Ectopia cordis.

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.[23]

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.