Pentalogy of Cantrell
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At the time the article was created Yuranga Weerakkody had no recorded disclosures.View Yuranga Weerakkody's current disclosures
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The pentalogy of Cantrell is an extremely rare phenomenon with an incidence estimated at around 6 per million live births 3.
It encompasses the following 5 main features 1.
- ectopia cordis (abnormal location of heart)
- diaphragmatic defect
- pericardial defect or sternal cleft
- cardiovascular malformations
It is thought to result from an abnormal migration of the sternal anlage and myotomes in the early embryonic stage ( ~ 6th to 7th week of gestation).
- cystic hygroma
- craniofacial anomalies
- vertebral anomalies
- limb anomalies 3
Treatment and prognosis
Most cases carry a very poor prognosis. The diversity and complexity of pentalogy of Cantrell results in a variable clinical course requiring unique surgical approaches 9. Surgical management aims to 9:
- correct cardiac malformations
- ensure adequate space for return of the heart to the mediastinum
- protect the heart from compression and distortion via repair thoracoabdominal wall defects
History and etymology
It was first described by James R Cantrell in 1958.
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