Managing Duchenne muscular dystrophy--the additive effect of spinal surgery and home nocturnal ventilation in improving survival.

摘要

OBJECTIVES: To determine the long term survival in patients with Duchenne muscular dystrophy (DMD) following spinal surgery and nocturnal ventilation. STUDY DESIGN: A retrospective review of 100 consecutive patients born between 1970 and 1990 was conducted. RESULTS: Forty-seven patients had surgical spinal fusion, 27 were subsequently ventilated. Fourteen patients received ventilation only. Thirty-nine patients received neither intervention. The age at which ventilation was required correlated with the age at which ambulation was lost. Those who walked for longer were less likely to require spinal surgery. Mean vital capacity dropped from 1.4 to 1.13 L 1 year post-operatively. Patients having both spinal surgery and ventilation had a median survival of 30 years whereas those who were only ventilated survived to 22.2 years. CONCLUSION: Nocturnal ventilation improves survival in DMD. Spinal surgery does not increase forced vital capacity but in combination with nocturnal ventilation further improves median survival to 30 years.