Related Experiment Video
Updated: Dec 11, 2025
Home-Based Prescribed Pulmonary Exercise in Patients with Stable Chronic Obstructive Pulmonary Disease
Published on: August 24, 2019
Improvement of Pulmonary Function Measured by Patient-reported Outcomes in Patients With Spinal Muscular Atrophy
Hiroko Matsumoto1, John Mueller1, Matthew Konigsberg1
1Department of Orthopedic Surgery, Columbia University Medical Center, New York, NY.
Background:
Children with spinal muscular atrophy (SMA) sustain a progressive reduction in pulmonary function (PF) related to both muscular weakness and the concomitant effects of spinal deformity on the thorax. Growth-friendly instrumentation is commonly utilized for younger patients with scoliosis and SMA to halt the progression of spinal curvature, but its effect on PF in these patients has not previously been investigated. Using the change in Early Onset Scoliosis 24-Item Questionnaire (EOSQ-24) PF subdomain scores, the authors will investigate whether PF improves in patients with SMA after a growth-friendly intervention.
Methods:
This was a multicenter retrospective cohort study from 2 international registries of patients with SMA undergoing spinal deformity surgery from 2005 to 2015. Data collected were age, sex, degree of major coronal curve, type of growth-friendly construct, forced vital capacity (FVC), and EOSQ-24 scores at the patient's preoperative, 1-year postoperative, and 2-year postoperative visits. Differences in EOSQ-24 PF scores and FVC between baseline and postoperative assessment were examined by paired tests.
Results:
A total of 74 patients were identified (mean age, 7.6±2.3 y, major curve 68.1±22.4 degrees, 51.4% female individuals). The mean EOSQ-24 PF scores improved significantly from 70.6 preoperatively to 83.6 at 1 year (P=0.092) and 86.5 at 2 years postoperatively (P=0.020). The scores in patients with rib-based constructs showed steeper increases at 1-year assessments than those in patients with spine-based constructs. The mean paired FVC value decreased from 63.9% predicted preoperatively, to 57.6% predicted at 1 year postoperatively (P=0.035), and 61.9% predicted preoperatively, to 56.3% predicted at 2 years postoperatively (P=0.178).
Conclusions:
Patients with SMA who received growth-friendly instrumentation did experience improvements in PF as measured by EOSQ-24 assessing the caregivers' perception. Given the uncertain reliability of PFTs in this young population, EOSQ-24 is an important tool for measuring improvements in health-related quality of life.
Level Of Evidence:
Level III-retrospective study.
More Related Videos
06:28Biomechanical Changes Related to Low Back Pain: An Innovative Tool for Movement Pattern Assessment and Treatment Evaluation in Rehabilitation
Published on: December 13, 2024
09:18Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
Published on: January 12, 2019
Related Concept Videos
01:25Pulmonary Function Tests
Pulmonary Function Tests are crucial diagnostic tools for assessing respiratory function, particularly in patients with chronic respiratory disorders. They comprehensively evaluate lung volumes, ventilatory function, breathing mechanics, diffusion, and gas exchange. These tests help diagnose pulmonary diseases and play a significant role in monitoring disease progression, evaluating disability, and assessing response to therapy.
PFTs involve using a spirometer, a...
01:27Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies
Medical History