Barois A
Service Pédiatrie, Hôpital Raymond Poincaré, Garches.
Bull Acad Natl Med. 1999;183(4):721-30.
In kyphoscoliosis restrictive ventilatory defect occurs. In idiopathic scoliosis vital capacity failure is significantly correlated with Cobb angle, vertebral rotation, and thoracic lordosis. Maximum voluntary ventilation is the most affected measurement. Forced expiratory volume in 1 second is reduced. Residual volume remains longtime normal. Hypoxemia due to decrease of diffusing capacity occurs, with initially reflex hyperventilation hypocapnia, and secondary hypercapnia. Pulmonary hypertension and cor pulmonale is related to hypoventilation and hypoxia. The lung situated on the concave side of the scoliosis curve shows a more functional derangement. Ventilatory pattern consists of low tidal volume and high respiratory rate with increase of ventilatory work. Scoliosis that appears in the earlier stage of the life has the worst respiratory prognosis (before 5 years of age) with impairement of lung and thoracic growth. To stimulate pulmonary and thoracic growth, intermittent ventilatory assistance by pressure preset ventilator should be performed as soon as possible and pursued up to 8 years of age, at least, more if necessity. In over 60 degrees angle idiopathic scoliosis, respiratory failure appears after 40 to 50 years of age. Non invasive ventilatory assistance with preset pressure ventilator by oral way in moderate cases and nocturnal nasal ventilation by volume ventilator or inspiratory assistance ventilator, in the most severe cases are efficient. In very severe and acute respiratory insufficiency (scoliosis over 90 degrees) ventilation by intubation then tractheostomy may be required. Earlier orthopedic management and surgical procedure to correct and stabilize spinal deformities is the best to prevent respiratory insufficiency. For scoliosis below 60 degrees, post operative pulmonary complications are very low, with no requirement of post operative ventilatory support. In very severe respiratory insufficiency treatment of respiratory failure precedes, and follows, orthotic treatment and surgical procedures; it shouldle pursued afterwards.
脊柱后凸侧弯时会出现限制性通气功能障碍。在特发性脊柱侧弯中,肺活量衰竭与 Cobb 角、椎体旋转和胸椎后凸显著相关。最大自主通气量是受影响最明显的指标。一秒用力呼气量降低。残气量长期保持正常。由于弥散功能下降会出现低氧血症,起初会有反射性过度通气导致的低碳酸血症,继而出现高碳酸血症。肺动脉高压和肺源性心脏病与通气不足和缺氧有关。位于脊柱侧弯曲线凹侧的肺功能紊乱更严重。通气模式表现为潮气量低、呼吸频率高,通气功增加。在生命早期出现的脊柱侧弯(5岁前)呼吸预后最差,会影响肺和胸廓的生长。为刺激肺和胸廓生长,应尽快使用压力预设通气机进行间歇性通气辅助,并至少持续到8岁,如有必要可延长。在特发性脊柱侧弯角度超过60度时,40至50岁后会出现呼吸衰竭。中度病例采用经口压力预设通气机进行无创通气辅助,最严重的病例采用容积通气机或吸气辅助通气机进行夜间鼻通气有效。在非常严重的急性呼吸功能不全(脊柱侧弯超过90度)时,可能需要插管然后行气管造口术进行通气。早期进行骨科治疗和手术矫正及稳定脊柱畸形是预防呼吸功能不全的最佳方法。对于60度以下的脊柱侧弯,术后肺部并发症非常低,无需术后通气支持。在非常严重的呼吸功能不全时,呼吸衰竭的治疗先于、伴随并在矫形治疗和手术之后进行;之后应持续进行。