Polysplenia

The presence of several small spleens. It is often isolated, but may be associated with other cardiac or visceral malformations. This malformation is as frequent in boys as in girls.

These malformations are (see also heterotaxy):

-        cardiac: 90 %: persistent left superior vena cava (33-50 %); lack of coronary sinus (26-42 %); partial anomalous pulmonary venous return (37-50 %); dextrocardia (33-42 %); congenital atrioventricular (80 %); sometimes hypoplasia of one ventricle (37 %); A-V block

-        pulmonary: 2 bilobed lungs (72-88 %).

-        liver: median liver  (50-67 %); interruption of the inferior vena cava that drains into the azygos vein (58-100 %); biliary atresia


Anesthetic implications:

cardiac echography; complex cardiac malformation ; high incidence of left superior vena cava; risk of absence of inferior vena cava.


References : 


Updated: October 2018