[Lesions of the aortic arch in Recklinghausen's disease]

Two young patients with Von Recklinghausen's disease developed an obstructive aortic arch syndrome. Follow-up over several years showed progressive changes in the clinical signs with no development of symptoms. Angiography demonstrated great changes in the anatomical relationship of the supraaortic trunks which lead to surgical repair. In one case, the histology of operative biopsies established a relation between the neurofibromatous and the arterial lesions. Modern investigative procedures and therapeutic possibilities are reviewed in the light of these two cases. These two patients seem to be the first reported cases of neurofibromatosis causing the aortic arch syndrome.