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

  • omphalocele
  • ectopia cordis (abnormal location of heart)
  • diaphragmatic defect
  • pericardial defect or sternal cleft
  • cardiovascular malformations 
  • Pathology

    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).


    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.

    See also

    Siehe auch:
    und weiter: