Ectopia cordis in man.

Ectopia cordis in man is a relatively rare anomaly and has been known for many years, being first reported in 1671 by Neil Stensen (Willius, 1948). A translation of the relevant excerpt from Stensen's article reads, ". . . the sternum was split and the heart, liver and spleen, most of the intestine and right kidney have passed out through the split being thus uncovered." This description is undoubtedly that of a case of ectopia cordis and antedates the case of Haller (1706, quoted by Byron, 1948) and Martinez (1706), usually regarded as the first to have been reported. Since then over 180 cases have been described. A survey of the available literature indicates confusion as to the exact meaning of ectopia cordis. Ectopia is derived from the Greek ektopos, meaning away from a place, and implies an abnormality in the position of an organ or a part of the body, often congenital in origin. According to this definition ectopia cordis should include conditions such as dextrocardia, in which the heart remains within the thorax. Barlow (1938) is correct in suggesting that if ectopia cordis is to include this type of defect it should be qualified as intrathoracic. It seems, however, that ectopia cordis has been mainly limited to those congenital anomalies in which the heart is not entirely within the chest cavity, and Abbott (1927) qualifies ectopia cordis as a " displacement so that the heart passes out of the thorax and comes to lie either on the outer surface of the body or in the abdominal cavity." Even this definition overlooks malpositions of the heart in relation to the cervical region. Similarly, Kalter and Warkany (1959) disregard the cervical and abdominal types of the defect when they define ectopia cordis or ectocardia as a " congenital exposure of the heart due to insufficient development of the chest." Moreover, it is evident that the term " displacement" is inappropriate when referring to the cervical type of ectopia cordis, since in these cases the heart is merely retained in its embryonic position in the neck. In view of these difficulties we suggest that ectopia cordis be defined as a congenital malposition of the heart, partially or completely outside the thorax.