Pentalogy of Cantrell

Orpha code: 1335OMIM code: 313850

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.

Disease data
Classification

Malformation syndrome

Synonyms
Cantrell deformity
Deformacja Cantrella
Zespół Cantrella
Zespół piersiowo-brzuszny
Cantrell syndrome
Thoraco-abdominal syndrome
ORPHA code
1335
OMIM code
313850
ICD10 code
Q89.7
ICD11 code
LD2F.1Y

No additional description.

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