Surgical Correction of Spine Deformity in Patients with Spinal Muscular Atrophy: Multicenter Analysis of Domestic Experience and Literature Review

Abstract

Purpose: to analyze the domestic experience in surgical correction of spine deformity in patients with spinal muscular atrophy (SMA). Patients and methods. Retrospective multicenter nonrandomized study (evidentiary level III) was performed. Treatment results were analyzed for 26 patients aged 6 - 25 years who were operated on at four RF centers. Inclusion criteria: patients with neurogenic scoliosis on a background of SMA, genetically confirmed diagnosis of type II and III SMA, frontal-plane Cobb angle over 40°, availability of radiologic archives. Results. Preoperative scoliotic curve ranged from 40° to 135° (mean 92°), postoperatively - from 10° to 92° (mean 52°). Correction index varied within 13-75% (mean 40%). Frontal balance correction made up 23%, frontal pelvic tilt - 17%. Indices of external expiration functions showed a slight increase. Functional status by GMFCS was determined preoperatively as class 4 in 2 (8.3%) and class 5 in 24 (91.7%) patients; after intervention as class 10 (38.5%) and 16 (61.5%) patients, respectively. Conclusion. Patients with axial skeleton deformity on a background of SMA are at high risk group and require multidisciplinary examination and peri/postoperative management. Surgical correction is indicated in decompensated deformity over 40°. Surgical rehabilitation improves the self-care of patients as well as the life quality of both the patients and their surroundings.

Frontal and Sagittal Patterns of Neuromuscular Spinal Deformities Based on Nosological Profile

“…4 Most studies show improvement in the quality of patients' and caregivers' life and caregivers' satisfaction. [23][24][25][26] They found that most of the patient caregivers felt that those patients treated surgically were more comfortable than those who were not. Not all research results demonstrated significant changes in the quality of life of the patients or improvement in vital signs.…”