Ectopia cordis
| Ectopia cordis | |
|---|---|
| Classification and external resources | |
| ICD-10 | Q24.8 |
| ICD-9 | 746.87 |
Ectopia cordis is a birth defect in which the heart is abnormally located. In the most common form, the heart protrudes outside the chest through a split sternum. Less often, the heart may be situated in the abdominal cavity or neck.
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[edit] Classification
Depending on the position of the heart, ectopia cordis can be classified into four categories:
- Cervical - in the neck
- Thoracic - where the heart would lie within the thoracic cavity.
- Thoracoabdominal - where the heart would lie somewhere between the thoracic and abdominal cavities.
- Abdominal - where the heart would lie in the abdominal cavity.
[edit] Cause
The occurrence of ectopic cordis is about 7.9 per million births. Ectopia cordis is a very rare congenital heart malformation. It is caused by a failure of the ventral body wall to close properly during embryonic development.
No exact cause has been identified but this condition has also been seen more frequently in Turner Syndrome and Trisomy 18; however it does not appear to be an inherited disease.
The ectopic heart is not protected by the skin or sternum. Other organs may also have formed outside the skin. Often the heart is not formed properly and many other heart defects are associated with this condition including: Tetralogy of Fallot, pulmonary atresia, atrial and ventricular septal defects, double outlet right ventricle. Other non-cardiac malformations may be present such as cleft palates, and spine malformations that can cause kyphosis.
[edit] Prognosis
Most cases result in stillbirth or death shortly after birth. Some cases of ectopia cordis can be treated surgically.
[edit] Treatment
Surgical treatment is possible, but there are frequently other birth defects present.
Doctors at AIIMS, New Delhi, India successfully operated on a 10-day-old male, who was born with this abnormality, on 2 September 2009. The doctors created a record by completing the surgery without suspending his bodily functions.[1] However, the boy died at 22 days.[2]
[edit] References
- Amato J, Douglas W, Desai U, Burke S (2000). "Ectopia cordis.". Chest Surg Clin N Am 10 (2): 297–316, vii. PMID 10803335.
- Ellis-Christensen, Tricia. "Ectopia cordis.". Theswisegeek.com.
- [1] Video of Christopher Wall on Guinness World Records Primetime.
[edit] External links
[edit] References
- ^ Chitlangia, Risha (3 September 2009). "AIIMS docs successfully operate on 'miracle baby'". The Times Of India. http://timesofindia.indiatimes.com/news/city/delhi/AIIMS-docs-successfully-operate-on-miracle-baby/articleshow/4967437.cms.
- ^ "Miracle baby dies after 22-day ordeal". The Times Of India. 17 September 2009. http://timesofindia.indiatimes.com/articleshow/msid-5019827.cms.
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