Arterial manifestations of the Ehlers' Danlos syndrome
Beylot, C.; Bioulac, P.; Doutre, M.S.
Annales de Medecine Interne 134(5): 451-457
1983
ISSN/ISBN: 0003-410X PMID: 6651066 Document Number: 204773
The Ehlers-Danlos syndrome, or rather syndromes includes a heterogeneous group of diseases secondary to abnormalities of collagen fibres, for certain of which the specific biochemical defect has been identified. The preponderance of collagen fibers in the body explains the diversity of clinical manifestations which can occur as part of this form of hereditary connective tissue dysplasia. The involvement of the arteries is the most serious manifestation and determine the prognosis of the disease. The rupture of arteries, true vascular catastrophy occurring in young patients, is most characteristic of the type IV Ehlers-Danlos syndrome due to a deficiency in type III collagen. Aneurysms, and less frequently stenoses are also manifestations of the structural abnormality of the arterial walls. After a brief review of the other clinical manifestations of the Ehlers-Danlos syndrome, the authors analyse the Literature regarding the signs of arterial involvement in view of such a degree of arterial fragility, the authors underline the difficulties involved with surgery and the dangers of arteriography.