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 imagi ng techniques. Methods: To determine the prognosis of prenatally diagnosed biliary disease , the authors reviewed the treatment of 3 children who had biliary cystic l esions found during routine prenatal ultrasonography. Results: All 3 children were born at term. They had elevated bilirubin leve ls, and postnatal ultrasound scans confirmed the presence of a biliary cyst ic 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 malrotati on at exploration. He underwent cystectomy with Roux-en-Y hepaticojejunosto my and a Ladd's procedure and is doing well at 3 years of age. Conclusions: Prenatally diagnosed biliary cysts represent a different spect rum 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 choledocha l cysts and biliary atresia on antenatal ultrasound scan or magnetic resona nce imaging, children with presumed choledochal cysts should undergo early exploration to rule out potential biliary atresia. Excellent outcome is pos sible with early operation in the absence of severe associated anomalies. J Pediatr Surg 36:1241-1243. Copyright (C) 2001 by W.B. Saunders Company.