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伏格特-小柳-原田综合征(葡萄膜脑膜脑炎综合征)。

Vogt-Koyanagi-Harada syndrome (uveomeningoencephalitic syndrome).

作者信息

Manethova Katerina, Ernest Jan, Hrevus Michal

机构信息

Department of Ophthalmology, Central Military University Hospital, Prague - Czech Republic.

出版信息

Eur J Ophthalmol. 2017 Jan 19;27(1):e5-e8. doi: 10.5301/ejo.5000902.

Abstract

PURPOSE

To report a case of a 29-year-old man who was examined at the Eye Clinic of Central Military University Hospital Prague for a severe headache and acute blurring of vision in both eyes diagnosed as incomplete Vogt-Koyanagi-Harada syndrome (VKH).

METHODS

This is a retrospective and descriptive case report based on data from clinical records, patient observation and follow-ups and analysis of acquired diagnostic tests.

RESULTS

A 29-year-old man presented with headache and decreased vision in his left eye (LE) for 2 days. Best-corrected visual acuity was 20/20 in both his eyes. Pupillary function, intraocular pressure, results of external segment examinations, and slit-lamp biomicroscopy were normal bilaterally. Right eye funduscopy was normal; in the LE, funduscopy revealed posterior pole exudative retinal detachments. Optical coherence tomography confirmed multiple serous (bullous) retinal detachments and showed thickening of the posterior choroid also revealed by orbital ultrasound. Fluorescein angiography showed angiographic features typical of VKH disease: disseminated spotted choroidal hyperfluorescence and choroidal multifocal hypofluorescence, multifocal profuse leakage in the retina with late phase pooling, serous retinal detachment, and optic disc hyperfluorescence. All serologic tests for the diagnosis of infectious pathologies were negative. Except for severe headache, the patient did not have any neurologic, integumentary, or auditory manifestations. Immediate high-dose systemic corticosteroid treatment was given.

CONCLUSIONS

Despite prompt diagnosis, prompt aggressive systemic therapy, and a rapid response in this case, progressive pigmentary changes of retinal pigment epithelium developed shortly thereafter.

摘要

目的

报告一例29岁男性患者,其因严重头痛和双眼急性视力模糊在布拉格中央军事大学医院眼科诊所接受检查,被诊断为不完全型小柳原田综合征(VKH)。

方法

这是一份基于临床记录、患者观察及随访数据以及所获得的诊断检查分析的回顾性描述性病例报告。

结果

一名29岁男性患者出现头痛及左眼视力下降2天。双眼最佳矫正视力均为20/20。双侧瞳孔功能、眼压、眼外段检查结果及裂隙灯生物显微镜检查均正常。右眼眼底检查正常;左眼眼底检查发现后极部渗出性视网膜脱离。光学相干断层扫描证实存在多处浆液性(大疱性)视网膜脱离,并显示后脉络膜增厚,眼眶超声检查也发现了这一情况。荧光素血管造影显示出VKH病典型的血管造影特征:弥漫性斑点状脉络膜高荧光和脉络膜多灶性低荧光、视网膜多灶性大量渗漏伴晚期积血、浆液性视网膜脱离及视盘高荧光。所有用于诊断感染性病变的血清学检查均为阴性。除严重头痛外,患者无任何神经、皮肤或听觉表现。立即给予大剂量全身糖皮质激素治疗。

结论

尽管本病例诊断及时、积极进行了全身治疗且反应迅速,但此后不久仍出现了视网膜色素上皮的进行性色素沉着改变。

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