Asplenia Syndrome: A Study of Congenital Anomalies in 16 Cases

Sixteen new cases of the asplenia syndrome with autopsy findings had a female preponderance of 2:1. Racial incidence of splenic agenesis was nine whites and seven blacks. The average life span of the infants born alive was approximately 38 days. All infants with the asplenia syndrome had recorded weights and lengths below normal standard values. Surgical palliation of the cardiovascular anomalies in the asplenia syndrome was attempted in two cases. The incidence of the asplenia syndrome from the large autopsy series at the Medical University of South Carolina is one case per 2,000 autopsies. ( JAMA 240:1508-1510, 1978)