[Vogt Koyanagi Harada syndrome in children: diagnosis and management].

N Benfdil, T Baha Ali, B Jellab, A Moutaouakil
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Abstract

Uveitis accounts for 5% to 10% of all cases of uveitis in children. Vogt-Koyanagi-Harada syndrome affects children very unfrequently. We report a case of a 14-year-old girl who presented with a bilateral uveitis with posterior predominance associated with an important serous retinal detachment and signs of meningeal irritation. No specific cause could be identified. The initial treatment consisted in administration of high dose steroids given in i.v. bolus at the dose of 10 mg/Kg/day then in oral steroids at the dose of 1 mg/Kg/day with slow tapering during 1 year. The evolution was marked by an excellent recuperation of the visual acuity, a total disappearance of the serous retinal detachment, the development of choroidal depigmentation and the occurrence of poliosis. This evolution was in favour of a Vogt-Koyanagi-Harada syndrome. The diagnosis of this syndrome is usually difficult to establish at its onset. Most often the evolution allows to correct and confirm the diagnosis. Megadoses of corticosteroid therapy and cytotoxic agents may be necessary. The visual prognosis may be poor in severe cases.

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儿童Vogt Koyanagi Harada综合征的诊断和治疗。
葡萄膜炎占所有儿童葡萄膜炎病例的5%至10%。Vogt-Koyanagi-Harada综合征很少发生在儿童身上。我们报告一个病例14岁的女孩谁提出了双侧葡萄膜炎与后部优势相关的重要浆液性视网膜脱离和脑膜刺激的迹象。目前还无法确定具体原因。最初的治疗是静脉注射高剂量类固醇,剂量为10mg /Kg/天,然后口服类固醇,剂量为1mg /Kg/天,在1年内缓慢减少。进化的标志是视力恢复良好,浆液性视网膜脱离完全消失,脉络膜色素沉着的发展和脊髓灰质炎的发生。这种进化有利于Vogt-Koyanagi-Harada综合征。这种综合征的诊断通常很难在发病时确定。大多数情况下,这种进化允许纠正和确认诊断。可能需要大剂量的皮质类固醇治疗和细胞毒性药物。严重者视力预后可能较差。
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