Pentalogy of Cantrell

**Definition:** Rare congenital deformity syndrome characterized by a combination of five anomalies as a result of neural tube defect. The five anomalies are a midline supraumbilical abdominal wall defect (e.g., OMPHALOCELE), a lower STERNUM defect, a congenital intracardiac defect, an anterior DIAPHRAGM defect, and a diaphragmatic PERICARDIUM defect (e.g., PERICARDIAL EFFUSION). Variants with incomplete and variable combinations of the defects are known. ECTOPIA CORDIS; CLEFT LIP; and CLEFT PALATE are often associated with the syndrome.

**Tree numbers:** - C10.500.680.705 - C16.131.077.696 - C16.131.666.680.705

**Synonyms:** - Cantrell Haller Ravitch syndrome - Cantrell Pentalogy - Cantrell's Pentalogy - Thoracoabdominal Syndrome