pentalogy of Cantrell
The pentalogy of Cantrell is an extremely rare phenomenon with an incidence estimated at around 6 per million live births .
It encompasses the following 5 main features
It is thought to result from an abnormal migration of the sternal anlage and myotomes in the early embryonic stage ( ~ 6 to 7 week of gestation).
- cystic hygroma
- craniofacial anomalies
- vertebral anomalies
- limb anomalies
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 . Surgical management aims to :
- 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.
- angeborene Wirbelanomalien
- Trisomie 18
- Fallot'sche Tetralogie
- ventricular septal defect (VSD)
- diaphragmatic defect
- ectopia cordis
- pentalogy of Fallot
- cardiovascular malformations
- zystisches Lymphangiom