Choledochal cyst is a rare but remediable cause of neonatal cholestasis. This uncommon biliary malformation, although thought to be a congenital lesion, has seldom been diagnosed in the first month of life. We describe a 3-week-old infant with cholestasis caused by a choledochal cyst and associated with biliary cirrhosis.
Report of a Case.—A female infant was admitted to the hospital at 3 weeks of age for evaluation of conjugated hyperbilirubinemia. A 2,600-g newborn, she was noted to be jaundiced on the first day of life. The total serum bilirubin level was 10.5 mg/dL; at 7 days of age, it was 6.5 mg/dL. During the first three weeks of life, the infant gained 340 g, but she remained jaundiced and had pale stools.
She was a small, active, icteric infant with a firm liver that was palpable 3 cm below the right costal margin (span, 5 cm) and a
ORENSTEIN SR, WHITINGTON PF. Choledochal Cyst Resulting in Congenital Cirrhosis. Am J Dis Child. 1982;136(11):1025-1027. doi:10.1001/archpedi.1982.03970470069018