Ectopia Cordis

Abstract

Ectopia cordis is a rare congenital anomaly. Congenital anomaly scan can detect it at 18–23 weeks of gestation. Four chamber view of the heart in routine fetal anomaly scans at >18 weeks is the most effective technique to detect CHD prenatally.

INTRODUCTION

Ectopia cordis: This case is that of a 1-day-old female born at 34 weeks of gestation by Emergency LSCS [Figure 1]. Baby had normal APGAR and examination revealed chest wall defect with mediastinal contents outside. Antenatal USG did reveal a pulsating mass outside the body cavity, but it was overlooked [Figure 2]. Antenatal fetal anomaly scan at 18-24 weeks can diagnose 50% of the major congenital heart disease. However routine four-chamber view appears to be inadequate for the outflow tract lesions, and therefore specific assessment of the outflow tracts is necessary to improve the detection rate.

Figure 1.

Ectopia cordis

Figure 2.

Fetal anomaly scan – ectopia cordis

Postnatal ECHO and CT thorax revealed AV canal defect with pulmonary hypoplasia.

DISCUSSION

Ectopia cordis is a rare anomaly with a prevalence of 0.08/10,000 live births.[1] Four types have been recognized, viz., (1) thoracic (65%), (2) thoraco-abdominal (20%), (3) abdominal (10%), and (4) cervical.[2,3]

It is associated with other congenital defects, e.g. hydrocephalus, cleft palate, congenital heart defects, hypoplastic lung disease, skeletal dysplasia, diaphragmatic hernia, abdominal wall defect, etc.[4]

Management

Prognosis is poor and parents should be counseled for mid-trimester termination of pregnancy. Attempts at surgical correction have been largely unsuccessful.