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首页> 外文期刊>Seminars in cutaneous medicine and surgery. >Sturge-Weber syndrome.
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Sturge-Weber syndrome.

机译：斯特奇-韦伯综合征。

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摘要

Sturge-Weber syndrome is a sporadic neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities (glaucoma and choroidal hemangioma) and leptomeningeal angioma. Although the precise pathogenesis is unknown, available data regarding genetics, embryogenesis, and pathologic features are briefly reviewed. Clinical features vary from mild incomplete forms to full-blown disease with facial stain, seizures, and glaucoma. Frequencies of associated complications are reviewed. To plan treatment and further follow-up, diagnosis of glaucoma and intracranial involvement, even if asymptomatic, is fundamental in children at risk. Early neuroimaging features are important to recognize. Management of patients with Sturge-Weber syndrome is focused on treating associated neurologic and ocular abnormalities.
机译：Sturge-Weber综合征是一种偶发性神经皮肤疾病，其特征是面部酒斑，眼部异常（青光眼和脉络膜血管瘤）和软脑膜血管瘤。尽管确切的发病机制尚不清楚，但简要回顾了有关遗传，胚胎发生和病理特征的可用数据。临床特征从轻度不完整形式到具有面部色斑，癫痫发作和青光眼的全面疾病。复习相关并发症的频率。为了计划治疗和进一步的随访，对有风险的儿童来说，即使没有症状，诊断青光眼和颅内受累也是至关重要的。早期的神经影像学特征很重要。患有Sturge-Weber综合征的患者的治疗重点是治疗相关的神经系统和眼部异常。

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