|A 12-year-old female admitted with cardiac failure. What is the diagnosis?|
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Ectopia cordis with bilateral pleural effusions
DiscussionEctopia cordis is a rare congenital defect in fusion of the anterior chest wall resulting in an extrathoracic location of the heart. Depending on the position of the heart, ectopia cordis has been categorized into four types: cervical, thoracic, thoracoabdominal and abdominal. The combination of thoracoabdominal ectopia cordis, lower sternal defect, anterior diaphragmatic hernia, midline supraumbilical defect along with pericardial and intracardiac defects constitute the Pentalogy of Cantrell
Predominant theories include: primary failure of descent and midline fusion of the lateral body folds, early rupture of chorion and/or yolk sac causing failure of midline fusion and amniotic band syndrome.
The prenatal diagnosis is easily made with ultrasound by visualizing the heart outside the thoracic cavity.
Prognosis is generally
poor and depends on the severity of intracardiac malformations and the
presence of associated abnormalities. Most infants are stillbom or die
within the first hours or days of life. Attempts at surgical correction
have been largely unsuccessful due to the extent of the associated anomalies.
Recommendations for surgical management include immediate covering of
the exposed heart and abdominal contents with a silastic prosthesis, as
well as full evaluation and correction of any intracardiac defects prior
to replacement of abdominal contents.
Ashok Raghavan, Manipal Hospital, Bangalore