Polysplenia syndrome

Polysplenia syndrome, also known as left isomerism, is a type of heterotaxy syndrome where there are multiple spleens congenitally as part of left-sided isomerism.

Polysplenia is seen predominantly in female patients. It is usually diagnosed in childhood or adulthood, later than asplenia syndrome, since associated congenital heart diseases tend to be less severe than those encountered in the latter. 

Clinical presentation is related to the associated pathology.

Polysplenia results from failure of development of the usual left-right asymmetry of organs and consists of situs ambiguous, with features of bilateral left-sidedness.

It is characterised by multiple splenules without a parent spleen. It occurs on the patient’s left side but may be bilateral. The most common associated feature is inferior vena cava interruption with azygous or hemiazygous continuation 3.

Other characteristic features include:

Share article

Article information

rID: 21610
Section: Anatomy
Synonyms or Alternate Spellings:
  • Left isomerism
  • Polysplenia
  • Bilateral left-sidedness
  • Left sided isomerism

Support Radiopaedia and see fewer ads

Cases and figures

  • Drag
    Case 1
    Drag here to reorder.
  • Drag
    polysplenia, azyg...
    Case 2 : with azygous continuation of the IVC
    Drag here to reorder.
  • Drag
    Multiple spleens ...
    Case 3 : with multiple spleens of variable size
    Drag here to reorder.
  • Drag
    Axial CECT in pat...
    Case 4
    Drag here to reorder.
  • Drag
    Case 5 : situs inversus abdominalis
    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.