title: Managing Duchenne muscular dystrophy--the additive effect of spinal surgery and home nocturnal ventilation in improving survival.
abstract: OBJECTIVE
  To determine the long term survival in patients with Duchenne muscular dystrophy (DMD) following spinal surgery and nocturnal ventilation.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: University of Newcastle and Newcastle upon Tyne Hospitals Trust, New Castle Muscle Centre, Institute of Genetics, Center for Life, Central Parkway, Newcastle, NE13BZ, UK. Michelle.Eagle@ncl.ac.uk
authors.forename: Michelle
authors.initials: M
authors.lastname: Eagle
authors.forename: John
authors.initials: J
authors.lastname: Bourke
authors.forename: Robert
authors.initials: R
authors.lastname: Bullock
authors.forename: Mike
authors.initials: M
authors.lastname: Gibson
authors.forename: Jwalant
authors.initials: J
authors.lastname: Mehta
authors.forename: Dave
authors.initials: D
authors.lastname: Giddings
authors.forename: Volker
authors.initials: V
authors.lastname: Straub
authors.forename: Kate
authors.initials: K
authors.lastname: Bushby
mesh_terms: D000293:Adolescent; D000328:Adult; D015331:Cohort Studies; D018450:Disease Progression; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000401:mortality* / Q000503:physiopathology / Q000628:therapy*; D012121:Respiration, Artificial; D012189:Retrospective Studies; D013123:Spinal Fusion; D014797:Vital Capacity / Q000502:physiology; D016138:Walking / Q000502:physiology