ABSTRACT

Purpose

To describe disease characteristics and outcomes of Vogt-Koyanagi-Harada (VKH) syndrome in paediatric patients.

Study Design

Retrospective chart analysis

Methods: A retrospective

Analysis of all patients ≤16 years with VKH syndrome was done. Clinical presentations, complications, recurrences and outcomes in cases of paediatric VKH were reviewed.

Results

72 eyes of 36 patients with a mean age at presentation of 13.7 ± 2.34 years were assessed. Mean duration of symptoms and follow up were 9.88 ± 17.3 weeks and 55 months respectively. Clinical signs at presentation included anterior chamber cells >2+(34/72eyes, 47.2%), granulomatous keratic precipitates (6 eyes, 8.3%), posterior synechiae (35 eyes,48.6%), disc edema (46 eyes, 63.8%), neurosensory retinal detachments (44 eyes, 61.1%) and ‘sunset-glow’ fundus (9 eyes, 12.5%). Best corrected visual acuity (BCVA) at the time of presentation was 1.3logMAR or a Snellens equivalent of 20/400 which improved to 0.51logMAR (Snellens equivalent of 20/63) at last follow up. Remission was achieved in 61.1% cases. More than half of our patients developed one or more complications.

Conclusion

VKH in paediatric patients poses a challenge due to a delayed presentation and paediatric VKH patients have a worse visual acuity at the time of presentation as compared to adult age groups. Rates of remission may be low along with high risk of complications and hence there is a need for prolonged immunosuppression.

中文翻译：

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儿科年龄组的 Vogt Koyanagi Harada 病：亚洲印度人群的临床特征、缓解、复发和并发症

摘要

目的

描述儿科患者 Vogt-Koyanagi-Harada (VKH) 综合征的疾病特征和结果。

学习规划

回顾性图表分析

方法：回顾性

对所有≤16 岁的 VKH 综合征患者进行了分析。回顾了儿童 VKH 病例的临床表现、并发症、复发和结果。

结果

评估了 36 名患者的 72 只眼，平均年龄为 13.7 ± 2.34 岁。症状和随访的平均持续时间分别为 9.88 ± 17.3 周和 55 个月。就诊时的临床体征包括前房细胞>2+（34/72眼，47.2%）、肉芽肿性角化沉淀物（6眼，8.3%）、后粘连（35眼，48.6%）、椎间盘水肿（46眼，63.8%） ，神经感觉视网膜脱离（44 只眼，61.1%）和“日落辉光”眼底（9 只眼，12.5%）。就诊时的最佳矫正视力 (BCVA) 为 1.3logMAR 或 20/400 的 Snellens 等效值，最后一次随访时改善为 0.51logMAR（Snellens 等效值 20/63）。61.1% 的病例达到缓解。我们超过一半的患者出现了一种或多种并发症。

结论

由于延迟出现，儿科患者的 VKH 带来了挑战，并且与成人年龄组相比，儿科 VKH 患者在就诊时的视力较差。缓解率可能较低，并发症风险较高，因此需要延长免疫抑制。