Lewis J R, Glaser J S, Schatz N J, Hutson D G
Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami School of Medicine, Florida 33101.
J Clin Neuroophthalmol. 1993 Dec;13(4):242-9.
Pulseless disease (PD) is a rare disorder in which inflammation of the aorta and its major branches leads to stenosis or occlusion of these arteries. It mainly affects young Oriental women, who suffer chronic ischemic injury to tissues of the brain, orbits, upper limbs, myocardium, and kidneys. The ophthalmologic features of pulseless disease tend to be late manifestations, and can include ischemia of the retina, choroid, and anterior segment. The inflammatory process may be reversed in early stages with systemic corticosteroids, but, more frequently, significant arterial stenosis necessitates arterial bypass surgery. A 59-year-old Caucasian woman with stenosis of all four major cervical arteries presented with recurrent blurred vision, syncope, mental obtundation, and a remarkable funduscopic appearance due to bilateral orbital hypoperfusion. Her acute symptoms improved slightly on high-dose systemic corticosteroids, and then resolved completely following arterial bypass surgery.
无脉症（PD）是一种罕见疾病，其中主动脉及其主要分支的炎症会导致这些动脉的狭窄或闭塞。它主要影响年轻的东方女性，这些女性会遭受大脑、眼眶、上肢、心肌和肾脏组织的慢性缺血性损伤。无脉症的眼科特征往往是晚期表现，可能包括视网膜、脉络膜和眼前节的缺血。在早期阶段，全身性皮质类固醇可能会使炎症过程逆转，但更常见的是，严重的动脉狭窄需要进行动脉搭桥手术。一名59岁的白种女性，四条主要颈动脉均出现狭窄，因双侧眼眶灌注不足而出现反复视力模糊、晕厥、精神迟钝以及显著的眼底表现。她在大剂量全身性皮质类固醇治疗下急性症状稍有改善，随后在动脉搭桥手术后完全缓解。
