Pediatric Spine Foundation

Title: Impact of Growth Friendly Interventions on Spine and Pulmonary Outcomes of Patients with Spinal Muscular Atrophy Publication Summary

Authors: Ishaan Swarup, Elle M. MacAlpine, Oscar H. Mayer, Robert K. Lark, John T. Smith, Michael G. Vitale, John M. Flynn, Jason B. Anari, Pediatric Spine Study Group, Patrick J. Cahill

Journal: European Spine Journal

Date: August 01, 2020

Excerpt: Patients with spinal muscular atrophy (SMA) are often treated with growth-friendly devices such as vertical expandable prosthetic titanium rib (VEPTR) and magnetically controlled growing rods(MCGR) to correct spinal deformity and improve pulmonary function. There is limited data on this topic, and the purpose of this study was to assess the effect of these constructs and the addition of chest wall support (CWS) on spinal deformity, chest structure and pulmonary outcomes.

Keywords: Spinal muscular atrophy, Early-onset scoliosis, Thoracic insufficiency syndrome, Vertical expandable prosthetic titanium rib, Growing rod

Summary:

Purpose

This study aims to explore the effects of growth-friendly treatment on patients with Spinal Muscular Atrophy (SMA), with and without the addition of Chest Wall Support.

Results

  • In the study of 66 patients, the average Cobb angle, or spinal curvature improved from 67° to 50° after 2 years in patients with Chest Wall Support, and from 59° to 46° at 2 years in patients without Chest Wall Support.
  • 15 patients were treated with Chest Wall support. There was no difference in improvement in spinal curvature at 2-year follow-up between patients with and without CWS, however patients treated with CWS showed improved chest width, while those without CWS showed no improvement.
  • Growth friendly constructs improved spinal deformity and may be effective in altering the progression toward respiratory failure in patients with SMA. Patients treated with Chest Wall Support had significant improvements in chest structure and growth compared to patients without CWS. Chest wall support may be a helpful option in addition to growth-friendly treatment to prevent respiratory issues in patients with SMA.

Discussion Points

  • The complication rate during treatment was 38%, 24% in patients treated with magnetically controlled growing rods and 45% in patients treated with a VEPTR device.
  • This study has concluded that Chest Wall Support improves chest width, however further research must be conducted to determine the effects of this improvement on lung function and rib deformity.