Pentalogy of Cantrell

  • Etiology: anterior abdominal wall defect with defect in lower sternum + defect in anterior diaphragm + defect in diaphragmatic portion of pericardium leading to ectopia cordis
  • Imaging: ectopia cordis
  • Clinical: also has congenital heart disease

Cases of Pentalogy of Cantrell

CXR of Pentalogy of Cantrell
CXR and AXR shows the cardiac margin to be absent below the level of the diaphragm. The left lung is hyperlucent compared to the right. There is a paucity of bowel gas within the abdomen and centralization of bowel loops
MRI of Pentalogy of Cantrell
Axial (above), sagittal to the right of midline (below left) and sagittal midline (below right) T2 MRI without contrast of the chest shows a midline defect in the lower anterior chest wall with liver herniating through it.
Clinical image of Pentalogy of Cantrell
Clinical image shows the umbilical cord entering an omphalocele sac. The apex of the heart can be seen under the superior portion of the sac. The upper portion of the heart is covered by skin but the sternum is split.