Regression of antenatally diagnosed localized Caroli's disease

The authors report on an infant who had a multiloculated cystic lesion located in segment IV of the liver, consistent with Caroli's disease diagnosed, by routine prenatal ultrasound at 25 weeks' gestation, and confirmed by hepatobiliary HIDA scan and computed tomography soon after birth. Because there was no sign of biliary obstruction, the patient was observed initially, with gradual regression of the cysts noted by serial sonograms. Caroli's disease in older children and adults often is associated with recurrent cholangitis and cirrhosis, mandating resection when the disease is unilobar. However, the natural history of Caroli's disease diagnosed in utero is unclear, and a period of observation appears warranted in the asymptomatic patient.