Fauroux Brigitte, Khirani Sonia, Griffon Lucie, Teng Theo, Lanzeray Agathe, Amaddeo Alessandro
Pediatric Non-invasive Ventilation and Sleep Unit, AP-HP, Hôpital Necker-Enfants Malades, Paris, France.
Université de Paris, VIFASOM, Paris, France.
Front Pediatr. 2020 Nov 16;8:482. doi: 10.3389/fped.2020.00482. eCollection 2020.
The respiratory muscles are rarely spared in children with neuromuscular diseases (NMD) which puts them at risk of alveolar hypoventilation. The role of non-invasive ventilation (NIV) is then to assist or "replace" the weakened respiratory muscles in order to correct alveolar hypoventilation by maintaining a sufficient tidal volume and minute ventilation. As breathing is physiologically less efficient during sleep, NIV will be initially used at night but, with the progression of respiratory muscle weakness, NIV can be extended during daytime, preferentially by means of a mouthpiece in order to allow speech and eating. Although children with NMD represent the largest group of children requiring long term NIV, there is a lack of validated criteria to start NIV. There is an agreement to start long term NIV in case of isolated nocturnal hypoventilation, before the appearance of daytime hypercapnia, and/or in case of acute respiratory failure requiring any type of ventilatory support. NIV is associated with a correction in night- and daytime gas exchange, an increase in sleep efficiency and an increase in survival. NIV and/or intermittent positive pressure breathing (IPPB) have been shown to prevent thoracic deformities and consequent thoracic and lung hypoplasia in young children with NMD. NIV should be performed with a life support ventilator appropriate for the child's weight, with adequate alarms, and an integrated (±additional) battery. Humidification is recommended to improve respiratory comfort and prevent drying of bronchial secretions. A nasal interface (or nasal canula) is the preferred interface, a nasobuccal interface can be used with caution in case of mouth breathing. The efficacy of NIV should be assessed on the correction of alveolar ventilation. Patient ventilator synchrony and the absence of leaks can be assessed on a sleep study with NIV or on the analysis of the ventilator's in-built software. The ventilator settings and the interface should be adapted to the child's growth and progression of respiratory muscle weakness. NIV should be associated with an efficient clearance of bronchial secretions by a specific program on the ventilator, IPPB, or mechanical insufflation-exsufflation. Finally, these children should be managed by an expert pediatric multi-disciplinary team.
患有神经肌肉疾病（NMD）的儿童，其呼吸肌很少能幸免，这使他们面临肺泡通气不足的风险。无创通气（NIV）的作用是辅助或“替代”衰弱的呼吸肌，通过维持足够的潮气量和分钟通气量来纠正肺泡通气不足。由于睡眠时呼吸的生理效率较低，NIV最初将在夜间使用，但随着呼吸肌无力的进展，NIV可在白天延长使用，优先通过口含器进行，以便能说话和进食。虽然患有NMD的儿童是需要长期NIV的最大儿童群体，但启动NIV缺乏经过验证的标准。对于孤立的夜间通气不足，在出现日间高碳酸血症之前，和/或对于需要任何类型通气支持的急性呼吸衰竭情况，一致同意启动长期NIV。NIV与夜间和日间气体交换的改善、睡眠效率的提高以及生存率的增加相关。NIV和/或间歇性正压通气（IPPB）已被证明可预防患有NMD的幼儿的胸廓畸形以及随之而来的胸廓和肺发育不全。应使用适合儿童体重的生命支持呼吸机进行NIV，要有适当的警报器和集成（±额外）电池。建议进行湿化以提高呼吸舒适度并防止支气管分泌物干燥。鼻面罩（或鼻导管）是首选的接口，在口呼吸的情况下可谨慎使用口鼻面罩。NIV的疗效应根据肺泡通气的纠正情况进行评估。患者与呼吸机的同步性以及有无漏气可通过NIV睡眠研究或呼吸机内置软件分析来评估。呼吸机设置和接口应根据儿童的生长和呼吸肌无力的进展进行调整。NIV应与通过呼吸机上的特定程序、IPPB或机械吸气-呼气进行的有效支气管分泌物清除相结合。最后，这些儿童应由专业的儿科多学科团队进行管理。
