Center of Sleep Disorders, Medical School of Athens University, Department of Critical Care and Pulmonary Services, Evangelismos Hospital, Athens, Greece.
J Clin Sleep Med. 2010 Dec 15;6(6):609-12.
Congenital central hypoventilation syndrome (CCHS) is an uncommon disorder characterized by the absence of adequate autonomic control of respiration, which results in alveolar hypoventilation and decreased sensitivity to hypercarbia and hypoxemia, especially during sleep. Patients with CCHS need lifelong ventilatory support. The treatment options for CCHS include intermittent positive pressure ventilation administered via tracheostomy, noninvasive positive pressure ventilation, negative-pressure ventilation by body chamber or cuirass, and phrenic nerve pacing. However, it may be necessary to alter the mode of ventilation according to age, psychosocial reasons, complications of therapy, and emergence of new modes of ventilation. We present a case of a 16-year-old girl with CCHS who was mechanically ventilated via tracheostomy for 16 years and was successfully transitioned to a new modality of noninvasive ventilation (average volume-assured pressure support [AVAPS]) that automatically adjusts the pressure support level in order to provide a consistent tidal volume.
先天性中枢性低通气综合征（CCHS）是一种罕见的疾病，其特征是呼吸的自主控制不足，导致肺泡通气不足和对高碳酸血症及低氧血症的敏感性降低，尤其是在睡眠期间。CCHS 患者需要终身通气支持。CCHS 的治疗选择包括经气管切开间歇性正压通气、无创正压通气、体腔或胸甲负压通气以及膈神经起搏。然而，可能需要根据年龄、社会心理原因、治疗并发症以及新通气模式的出现来改变通气模式。我们报告了 1 例 16 岁女性 CCHS 患者，该患者经气管切开机械通气 16 年，成功过渡到新的无创通气模式（平均容量保证压力支持[AVAPS]），该模式自动调整压力支持水平，以提供一致的潮气量。
