short- and long-term period. Excision with reconstruction is the procedure of choice to treat this type of cyst in nonpregnant patients. In pregnancy, however, a more conservative approach may have to be adopted until the second trimester or after delivery, when the surgical risk is lowest. Elective cesarean section should be undertaken in patients in whom the cyst has not been decompressed so as to avoid the complication of cystic rupture postpartum.