Ectopia cordis represents one end of the spectrum
of anomalies, close examination of such patients often reveals further
features of the pentalogy of Cantrell. Pentalogy of Cantrell is comprised of
(1) midline supra-umbilical abdominal defect, (2) sternal fusion defects,
(3) deficiency or absence of the diaphragmatic pericardium, (4) deficiency
of the anterior diaphragm, and (5) congenital cardiac disease (atrial septal
defect [ASD], ventricular septal defect [VSD], tetralogy of Fallot). Mildly
hypoplastic lungs are a common finding. Additional malformations may include
hydrocephalus, anencephaly, cleft lip/palate, cystic hygroma, transposition
of the great vessels, patent ductus arteriosus, ventral hernia, omphalocele,
renal agenesis, and hypospadias.