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.4

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

Howell-Joly and Heinz body are seen on H&E blood smear.

General

Main characteristic radiographic features include:

Associations
Share article

Article information

rID: 25433
System: Paediatrics
Sections: Gamuts, Syndromes
Tag: cases
Synonyms or Alternate Spellings:
  • Right isomerism
  • Asplenia syndrome
  • Right sided isomerism
  • Bilateral ride-sidedness

Support Radiopaedia and see fewer ads

Cases and figures

  • Drag
    Case 1
    Drag here to reorder.
  • Drag
    Case 1
    Drag here to reorder.
  • Updating… Please wait.
    Loadinganimation

    Alert accept

    Error Unable to process the form. Check for errors and try again.

    Alert accept Thank you for updating your details.