Sousa Uva M, Bical O, Voung P N, Gigou F, Foiret J C, Braunberger E, Vanetti A
Service de chirurgie cardiovasculaire, hôpital Saint-Joseph, Paris.
Arch Mal Coeur Vaiss. 1994 Sep;87(9):1233-6.
Fibromuscular dysplasia is a non-inflammatory disease unrelated to atherosclerosis of the small and medium sized arterial walls, which often affects the renal and carotid arteries and occurs mainly in women. The authors report a case of atypical coarctation of the thoracic aorta due to fibromuscular dysplasia confirmed histologically in a 27 year old man. Arteriography was performed because of hypertension and asymmetry of blood pressure measurements and showed irregular stenosis of the aortic isthmus and of the origin of the left subclavian artery. Surgery comprised resection of the pathological segment of the aorta which was replaced by a prosthetic tube with reimplantation of the left subclavian artery. Histological examination showed fibromuscular dysplasia in perimedial areas and diffusely throughout the media. To the author's knowledge this is the first case to be reported in the medical literature.
纤维肌发育不良是一种与中小动脉壁动脉粥样硬化无关的非炎症性疾病，常累及肾动脉和颈动脉，主要发生于女性。作者报告了一例27岁男性因纤维肌发育不良导致胸主动脉非典型缩窄的病例，该病例经组织学确诊。因高血压和血压测量不对称而行动脉造影，显示主动脉峡部及左锁骨下动脉起始部不规则狭窄。手术包括切除主动脉病变节段，并用人工血管置换，同时将左锁骨下动脉重新植入。组织学检查显示中膜周围区域及整个中膜弥漫性纤维肌发育不良。据作者所知，这是医学文献中报道的首例此类病例。
