Exencephaly With Ectopia Cordis

Abstract

Ectopia cordis is an anomaly of embryonic cell growth that causes the heart to form outside the chest cavity during fetal development. Other birth defects are often present with ectopia cordis as well. It is usually a fatal condition, commonly associated with pentalogy of Cantrell, and occurs in 5.5 to 7.9 children per one million live births. Exencephaly is the exposure of a well-developed and differentiated brain outside the skull during the embryonic period. It is the second of three phases in the development of anencephaly and is a lethal neural tube defect with only a small number of cases reported in the literature. Sonographically, it appears as a bilobed-shaped head termed the “Mickey Mouse” head with a relatively large amount of brain tissue present floating in the amniotic fluid.