A rare disease with an obscure etiology is described. It affects predominantly the aorta and occasionally some of its branches, producing a decrease on the lumen vessel. In our experience it has been diagnosed in four young patients and the most important symptom has been arterial hypertension. In three cases the thoracic aorta was involved, with aneurysmal dilatation in one of them. In the rest the thoracic as well as the abdominal aorta were involved. All of them were treated surgically; on three, a latero lateral by pass was performed and in the fourth an aneurysmal resection was done and the aorta reconstructed with a dacron prosthesis. The clinical follow up 4 1/2 years later, showed normal arterial pressure, and patent by-passes, on angiography. We conclude that the revascularization technique can change the natural history of this disease.
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