[Sudden death in children and adolescents, carriers of an unrecognized cystic medionecrosis of the aorta].
Four instances of sudden death in childhood and adolescence with autopsy findings are presented. In the four cases unsuspected cystic medionecrosis of the aorta was discovered. In three of the four patients it was associated with a cardiovascular malformation, viz (1) double coarctation of the aorta, (2) coarctation of the aorta associated with Ebstein anomaly, and (3) subvalvular aortic stenosis. In three patients ocular abnormalities were demonstrated as an additional autopsy finding. Only one child could be said to have the Marfan habitus. We suggest that until new biological criteria emerge such observations should remain classified under the eponym of Marfan syndrome.
D000293:Adolescent; D000328:Adult; D001012:Aorta, Abdominal / Q000473:pathology; D001013:Aorta, Thoracic / Q000473:pathology; D001014:Aortic Aneurysm / Q000473:pathology; D001017:Aortic Coarctation / Q000473:pathology; D001018:Aortic Diseases / Q000473:pathology*; D001019:Aortic Rupture / Q000473:pathology; D001020:Aortic Stenosis, Subvalvular / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D003560:Cysts / Q000473:pathology*; D003645:Death, Sudden / Q000473:pathology*; D005260:Female; D006330:Heart Defects, Congenital / Q000473:pathology; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000473:pathology; D009131:Muscle, Smooth, Vascular / Q000473:pathology*; D009336:Necrosis
Décès brutal d'enfants et d'adolescents porteurs d'une médionécrose kystique de l'aorte méconnue.