Amniotic band syndrome as a cause of ectopia cordis thoracalis and other craniofacial defects

A 24 week gestation fetus, on ultrasound, was found to have Ectopia Cordis, besides other anomalies like frontonasal dysgenesis, facial cleft, hydrocephalus and a distorted skull vault. Fibrous bands were seen to be attached to the apex of the heart, face, and placenta. Isolated Ectopia Cordis occurs due to malformed thoracic chest wall during early embryogenesis. However, fibrous bands adherent to the heart causing mechanical forces may also lead to ectopia cordis, referred to as mechanical teratogenesis. These amniotic bands are formed due to early rupture of chorion or amnion in embryogenesis and a spectrum of defects is found following it, the variety of which is based upon the timing of rupture. As is illustrated by our ultrasound pictures and clinical specimens, a cephalic pointing cardiac apex secondary to tethering of the heart to frontonasal region of face by amniotic bands could be the etiology of this thoracoabdominal ectopia cordis Thus Ectopia cordis with amniotic bands appears to have an etiology entirely different from isolated ectopia cordis. This suggests distinct etiologies for ectopia cordis and in the light of literature, they have been discussed with this case repor