Life threatening hypoventilation in kyphoscoliosis: successful treatment with a molded body brace-ventilator.

We report sustained successful treatment of end-stage hypoventilation in a 37-yr-old patient with kyphoscoliosis caused by childhood poliomyelitis. The patient presented with progressive ventilatory failure, pure restrictive lung function, and no concurrent illness. He first responded to respiratory stimulation with medroxyprogesterone acetate and aminophylline. Because of skeletal deformity and the need for a chronic brace he was then fitted with a customized polyethylene foam body brace with an anterior free space and connecting hole for a Monaghan ventilator. No longer taking any medication, and with only nocturnal assisted ventilation, he now has normal daytime arterial blood gas values 20 months later. This approach may be useful in other patients with severe kyphoscoliosis.