The authors report the case of a 2-year-old girl with hepatomegaly and failure to thrive in whom the diagnosis of congenital hepatic fibrosis was first considered on the basis of the histological examination of a percutaneous liver biopsy. Further radiographic and ultrasonic investigations of the biliary tree showed a choledocal cyst and dilatation of the intrahepatic ducts. Surgical operation consisted in complete removal of the cyst with hepaticojejunostomy. The congenital intrahepatic abnormalities associated with the choledochal cyst are commented.
Buts, J.-P., Otte, J.-B., Claus, D., Van Craynest, M. P., & De Meyer, R. (1980). Kyste du cholédoque: un cas avec dilatation des voies biliaires intrahépatiques et fibrose hépatique congénitale. Helvetica Paediatrica Acta, 35(3), 289-295. https://hdl.handle.net/2078.5/133119 (Original work published 1980)