" /> Cantrell pentalogy - CISMeF





Preferred Label : Cantrell pentalogy;

ICD-11 definition : Cantrell pentalogy is a malformation syndrome of unknown etiology that consists of the following five features: midline supraumbilical abdominal wall defects; deficiency of the anterior diaphragm; defects in the diaphragmatic pericardium; defects of the lower sternum and congenital intracardiac defects, with exteriorisation of the heart, or 'ectopia cordis', being the most severe malformation.;

ICD-11 synonym : Cantrell syndrome; Pentalogy of Cantrell;

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Cantrell pentalogy is a malformation syndrome of unknown etiology that consists of the following five features: midline supraumbilical abdominal wall defects; deficiency of the anterior diaphragm; defects in the diaphragmatic pericardium; defects of the lower sternum and congenital intracardiac defects, with exteriorisation of the heart, or 'ectopia cordis', being the most severe malformation.

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29/05/2025


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