Pentalogy of Cantrell

Pentalogy of Cantrell

Pentalogy of Cantrell

Five defects of the pentalogy as described in Cantrell’s initial paper are as follows:

  1. Supraumbilical omphalocele
  2. Lower sternal cleft
  3. Defect in the central tendon of the diaphragm
  4. Defect in the pericardium
  5. Intracardiac anomaly

Pericardial Defects

  • Pericardial effusion is found in some cases, although relatively less severe and defects in pericardium do not present an immediate threat to life.
  • It can present with muffled heart sound on cardiac auscultation.

Cardiac Defects may be present

  1. Atrial septal defects
  2. Ventricle septal defects
  3. Left ventricular diverticulum
  4. Dextrocardia
  5. Pulmonary stenosis
  6. Tetralogy of Fallot have all been associated with this disease