与厄普肖-舒尔曼综合征相关的双侧缺血性视网膜病变：一例报告
Bilateral Ischemic Retinopathy Associated With Upshaw-Schulman Syndrome: A Case Report.
作者信息
Cernichiaro-Espinosa Mariam, Meizner-Grezemkovsky Daniela, López-Santiago Norma, Borbolla-Pertierra Ana M, Morales-Cantón Virgilio, Cernichiaro-Espinosa Linda A
机构信息
Pediatric Retina Unit, Retina and Vitreous Department, Asociación para Evitar la Ceguera en México, I.A.P. Ciudad de México, Mexico.
Pediatric Onco-Hemathology Department, Instituto Nacional de Pediatría, Ciudad de México, Mexico.
出版信息
J Vitreoretin Dis. 2021 Oct 21;6(2):163-166. doi: 10.1177/24741264211049694. eCollection 2022 Mar-Apr.
PURPOSE
This is the first report to our knowledge of ischemic retinopathy in a pediatric patient with Upshaw-Schulman syndrome (USS).
METHODS
A 6-year-old girl previously diagnosed with USS was referred to our clinic with exodeviation of the left eye and a 2-month-long decrease in vision of both eyes. A dilated fundus examination showed a total vitreous hemorrhage in both eyes. The first course of action was conservative treatment, with the patient experiencing visual-acuity improvement in her right eye.
RESULTS
An ischemic retina and optic nerve atrophy was found once the left eye was cleared of the hemorrhage.
CONCLUSIONS
We present a case of a vitreous hemorrhage, possibly secondary to an episode of severe thrombocytopenia. Following USS diagnosis, providers should perform dilated ophthalmologic examinations as part of initial and follow-up general evaluations. This case exemplifies that, in understudied and underdescribed pediatric retinal diseases, extreme therapeutic decisions-such as surgery-should not be rushed.
目的
据我们所知，这是关于患有厄普肖-舒尔曼综合征（USS）的儿科患者发生缺血性视网膜病变的首例报告。
方法
一名先前被诊断为USS的6岁女孩因左眼外斜视和双眼视力下降2个月前来我院就诊。散瞳眼底检查显示双眼全玻璃体出血。首要治疗措施为保守治疗，患者右眼视力有所改善。
结果
左眼出血清除后，发现存在缺血性视网膜和视神经萎缩。
结论
我们报告了一例可能继发于严重血小板减少症发作的玻璃体出血病例。在诊断USS后，医疗人员应进行散瞳眼科检查，作为初始和后续综合评估的一部分。该病例表明，在研究不足和描述较少的儿科视网膜疾病中，不应仓促做出诸如手术等极端治疗决策。
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