Pentalogy of Cantrell

Etiology: Anterior abdominal wall defect with defect in lower sternum and defect in anterior diaphragm and defect in diaphragmatic portion of pericardium
Imaging:
— Omphalocele
— Ectopia cordis
— Diaphragmatic defect
— Pericardial defect or sternal cleft
— Congenital heart disease – ventricular septal defect, atrial septal defect, tetralogy of Fallot, left ventricular diverticulm
DDX:
Complications:
Treatment: Surgical
Clinical:

Radiology 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 Cases of Pentalogy of Cantrell

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.

Surgical Cases of Pentalogy of Cantrell

Surgical image of Pentalogy of Cantrell — Surgical image after the omphalocele sac was excised shows the apex of the heart (superiorly) and the liver (inferiorly). The pericardium was intact. The diaphragm was intact. The omphalocele was closed by approximating the abdominal fascia. The heart was covered with skin.