Ivemark syndrome


pol·y·sple·ni·a

(pol'ē-splē'nē-ă), [MIM*208530] A condition in which splenic tissue is divided into nearly equal masses or totally absent; congenital heart disease and malposition and maldevelopment of abdominal organs are common; may be related to situs inversus. Most cases are sporadic, although some suggest autosomal recessive inheritance.
See also: bilateral left-sidedness. Synonym(s): asplenia with cardiovascular anomalies, Ivemark syndrome, polyasplenia [poly- + G. splēn, spleen]

Ivemark syndrome

A heterotaxy syndrome (OMIM:208530) in which both right and left atria of the heart are morphologically right. Other associated anomalies include asplenia, mid-line liver, small intestine malrotation and two morphological right lungs.
 
Clinical findings
In keeping with congenital heart defect—i.e., cyanosis, breathlessness, lethargy, poor feeding.

pol·y·sple·ni·a

(pol'ē-splē'nē-ă) A condition in which splenic tissue is divided into nearly equal masses; there are several small spleens; congenital heart disease and malposition and maldevelopment of abdominal organs are common; may be related to situs inversus. Most cases are sporadic, although some suggest autosomal recessive inheritance.
Synonym(s): Ivemark syndrome. [poly- + G. splēn, spleen]

Ivemark,

Björn, Swedish pathologist, 1925–. Ivemark syndrome - a possibly heritable disorder in which organs of the left side of the body are a mirror image of their counterpart on the right side, with associated splenic agenesis and cardiac malformations.