Syndrome of Congenital Absence of the Spleen with Associated Cardiovascular and Gastroenteric Anomalies

Abstract

Survey of 27 cases in the literature and the authors' 3 cases reveals a characteristic syndrome of congenital absence of the spleen with certain cardiovascular and gastrointestinal abnormalities. The hematologic and other findings permit a presumptive antemortem diagnosis. More accurate prognosis is made possible and valuable information is afforded the cardiac surgeon, since the cardiac anomalies often follow a predictable pattern.