Managing Duchenne muscular dystrophy – The additive effect of spinal surgery and home nocturnal ventilation in improving survival

Abstract 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 su...

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Published inNeuromuscular disorders : NMD Vol. 17; no. 6; pp. 470 - 475
Main Authors Eagle, Michelle, Bourke, John, Bullock, Robert, Gibson, Mike, Mehta, Jwalant, Giddings, Dave, Straub, Volker, Bushby, Kate
Format Journal Article
LanguageEnglish
Published England Elsevier B.V 01.06.2007
Subjects
Adolescent
Adult
Cohort Studies
Disease Progression
Duchenne muscular dystrophy
Humans
Kaplan-Meier Estimate
Male
Muscular Dystrophy, Duchenne - mortality
Muscular Dystrophy, Duchenne - physiopathology
Muscular Dystrophy, Duchenne - therapy
Neurology
Nocturnal ventilation
Respiration, Artificial
Retrospective Studies
Scoliosis
Spinal Fusion
Spinal surgery
Survival
Vital Capacity - physiology
Walking - physiology
Nocturnal ventilation
Scoliosis
Spinal surgery
Survival
Duchenne muscular dystrophy
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Summary:Abstract 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.
Bibliography:ObjectType-Article-1
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ISSN:0960-8966
1873-2364
DOI:10.1016/j.nmd.2007.03.002