Vascular Manifestations in Patients With Ehlers-Danlos Syndrome

Abstract
• Ehlers-Danlos syndrome (EDS) is clinically and genetically a heterogenous disorder of connective tissue synthesis. Seven clinical types of this disease have been identified and the underlying biochemical defects defined in types IV through VII. Unfortunately, most patients with major vascular complications of EDS have few, if any, of the commonly recognized musculoskeletal and cutaneous abnormalities. Recognition of the correct diagnosis and the application of accepted vascular surgical techniques may improve the morbidity and mortality for these patients. (Arch Surg 1982;117:495-498)