Ventilatory Management of Muscular Dystrophy Patients following Spinal Fusion

Abstract

Pediatric patients with various forms of dystrophic muscle disease can develop pro-gressive spinal curvature, resulting in increasing respiratory compromise. Spinal fusion is most often considered when significant scoliosis has developed, accompanied by severe reduction in pulmonary function. The potential for developing ventilatory failure in the postoperative period is high. The consequences of lung damage and airway injury due to prolonged positive-pressure ventilatory assistance and an ar-tificial airway are significant. In an alternative ventilatory management program, we used the Emerson tank ventilator on six pediatric patients with vital capacities of < 60% of predicted. The program included a preoperative period of assessment and education lasting at least 5 days and consisting of baseline pulmonary function testing, arterial blood gas and end-tidal CO₂ analysis, adaptation to negative pressure inside the tank ventilator, IPPB therapy, incentive breathing exercises, and chest physical therapy. During the immediate postoperative period (24-48 hours) the patients re-quired nasotracheal intubation and positive-pressure ventilatory support for the pur-pose of initial stabilization and wound evaluation. Transfer to the Emerson tank ventilator and subsequent extubation avoided long-term complications associated with positive-pressure ventilation and an artificial airway. All patients were weaned from the tank ventilator within 14 days for a maximum recovery time of 17 days of mechan-ical ventilatory support. The use of the Emerson tank ventilator thus allowed an extremely valuable surgical procedure to be performed with an acceptable risk and with reduced potential for the significant lung damage that can occur from positive-pressure ventilatory support and an artificial airway over a prolonged period of time.