The management of prenatally diagnosed choledochal cysts.

BACKGROUND: Although congenital biliary tree abnormalities are uncommon in the United States, more are being diagnosed antenatally with improved imaging techniques.

METHODS: To determine the prognosis of prenatally diagnosed biliary disease, the authors reviewed the treatment of 3 children who had biliary cystic lesions found during routine prenatal ultrasonography.

RESULTS: All 3 children were born at term. They had elevated bilirubin levels, and postnatal ultrasound scans confirmed the presence of a biliary cystic mass. They underwent exploration within 2 weeks of life. At operation, 2 children were found to have biliary atresia with a cystic biliary lesion. They underwent Kasai procedures and are doing well at 5 and 9 months of age. The third child was found to have a type I choledochal cyst and malrotation at exploration. He underwent cystectomy with Roux-en-Y hepaticojejunostomy and a Ladd's procedure and is doing well at 3 years of age.

CONCLUSIONS: Prenatally diagnosed biliary cysts represent a different spectrum of disease than those diagnosed later in life. The sequelae of biliary disease start before birth, and early operation may be necessary to achieve a good outcome. Because it is impossible to distinguish between choledochal cysts and biliary atresia on antenatal ultrasound scan or magnetic resonance imaging, children with presumed choledochal cysts should undergo early exploration to rule out potential biliary atresia. Excellent outcome is possible with early operation in the absence of severe associated anomalies.