Situs Ambiguous / Asplenia / Polysplenia Syndrome

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Etiology/Pathophysiology:
The positions of the viscera and their morphologic characteristics are indeterminant and asymetric structures appear to be symmetric.

Asplenia has right sided symmetry with bilateral trilobed lungs, an absent spleen, horizontally positioned liver, with the cardiac apex on the left or the right. The superior vena cava is usually bilateral, and interruption of the inferior vena cava is unusual. Multiple and complex cardiac malformations are often seen and malrotation is common. Survival beyond infancy is rare.

Pathology:
Not applicable

Imaging Findings:
Asplenia : a relatively small heart, decreased pulmonary vascularity, a bilateral right sided tracheobronchial tree, and a horizontal liver and midline stomach.

DDX:

• Not applicable

References:
See References Chapter.

Section Top | Title Page

See related Provider Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.

See related Patient Topics Congenital Heart Disease, Genetics/Birth Defects or Heart and Circulation.

All contents copyright © 1992-2004 the Author(s) and The University of Iowa. All rights reserved.

http://www.vh.org/pediatric/provider/radiology/PAP/CVDiseases/SitusAmbig.html