Panayiotopoulos Y P, Tyrrell M R, Koffman G, Reidy J F, Haycock G B, Taylor P R
Department of Surgery, Guy's Hospital, London, UK.
Br J Surg. 1996 Feb;83(2):235-40.
Mid-aortic syndrome (MAS) is an uncommon condition characterized by segmental narrowing of the proximal abdominal aorta and ostial stenosis of its major branches. It is usually diagnosed in young adults, but may present in childhood as a challenging problem. Over the past 20 years 13 patients with MAS have presented to this institution. All had hypertension, four had associated neurofibromatosis, three persistent eosinophilia and three had Williams syndrome. In all cases arteriography showed a smooth segmental narrowing of the abdominal aorta with concomitant stenosis at the origins of the renal arteries. Six children were successfully treated with antihypertensive medication alone. Percutaneous transluminal angioplasty was attempted in two cases with poor result. Surgery was indicated in seven children with refractory hypertension and progressive renal impairment. Techniques used to revascularize the kidneys included thoracoabdominal to infrarenal aortic bypass with renal artery reimplantation, splenorenal bypass, gastroduodenal to renal bypass, aortorenal bypass and autotransplantation.
中段主动脉综合征（MAS）是一种罕见的疾病，其特征为腹主动脉近端节段性狭窄及其主要分支开口处狭窄。该病通常在年轻成年人中被诊断出来，但在儿童期也可能作为一个具有挑战性的问题出现。在过去20年里，有13例MAS患者前来本机构就诊。所有患者均患有高血压，4例伴有神经纤维瘤病，3例有持续性嗜酸性粒细胞增多，3例患有威廉姆斯综合征。在所有病例中，血管造影显示腹主动脉有平滑的节段性狭窄，同时肾动脉起始处有狭窄。6名儿童仅通过抗高血压药物治疗即获成功。2例尝试了经皮腔内血管成形术，但效果不佳。7名患有难治性高血压和进行性肾功能损害的儿童接受了手术治疗。用于使肾脏血管再通的技术包括胸腹主动脉至肾下主动脉旁路移植术并肾动脉再植术、脾肾旁路移植术、胃十二指肠至肾旁路移植术、主动脉肾旁路移植术和自体肾移植术。
