A Rare Case of Complete Thoracic Ectopia Cordis with Clubfeet and Hands

Abstract

Ectopia cordis is a rare congenital condition. It is defined as the abnormal position of the heart outside the thoracic cavity, associated with defects in the parietal pericardium, diaphragm, sternum, and, in most cases, cardiac malformations. Ectopia cordis was first proposed by Abott in 1998, although cases of patients with similar defects have been reported in the past. Ectopia cordis is produced by segmental defects in the mesodermal development in the third week of intrauterine life, and/or amniotic band syndrome that causes simultaneous cerebral and thoracoabdominal malformations. The existence of ectopia cordis with severe congenital heart disease may be confirmed in the prenatal period by vaginal echocardiography at 10-12 weeks of gestation or by abdominal echocardiography at 20-22 weeks. For such defects surgical correction is the only hope of survival, although the overall success rate is very poor. In recent years, surgery has been attempted in one or two phases with variable results that depend mainly on the type of associated heart disease. A 37 years old woman with gestational age of 29.5 weeks was referred for third trimester ultrasound to detect fetal anomaly. The fetal heart was seen outside lower the chest wall which was later confirmed by elective cesarean section. The heart was not covered with a membrane (pericardium). The rest of the anterior abdominal wall was intact. The abdomen was distended due to ascites.