Abstract：
Louis-Bar Syndrome is a synonym for a very rare complex neurodegenerative disorder ataxia-telangiectasia (A-T). This is an autosomal recessive inherited disease that encompasses abnormalities in the cerebellum, multisystem degeneration, immunodeficiency, increased risk for malignancy and consecutive respiratory insufficiency. Most of the patients are radiosensitive and any exposing to ionization may lead to progression of the disease. Potential risks from anesthesia, mechanical ventilation, and postoperative complications in these patients have been insufficiently discussed in the literature. We present a successful anesthetic and respiratory management with one-lung ventilation in a patient with Louis-Bar Syndrome who underwent video assisted thoracoscopy (VATS) for recurrent pleural effusion.
摘要：
Louis-Bar综合征是非常罕见的复杂神经退行性疾病共济失调-毛细血管扩张症（A-T）的同义词。这是一种常染色体隐性遗传性疾病，包括小脑异常，多系统变性，免疫缺陷,恶性肿瘤和连续呼吸功能不全的风险增加。大多数患者对放射敏感，任何暴露于电离都可能导致疾病的进展。麻醉的潜在风险，机械通气,这些患者的术后并发症在文献中没有得到充分讨论。我们为一名接受电视辅助胸腔镜检查（VATS）治疗复发性胸腔积液的Louis-Bar综合征患者提供了成功的单肺通气麻醉和呼吸管理。
