Polysplenia sometimes occurs alone, but it is often accompanied by other developmental abnormalities.
There are frequent associated congenital anomalies all related to deviations in the development of anatomical asymmetries in early embryonic stages.
[2] Associated conditions include heterotaxy syndrome, intestinal malrotation, situs inversus, biliary atresia,[3] and several cardiac malformations.
[4] Associated cardiac conditions include dextrocardia, atrial situs ambiguus, ventricular inversion, and VA concordance with left posterior aorta.
Although present, the multiple small spleens are often ineffective; this is termed functional asplenia.