Pentalogy of Cantrell

Last revised by Joshua Yap on 24 Apr 2022

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.

  1. omphalocele
  2. ectopia cordis (abnormal location of heart)
  3. diaphragmatic defect
  4. pericardial defect or sternal cleft
  5. 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).

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

It was first described by James R Cantrell in 1958.

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