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, intestinal malrotation

Intestinal malrotation

Intestinal malrotation is a congenital anomaly of rotation of the midgut...

, situs inversus

Situs inversus

Situs inversus is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement is known as situs solitus...

, biliary atresia

Biliary atresia

Biliary atresia, also known as "extrahepatic ductopenia" and "progressive obliterative cholangiopathy" is a congenital or acquired disease of the liver and one of the principle forms of chronic rejection of a transplanted liver allograft. As a birth defect in newborn infants, it has an occurrence...

, and the range of congenital cardiac malformations.

Although present, the multiple small spleens are ineffective; this is termed functional asplenia.