Asplenia syndrome, also known as right isomerism or Ivemark syndrome, is a type of heterotaxy syndrome.
There is an increased male predilection. Asplenia syndrome is usually diagnosed in neonates .
Howell-Jolly and Heinz bodies are seen on H&E blood smear .
Main characteristic radiographic features include:
- absence of spleen
- bilateral eparterial bronchi
- bilateral trilobed lungs
- bilateral right atria
- transverse liver
- severe/complex congenital heart disease (50%), especially cyanotic congenital cardiac anomalies