A neonate 25 days old with a prolonged episode of jaundice from birth has a spine radiograph that shows an abnormal segmentation of the vertebral bodies at D8 level. An ultrasound scan shows a collapsed gallbladder and 99mTc-DISIDA scintigraphy shows tracer in the bowel at 24 hours. What is the likely diagnosis?

 

 

 

 

[size=20px]a Alagille’s syndrome

 

b biliary atresia (type I)

 

c neonatal jaundice

 

d biliary atresia (type III)

 

e Caroli’s disease[/size]

A

correct answer

a Alagille’s syndrome

 

The spine X-ray shows a hemivertebrae at D8 level. The collapsed gallbladder and tracer that makes it into the bowel should suggest Alagille’s syndrome (biliary hypoplasia). Biliary atresia would result in no tracer leaving the hepatic system and hence, no tracer would make it into the bowel.

A