Acardiac anomaly spectrum

Abstract

Background: Acardiac anomaly spectrum is a rare congenital malformation found in monozygotic twin pregnancy. Besides the absence of heart, the condition is associated with variable grades of developmental disruption. Thus, no two cases are similar. Methods: This case report is based on physical examination and autopsy findings. Results: The twin had acardia and partial development of head and face. There was complete absence of upper extremities. Conclusions: The twin reversed arterial perfusion (TRAP) theory is the most accepted etiology of the disorder. Normally, the cephalic pole is the most severely affected, being most distal to the retrograde perfusion. In acardia, partial development of head, face, and brain is usually associated with the development of the upper extremities. However, in the present case, there was extensive cephalic development in the absence of upper extremity development. (C) 2000 Wiley-Liss, Inc.