A 5 week old girl presents with persistent yellowish discoloration of her skin and sclera for 3 weeks. She is feeding well and has no other symptoms. She is the product of a normal pregnancy and delivery, with a normal birth weight. Her growth and development are age appropriate.
Total bilirubin: 15.8 mg/dL Direct bilirubin: 13.3 mg/dL
Ultrasonography shows an atretic gallbladder with a thin wall and irregular contour. The liver parenchyma is inhomogeneous and there is increased periportal echogenicity. Moderate splenomegaly is present.
The distal common bile duct and gallbladder are visualized. The remainder of the biliary tree cannot be identified.
Microscopy shows evidence of biliary obstruction. There is diffuse proliferation of the bile ductules, while the interlobular ducts are filled with bile plugs. There is no evidence of toxic damage to the liver cells.