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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.

The paper presents a review of 28 domestic and foreign publications with the level of evidence of not less than 2 reporting the treatment results in children with congenital monosegmental anomalies of the spine over the past 15 years. A total of 657 clinical cases were analyzed, including 593 literature cases and 64 cases from authors' experience. Results of hemivertebra treatment in children using combined approach, extended posterior approach and local posterior approach with transpedicular excision of hemivertebra were analyzed. The following criteria were used for analysis: patient's age at the time of treatment, magnitude of the local (segmental) curvature before and after surgery, percentage of correction, blood loss volume, duration of surgery, type and length of fixation, and nature and structure of complications. The advantages of transpedicular excision of abnormal vertebra through posterior approach were noted in terms of surgery duration, intraoperative blood loss, risk of neurological complications after comparable correction of deformity, and time required for patient activation and rehabilitation as compared with combined approach, as well as in terms of blood loss volume and correction of kyphotic component as compared with extended posterior approach.