Department of Ophthalmology and Visual Sciences, Vancouver General Hospital, University of British Columbia, Vancouver, BC, Canada.
J Endocrinol Invest. 2021 Mar;44(3):421-429. doi: 10.1007/s40618-020-01361-y. Epub 2020 Jul 29.
Dysthyroid optic neuropathy (DON) is a serious complication of Graves orbitopathy (GO) from optic nerve dysfunction that may result in permanent loss of vision.
This paper reviews the current knowledge of DON, including its pathogenesis and epidemiology, clinical and radiologic features, and management choices and outcomes.
Literature review and author retrospective case series.
Over 90% of DON cases are related to nerve compression by enlarged extraocular muscles (EOM) while the remainder are caused by stretching of the optic nerve without compression. DON's incidence is 5-8% of GO cases. Risk factors include advancing age, male gender, smoking and diabetes mellitus, and these cases should be referred promptly to an ophthalmologist or GO clinic to rule out DON and for ongoing care. Clinical features of DON may include reduction in central and colour vision (unexplained by other ocular disorders), afferent pupil defect and/or optic disc edema. Since most cases are associated with enlarged EOM, restricted motility and soft tissue venous congestion are common. Visual fields and optical coherence tomography (OCT) help confirm the diagnosis while CT or MRI Scans show apical optic nerve compression or proptosis with optic nerve stretch. Standard therapy includes iv/oral corticosteroids (CS) with partial response in most cases, but often relapse with tapering. Radiotherapy may delay or avoid surgery and may prevent the onset of DON when combined with CS in high-risk individuals. The benefits of newer biologic targeted therapy are not clear. Orbital decompression surgery often has positive outcomes, even in cases of severe vision loss or delayed surgery. The most common surgical complication is worsening strabismus, which may worsen visual function and quality of life. In rare cases, permanent vision loss from DON may occur despite full therapy.
Although DON may cause vision loss, most cases are reversible if recognized and managed in a timely manner.
甲状腺眼病(GO)引起的压迫性视神经病变(DON)是一种严重的并发症,会导致视神经功能障碍,进而导致视力永久性丧失。
本文综述了 DON 的相关知识,包括其发病机制和流行病学、临床和影像学特征、治疗选择和结局。
文献回顾和作者回顾性病例系列。
超过 90%的 DON 病例与眼外肌(EOM)肿胀引起的神经受压有关,其余病例则是由于视神经拉伸而非受压所致。DON 的发病率约占 GO 病例的 5-8%。危险因素包括年龄增长、男性、吸烟和糖尿病,这些病例应及时转至眼科医生或 GO 门诊,以排除 DON 并进行持续治疗。DON 的临床特征可能包括中央和色觉减退(排除其他眼部疾病所致)、传入性瞳孔缺陷和/或视盘水肿。由于大多数病例与 EOM 增大有关,因此运动受限和软组织静脉充血较为常见。视野检查和光学相干断层扫描(OCT)有助于确诊,而 CT 或 MRI 扫描则显示视盘上方的视神经受压或眶内突出伴视神经拉伸。标准治疗包括静脉/口服皮质类固醇(CS),多数情况下有部分反应,但在减量时经常复发。放射治疗可能会延迟或避免手术,并可能在高危人群中与 CS 联合使用时预防 DON 的发生。新型生物靶向治疗的益处尚不清楚。眼眶减压手术通常有良好的疗效,即使在视力严重丧失或手术延迟的情况下也是如此。最常见的手术并发症是斜视加重,这可能会使视觉功能和生活质量恶化。在极少数情况下,即使进行了充分的治疗,DON 仍可能导致永久性视力丧失。
尽管 DON 可能导致视力丧失,但如果及时识别和治疗,大多数病例是可以逆转的。