Caroli's disease complicating pregnancy

We describe two pregnancies in the same patient with Caroli's disease (congenital dilatation of the intrahepatic bile ducts). The first pregnancy was uneventful concerning the maternal Caroli's disease. The second pregnancy highlights the serious and potentially lethal complications of an acute episode to both the fetus and mother. The patient had acute ascending cholangitis, disseminated intravascular coagulopathy, and septic shock. Fetal distress necessitated delivery by cesarean section. The mother's postoperative course required prolonged critical care and interdisciplinary consultation. Both mother and neonate survived. We present the first report of Caroli's disease in pregnancy and review implications to the pregnant patient.