Obliteration of the distal bile duct in the development of congenital choledochal cyst.

The authors report the first case of antenatally diagnosed choledochal cyst having evidence of early fetal bile drainage and complete obliteration of the distal bile duct. This disputes the traditional hypothesis of reflux of pancreatic enzymes and supports the recent theory of primary obstruction as the etiologic cause of choledochal cyst. Coexisting congenital hypothyroidism and pulmonary stenosis had not been reported previously.