Pentalogy of Cantrell
ORPHA:1335· ICD-10 Q89.7
Definition
Pentalogy of Cantrell (POC) is a lethal multiple congenital anomalies syndrome, characterized by the presence of 5 major malformations: midline supraumbilical abdominal wall defect, lower sternal defect, diaphragmatic pericardial defect, anterior diaphragmatic defect and various intracardiac malformations. Ectopia cordis (EC) is often found in fetuses with POC.
- Prevalence
- 1-9 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- Antenatal, Neonatal