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Pentalogy of Cantrell

Disease 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.


Classification level: Disorder
  • Synonym(s):
    • Cantrell deformity
    • Cantrell syndrome
    • Thoraco-abdominal syndrome
  • Prevalence: <1 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Neonatal, Antenatal
  • ICD-10: Q89.7
  • ICD-11: LD2F.1Y
  • OMIM: 313850
  • UMLS: C0559483
  • MeSH: D058502
  • GARD: 7359
  • MedDRA: -
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