The Natural History of Cardiac and Pulmonary Function Decline in Patients With Duchenne Muscular Dystrophy
- 1 July 2011
- journal article
- deformity
- Published by Ovid Technologies (Wolters Kluwer Health) in Spine
- Vol. 36 (15), E1009-E1017
- https://doi.org/10.1097/brs.0b013e3181fea1ed
Abstract
Study Design. Retrospective review of scoliosis progression, pulmonary and cardiac function in a series of patients with Duchenne Muscular Dystrophy (DMD). Objective. To determine whether operative treatment of scoliosis decreases the rate of pulmonary function loss in patients with DMD. Summary of Background Data. It is generally accepted that surgical intervention should be undertaken in DMD scoliosis once curve sizes reach 35° to allow intervention before critical respiratory decline has occurred. There are conflicting reports, however, regarding the effect of scoliosis stabilization on the rate of pulmonary function decline when compared to nonoperative cohorts. Methods. We reviewed spinal radiographs, echocardiograms, and spirometry, hospital, and operative records of all patients seen at our tertiary referral center from July 1, 1992 to June 1, 2007. Data were recorded to Microsoft Excel (Microsoft, Redmond, WA) and analyzed with SAS (SAS Institute, Cary, NC) and R statistical processing software ( www.r-project.org ). Results. The percent predicted forced vital capacity (PPFVC) decreased 5% per year before operation. The mean PPFVC was 54% (SD = 21%) before operation with a mean postoperative PPFVC of 43% (SD = 14%). Surgical treatment was associated with a 12% decline in PPFVC independent of other treatment variables. PPFVC after operation declined at a rate of 1% per year and while this rate was lower, it was not significantly different than the rate of decline present before operation (P = 0.18). Cardiac function as measured by left ventricular fractional shortening declined at a rate of 1% per year with most individuals exhibiting a left ventricular fractional shortening rate of more than 30 before operation. Conclusion. Operative treatment of scoliosis in DMD using the Luque Galveston method was associated with a reduction of forced vital capacity related to operation. The rate of pulmonary function decline after operation was not significantly reduced when compared with the rate of preoperative forced vital capacity decline.Keywords
This publication has 29 references indexed in Scilit:
- Posterior Spinal Fusion for Scoliosis in Duchenne Muscular Dystrophy Diminishes the Rate of Respiratory DeclineSpine, 2007
- Comparison of Pelvic Fixation Techniques in Neuromuscular Spinal Deformity Correction: Galveston Rod Versus Iliac and Lumbosacral ScrewsSpine, 2006
- Iliosacral Screw Fixation for Pelvic Obliquity in Neuromuscular ScoliosisSpine, 1997
- Effect of spinal surgery on lung function in Duchenne muscular dystrophy.Thorax, 1995
- Duchenne Muscular DystrophyAmerican Journal of Physical Medicine & Rehabilitation, 1995
- Spinal fusion in duchenne muscular dystrophy: A multidisciplinary approachMuscle & Nerve, 1992
- Characterization of Dystrophin in Muscle-Biopsy Specimens from Patients with Duchenne's or Becker's Muscular DystrophyNew England Journal of Medicine, 1988
- Analysis of deletions in DNA from patients with Becker and Duchenne muscular dystrophyNature, 1986
- The treatment of scoliosis in duchenne muscular dystrophyMuscle & Nerve, 1984
- Respiratory function in the muscular dystrophiesMuscle & Nerve, 1981