Ectopia Cordis

Abdul Quddus,Kona Chowdhury,Mahmuda Hassan,Md Hamidur Rahman

doi:10.3126/jnps.v36i2.14807

Abstract

Ectopia Cordis is a rare congenital anomaly characterized by partial or complete displacement of the heart outside the thoracic cavity. Usually ectopiacordis is associated with other multiple anomalies and intra cardiac defects. The five main ectopic positions are adjacent to the thorax approximately 60%, abdominal is 15-30%, thoraco-abdominal is 7-18%, cervical is about 3% and least is the cervicothoracic. We are reporting a case of ectopiacordis of abdominal type. This is the first reported abdominal ectopia cordis case in Bangladesh.

Highlights

Hallerfirst described the term Ectopiacordis in 17061.The term was derived from Greek word ektopos meaning away from a place
Ectopiacordis is a rare and striking congenital heart defect, which was first observed 5000 years ago[5].The defect was described as malposition of heart, partially or completely outside the thorax
Ectopiacordis is usually associated with other congenital anomalies and intra cardiac defects[6].In abdominal variety of ectopiacordis heart passes through a defect in the diaphragm to enterthe abdominal cavity[3,7].Ventricular septaldefect, atrial septal defect, diverticulum of the ventricle and tetralogy of Fallot are the most common intra-cardiac defects[8,9]

Summary

Introduction

Hallerfirst described the term Ectopiacordis in 17061.The term was derived from Greek word ektopos meaning away from a place. In Cantrell’s pentalogythe full spectrum consists of five anomalies: A deficiency of the anterior diaphragm, a midline supraumbilical abdominal wall defect, a defect in the diaphragmatic pericardium, various congenital intracardiac abnormalities, and a defect of the lower sternum[4].Ectopiacordis interna, known as Tin. Man syndrome, is a rare variant of ectopiacordis in which the heart is located completely within the abdominal cavity. Man syndrome, is a rare variant of ectopiacordis in which the heart is located completely within the abdominal cavity It is almost always an asymptomatic condition found incidentally on imaging, or less often detected by physicians when attempting to auscultate the chest or at abdominal palpation. Baby was delivered normally and perinatal and the postnatal history was uneventful This was the 2nd issue of a nonconsanguineous parent.

Discussion

Conclusion

Taussing HB