Pentalogy of Cantrell

Pentalogy (a combination of five closely related usually simultaneous defects or symptoms)

Pentalogy of Cantrell
- is a rare syndrome
- characteristics :

1. Omphalocele
- abdominal wall defect at the base of the umbilical cord (umbilicus);
- infant is born with sac protruding through the defect which
- contains small intestine, liver, and large intestine.

2. Anterior diaphragmatic hernia
- an abnormal opening in the diaphragm
- that allows part of the abdominal organs to migrate into the chest cavity

3. Sternal cleft
- Fissure of the sternum

4. Ectopia cordis
- heart is exposed on the chest wall
- due to maldevelopment of the sternum and pericardium

5. Intracardiac defect:
- either a ventricular septal defect or
- a diverticulum (a pouch or sac opening) of the left ventricle