Ectopia cordis is a rare congenital malformation which usually results in death soon after birth. The entire group of anomalies would appear to be closely related embryologic development, arising as the result of defective formation and
differentiation
of the ventral mesoderm at about 14 to 18 days of embryonic life. According to the position of the heart, four types of ectopia cordis can be distinguished. The most frequently observed is the thoracic type, accounting for 60 percent of patients.
The
heart is displaced outside the thoracic cavity, protruding through a defect in the sternum. In the abdomonal type, which accounts for 30 percent of patients, the primary defect is though to be a gap in the diaphragm through which the heart
protrudes
inside the abdominal cavity. The throacoabdominal type, which accounts for 7 percent of patients, is the variety present in the pentalogy of Cantrell. The cervical type accounts for 3 percent of all cases of ectopia cordis.
Recently, a case of ectopia cordis which was diagnosed by ultrasonography at 36 weeks' gestation in a 27 years old primipara and thcracoabdominal type was experienced at our department.
We present this case with a brief review of literature.
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