Pentalogy of Cantrell

Excerpt

Pentalogy of Cantrell (POC) is a collection of five congenital midline birth anomalies that present a distinctive challenge for providers and surgeons. Those five defects are of the heart, pericardium, diaphragm, sternum, and abdominal wall. This condition has been divided into two categories, complete or partial. Complete, as the name indicates, refers to the presence of all five defects, while others may present with only partial defects. It is also referred to as thoracoabdominal ectopia cordis, a condition where the heart is covered by an omphalocele-like membrane. Ectopia cordis (EC) is often found in fetuses with POC. Infants usually have multiple cardiac malformations with ventricular septal defect, and tetralogy of Fallot being the most common. POC may also carry genetic associations with trisomy 13, 18, 21, and Turner syndrome. The initial management addresses the lack of skin overlying the heart and abdominal cavity.

Following initial management, additional surgery involve covering the midline defects, separating the abdominal and pericardial compartment, and repairing the diaphragm. Advanced reconstructive techniques are utilized for additional closure, including the use of flaps, skin closure only, and bioprosthetic agents. The intracardiac defects are often repaired at a later date. Survival depends on the associated cardiac anomalies and degree of thoracoabdominal defect.