Abstract

A syndrome featuring defects of the heart, sternum, diaphragm, and anterior abdominal wall has been designated as Cantrell's syndrome. Since Cantrell described this syndrome as one type of ectopia cordis there has been debate and confusion in the literature on its entity particularly in relationship to ectopia cordis. Since ectopia cordis is defined as "the heart is completely or incompletely located outside the mediastinum" and every case Cantrell's syndrome is associated with pericardial and diaphragmatic defects as well as low sternal defect, these two seemingly different entities should be closely related or may even be in the spectrum of same anomaly complex. With above speculation in mind we have reviewed two cases of typical Cantrell's syndrome, two cases of typical ectopia cordis and a case of bifid sternum from autopsy file of Seoul National University Children's Hospital. Findings pertinent to the differential diagnosis are tabulated. Based on postmortem findings on these 5 cases it was concluded that these three anomaly complexes are essentially same, being only different in severity and probably of time of occurrence during developmental period. Therefore it was suggested that the definition of Cantrell's syndrome should be extended to include all cases of ectopia cordis. Or the whole syndrome could be redesignated as midline defect syndrome because it involves the midline of the body and is always multiple and complex anomaly.

• Keywords: Ectopia cordis;Cantrell syndrome;Heart;Midline cleft;Diaphragm