|Year : 2017 | Volume
| Issue : 1 | Page : 50-51
Ectopia cordis : A case report
Kinthanliu Guanmei1, Namganglung Golmei2, Rajkumari Ajita1, Aribam Jaishree Devi1
1 Department of Anatomy, Regional Institute of Medical Sciences, Imphal, Manipur, India
2 Department of Pediatrics, Regional Institute of Medical Sciences, Imphal, Manipur, India
|Date of Web Publication||17-Jan-2017|
Department of Anatomy, Regional Institute of Medical Sciences, Lamphelpat, Imphal - 795 004, Manipur
Source of Support: None, Conflict of Interest: None
Ectopia cordis is a rare congenital heart anomaly where the heart is abnormally located outside the thoracic cavity. The defect lies in the failure of the midline closure of the body wall by lateral and head and tail folds. Because of its associated abnormalities, ectopia cordis is a challenging congenital anomaly. The prognosis of ectopia cordis depends on its classification and associated intracardiac anomalies.
Keywords: Ectopia cordis, head and tail folds, lateral folds
|How to cite this article:|
Guanmei K, Golmei N, Ajita R, Devi AJ. Ectopia cordis : A case report. J Med Soc 2017;31:50-1
| Introduction|| |
Ectopia cordis , also known as exocardia or ectocardia, is a rare congenital anomaly where the heart is abnormally located outside the thoracic cavity. Haller et al. first reported ectopic cordis in 1706, and it was classified into different types by Weese et al. in 1818 and Todd et al. in 1836. , Its prevalence rate is reported to be 5.5-7.9 per million live births. 
| Case Report|| |
A 24-year-old primigravida mother delivered a full-term male baby of 3.8 kg weight in Regional Institute of Medical Sciences (RIMS), Imphal, Manipur, India by normal vaginal delivery (NVD) presenting a pulsatile mass of heart projecting outwards and upwards along with the liver and the umbilicus through 10-12 cm midline sternal defects. The vitals and reflexes were normal. The beating heart and major vessels were covered with a serous membrane. There were no other visible physical abnormalities [Figure 1]. The baby died after 9 days of conservative comfort care treatment.
| Discussion|| |
Embryologically ectopia cordis is due to defect in the closure of lateral folds to form the ventral body wall. By the end of the 3 rd week the lateral plate mesoderm splits into outer somatic and inner splanchnic layers and created a space intervening the two layers. During the 4 th week, the sides of the embryo begin to grow ventrally and by the end of the 4 th week, the lateral folds meet in the midline and fuse to close the ventral body wall. This closure is aided by head and tail folds. The complete or incomplete failure of midline fusion and defect in mesoderm in embryonic state can result in a variety of disorders ranging from isolated ectopia cordis to complete ventral evisceration. 
Ectopia cordis may be of complete variety or partial variety. According to its location where the heart is present, the ectopia cordis may be of cervical (3%), cervicothoracic (<1%), thoracic (60%), thoracoabdominal (7%), or abdominal (30%) type. The thoracoabdominal type is frequently associated with Cantrell's pentalogy that includes bifid sternum, deficiency of the diaphragm, defect of the diaphragmatic pericardium, defect of the anterior abdominal wall, and intracardiac defects. 
Because of its rarity and other commonly associated abnormalities, ectopia cordis is a challenging congenital anomaly. The complete ectopia cordis is a neonate emergency. Many cases of these (80.2%) have associated intracardiac defects,  including ventricular septal defect (VSD, 100%), atrial septal defect (ASD, 53%), tetralogy of Fallot (TOF, 20%), left ventricular diverticulum (LVD, 20%), and pulmonary hypoplasia. 
The prognosis of ectopia cordis depends on its classification and associated intracardiac anomalies.  Reports of successful repair of thoracic ectopia cordis are rare, the cervical type is universally fatal, and the thoracoabdominal type has a higher rate of successful repair. ,,,
There are descriptions of ectopia cordis with chromosomal abnormalities, but there is no known genetic etiology in humans. 
It is important to distinguish true ectopia cordis from the failure of sternal fusion in which the heart is structurally normal. Many cases reported as thoracic ectopia, with intact skin, are more likely examples of isolated failure of sternal fusion.
If the diagnosis of ectopia cordis is confirmed during the pregnancy, an early plan should be made for elective atraumatic cesarean delivery.  Immediately after birth, the newborn should be stabilized and the lesion should be covered with saline-soaked gauze pads and wrapping to prevent desiccation and heat loss of the exposed viscera. After completing the preoperative evaluation, the patient should be taken promptly to the operation room for surgical repair of the defects. The overall objectives of ectopia cordis management are: Closure of the chest wall defect, including the sternal defect, repair of the associated omphalocele, placement of the heart into the thorax, and repair of the intracardiac defect. ,
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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