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超乎常见病因:单侧视盘水肿作为Vogt-小柳-原田综合征罕见的首发症状

Beyond the Usual Suspects: Unilateral Optic Disc Edema as a Rare Initial Sign of Vogt-Koyanagi-Harada Syndrome.

作者信息

Rana Vipin, Sharma Vikas, Singh Kanwaljeet, Tripathi Amit Nandan, Goenka Ranjit, Markan Ashish

机构信息

Department of Ophthalmology, Command Hospital (Eastern Command), Kolkata, India.

Department of Ophthalmology, Air Force Central Medical Establishment (AFCME), Subroto Park, New Delhi, India.

出版信息

Rom J Ophthalmol. 2025 Apr-Jun;69(2):275-279. doi: 10.22336/rjo.2025.44.

DOI:10.22336/rjo.2025.44
PMID:40698104
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12277989/
Abstract

OBJECTIVE

To report a case of unilateral optic disc edema as a rare initial presentation of Vogt-Koyanagi-Harada (VKH) syndrome and emphasize the importance of early diagnosis using advanced imaging and cerebrospinal fluid analysis.

CASE PRESENTATION

We present the case of a 23-year-old male who initially presented with unilateral optic disc edema, retro-orbital pain, and headache, progressing to bilateral involvement with serous retinal detachments. Advanced imaging, including fundus fluorescein angiography (FFA), Indocyanine green angiography (ICG), and Enhanced depth imaging-Optical coherence tomography (EDI-OCT), revealed hallmark findings of VKH, such as choroidal granulomas and increased choroidal thickness. Cerebrospinal fluid analysis confirmed pleocytosis and melanin-laden macrophages, which helped to establish the diagnosis. The patient was treated with high-dose intravenous corticosteroids and azathioprine, with significant improvement.

DISCUSSION

VKH progresses through prodromal, acute uveitic, chronic, and recurrent phases. Although typically presenting with panuveitis, isolated optic disc edema as an initial sign is rare. Early diagnosis requires multimodal imaging and cerebrospinal fluid analysis to differentiate VKH from other inflammatory and infectious aetiologies.

CONCLUSION

This case highlights the importance of considering VKH in patients presenting with disc edema, whether unilateral or bilateral, particularly when accompanied by vitreous cells. Advanced ocular imaging and thorough systemic evaluation are critical for early diagnosis. Prompt treatment can prevent progression to chronic disease and irreversible vision loss.

摘要

目的

报告一例单侧视盘水肿作为Vogt-小柳-原田(VKH)综合征罕见初始表现的病例,并强调使用先进影像学检查和脑脊液分析进行早期诊断的重要性。

病例介绍

我们报告一例23岁男性患者,最初表现为单侧视盘水肿、眶后疼痛和头痛,随后进展为双侧受累并伴有浆液性视网膜脱离。包括眼底荧光血管造影(FFA)、吲哚菁绿血管造影(ICG)和增强深度成像光学相干断层扫描(EDI-OCT)在内的先进影像学检查显示了VKH的典型表现,如脉络膜肉芽肿和脉络膜厚度增加。脑脊液分析证实有淋巴细胞增多和含黑色素巨噬细胞,这有助于确诊。患者接受了大剂量静脉注射皮质类固醇和硫唑嘌呤治疗,病情有显著改善。

讨论

VKH综合征经历前驱期、急性葡萄膜炎期、慢性期和复发期。虽然通常表现为全葡萄膜炎,但孤立的视盘水肿作为初始体征较为罕见。早期诊断需要多模式成像和脑脊液分析,以将VKH与其他炎症性和感染性病因相鉴别。

结论

本病例强调了对于出现视盘水肿的患者,无论单侧或双侧,尤其是伴有玻璃体细胞时,考虑VKH综合征的重要性。先进的眼部成像和全面的全身评估对于早期诊断至关重要。及时治疗可预防疾病进展为慢性疾病和不可逆的视力丧失。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7ae/12277989/437d6c56c0a9/RomJOphthalmol-69-275-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7ae/12277989/10cfe25548dd/RomJOphthalmol-69-275-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7ae/12277989/347bc9a8e6a2/RomJOphthalmol-69-275-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7ae/12277989/437d6c56c0a9/RomJOphthalmol-69-275-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7ae/12277989/10cfe25548dd/RomJOphthalmol-69-275-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7ae/12277989/347bc9a8e6a2/RomJOphthalmol-69-275-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c7ae/12277989/437d6c56c0a9/RomJOphthalmol-69-275-g003.jpg

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Catching the therapeutic window of opportunity in early initial-onset Vogt-Koyanagi-Harada uveitis can cure the disease.在初发性Vogt-小柳-原田葡萄膜炎早期抓住治疗时机可治愈该病。
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