神经肌肉疾病患者无创呼吸支持期间的喉镜检查评估：单中心服务评价

文献信息

中文摘要

引言： 无创通气（NIV）可改善神经肌肉疾病（NMD）患者的症状和生存，但一部分患者出现NIV不耐受，尤其是伴有延髓受累者。正压呼吸支持可能在易感个体中诱发动态喉部狭窄，这一现象被称为通气诱发的喉梗阻（V-ILO）。呼吸支持期间的连续喉镜检查可能有助于识别V-ILO并指导呼吸机设置的优化。
方法： 这项单中心观察性服务评价连续纳入确诊NMD且出现上气道症状、需要内镜评估的患者。在NIV、机械性吸-呼气（MI-E）或CPAP期间进行连续喉镜检查，适用于症状提示对正压呼吸支持不耐受者。在直视下评估喉部行为、分泌物负荷以及对呼吸机调整的反应。
结果： 共纳入49例患者；48例接受了喉镜检查。21例因NIV不耐受或相关症状而在正压呼吸支持期间接受连续喉镜检查。12例显示V-ILO，其特征为不适当的动态声门和/或声门上狭窄，主要发生在吸气期。这12例患者中有9例在喉镜引导优化后成功建立NIV，优化包括在直视下调整吸气压力和/或上升时间，以确定最初可耐受的支持水平和模式。随后在临床中滴定至治疗压力，未再出现临床可识别的V-ILO。
结论： V-ILO是神经肌肉疾病患者NIV不耐受的一种潜在可逆原因。呼吸支持期间的连续喉镜检查可提供有临床价值的信息，指导呼吸机优化、改善NIV耐受性，并为分泌物负荷和其他喉部异常的管理提供依据。这些发现支持在专科家庭机械通气服务中进一步评估喉镜引导的优化。

英文摘要

INTRODUCTION: Non-invasive ventilation (NIV) improves symptoms and survival in neuromuscular disease (NMD), but a subgroup of patients experience NIV intolerance, particularly those with bulbar involvement. Positive-pressure respiratory support may provoke dynamic laryngeal narrowing in susceptible individuals, a phenomenon termed ventilation-induced laryngeal obstruction (V-ILO). Continuous laryngoscopy during respiratory support may help identify V-ILO and guide optimisation of ventilator settings.
METHODS: This single-centre observational service evaluation included consecutive patients with confirmed NMD who presented with upper-airway symptoms warranting endoscopic assessment. Continuous laryngoscopy was performed during NIV, mechanical insufflation-exsufflation (MI-E) or CPAP where symptoms suggested intolerance of positive-pressure respiratory support. Laryngeal behaviour, secretion burden and response to ventilator adjustments were assessed under direct visualisation.
RESULTS: Forty-nine patients were included; 48 underwent laryngoscopy. Twenty-one underwent continuous laryngoscopy during positive-pressure respiratory support because of NIV intolerance or related symptoms. Twelve demonstrated V-ILO, characterised by inappropriate dynamic glottic and/or supraglottic narrowing, predominantly during inspiration. Nine of these twelve patients were successfully established on NIV following laryngoscopy-guided optimisation, which involved identifying an initially tolerable level and pattern of support by adjusting inspiratory pressure and/or rise time under direct visualisation. Subsequent titration to therapeutic pressures was performed clinically, with no recurrence of clinically recognised V-ILO.
CONCLUSION: V-ILO represents a potentially reversible cause of NIV intolerance in patients with neuromuscular disease. Continuous laryngoscopy during respiratory support provides clinically useful information that can guide ventilator optimisation, improve tolerance of NIV and inform management of secretion burden and other laryngeal abnormalities. These findings support further evaluation of laryngoscopy-guided optimisation within specialist home mechanical ventilation services.