Biliary atresia

Case contributed by Dr James Harvey

Presentation

Infant with conjugated hyperbilirubinemia.

Patient Data

Age: 6 weeks
Gender: Male
Ultrasound

There is no identifiable gallbladder despite three hours of fasting.  The extrahepatic common bile duct is not clearly identified. 

The liver has a normal echogenicity and echotexture. Normal antegrade portal vein flow.

Nuclear medicine

DISIDA study

There is symmetrical extraction of radiotracer from the blood pool by both lobes liver.  There is accumulation of radiolabelled radiotracer within the liver with no evidence of radiolabelled bile within the intra or extrahepatic biliary tree.

Delayed planar images do not demonstrate any evidence of radiolabelled bile within the small intestine.

Case Discussion

Biliary atresia is congenital disorder characterized by the absence or paucity of the extrahepatic biliary tree.

On ultrasound, the triangular cord sign refers to echogenic fibrous tissue adjacent to the portal vein, representing the abnormal bile duct. The gallbladder is absent in half of cases.

Early surgical intervention with Kasai procedure reduces the need for transplantation. Biliary atresia is the most common indication for liver transplantation in children. 

This child developed cirrhosis despite Kasai procedure and required a liver transplant at age 1.

How to use cases

You can use Radiopaedia cases in a variety of ways to help you learn and teach.

Creating your own cases is easy.