Biliary Atresia

Yellow

Step 1: View clinicals

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.


Step 2: Order all relevant investigations

Serum Bilirubin

Total bilirubin: 15.8 mg/dL Direct bilirubin: 13.3 mg/dL

Ultrasound Abdomen

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.

ERCP

The distal common bile duct and gallbladder are visualized. The remainder of the biliary tree cannot be identified.

Liver biopsy

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.


Step 3: Select appropriate management

Phototherapy
Exchange transfusion
Portoenterostomy
Urgent Liver transplantation


Score: ★★☆