Polysplenia is a

congenital disease A birth defect, also known as a congenital disorder, is an abnormal condition that is present at birth regardless of its cause. Birth defects may result in disabilities that may be physical, intellectual, or developmental. The disabilities can r ...

manifested by multiple small accessory spleens, rather than a single, full-sized, normal spleen. Polysplenia sometimes occurs alone, but it is often accompanied by other developmental abnormalities. Conditions associated with polysplenia include gastrointestinal abnormalities, such as

intestinal malrotation Intestinal malrotation is a congenital anomaly of rotation of the midgut. It occurs during the first trimester as the fetal gut undergoes a complex series of growth and development. Malrotation can lead to a dangerous complication called volvulus ...

or biliary atresia, as well as cardiac abnormalities, such as

dextrocardia Dextrocardia (from Latin ''dextro'', meaning "right hand side," and Greek ''kardia'', meaning "heart") is a rare congenital condition in which the apex of the heart is located on the right side of the body, rather than the more typical placement t ...

Associated conditions

There are frequent associated congenital anomalies all related to deviations in the development of anatomical asymmetries in early embryonic stages. These conditions considered together are called "polysplenia syndrome". Associated conditions include heterotaxy syndrome,

situs inversus Situs inversus (also called situs transversus or oppositus) is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement of internal organs is known as situs solitus. Al ...

, biliary atresia, and several cardiac malformations. Associated cardiac conditions include

, 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.

References

External links

Diseases of spleen {{congenital-malformation-stub