Asplenia syndrome

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 .

Clinical presentation

In contrast to polysplenia syndrome, most patients die before 1-year-old because of severe/complex congenital heart disease. Most patients are immunocompromised due to absent spleen.


Howell-Jolly and Heinz bodies are seen on H&E blood smear .

Radiographic features


Main characteristic radiographic features include:


See also