Table 1.

Summary Table of Polysplenia Syndrome.

Etiology	• Not yet fully understood • Factors embryogenic, genetic, and teratogenic have been suggested
Incidence	• 1 per 250 000 live births has polysplenia syndrome
Gender predilection	• Female gender
Age predilection	• Childhood and adulthood
Risk factors	• Factors embryogenic, genetic, and teratogenic • Mutations have been identified in the genes of patients with heterotaxia.
Treatment	• The surgical treatment is indicated according to the type of associated cardiac and vascular malformations also according to the age and the clinical tolerance
Prognosis	• Generally good prognosis but can sometimes depend on essentially cardiac malformations
Findings on imaging	• Situs ambiguous or rarely situs inversus • Polysplenia sometimes a polylobed or normal spleen • Left isomerism • Agenesis of the inferior vena cava with continuation of azygos type Often • Bilobed right lung • Common atrioventricular canal • Complete common mesentery and other malformations . . .