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Surgical treatment of spinal deformities in spinal muscular atrophy: a single-center experience from China

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Abstract

Purpose

To report the clinical characteristics and surgical outcomes of scoliosis in patients with spinal muscular atrophy (SMA) from Mainland China.

Methods

Nineteen patients were retrospectively analyzed. Demographic, anthropometric and respiratory parameters were collected preoperatively. Surgical program was analyzed. Radiographic data were measured perioperatively. Motor status, ventilation support, sitting ability and respiratory symptoms were evaluated preoperatively and at final follow-up.

Results

Age at surgery was 17.08 (12.83, 20.08) years. More than 40% of patients were diagnosed with low weight. Pulmonary dysfunction was observed in all patients. All patients received posterior spinal fusion (PSF). Sacroiliac fixation with sacral-2 alar iliac technique was used in 16 patients. Major curve correction rate was 54.87 ± 16.14%. Pelvic obliquity correction rate was 63.84 ± 23.70%. T1–T12 height, space-available-for-lung ratio and thoracic transverse diameter were increased (p < 0.001). Percentage of patients capable of sitting independently increased from 26.32% preoperatively to 73.68% at final follow-up. Cumulative scores of sitting-related items in muscular dystrophy spine questionnaire improved from 19.11 ± 5.40 preoperatively to 26.21 ± 5.20 at final follow-up. Total scores of symptomatic domains in St. George’s Respiratory Questionnaire decreased from 4 (2, 12) preoperatively to 1 (0, 3) at final follow-up.

Conclusions

SMA patients in China always present severe scoliosis at late adolescence, accompanied with high proportion of low weight and pulmonary dysfunction. PSF is effective for the correction of scoliosis and pelvic obliquity and the improvement of thoracic morphology. Sitting ability and respiratory symptoms were improved postoperatively.

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Data availability

The datasets generated during the current study are available from the corresponding author on reasonable request.

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Acknowledgements

We thank Huanhuan Sha from Jiangsu Cancer Hospital for language editing.

Funding

This study was funded by the National Natural Science Foundation of China (Grant Numbers: 81974354, 81772424).

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Authors and Affiliations

  1. Department of Orthopedics, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, People’s Republic of China

    Zhen Wang, Erwei Feng, Yang Jiao, Jiachen Lin, Junduo Zhao & Jianxiong Shen

  2. Department of Anesthesiology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, People’s Republic of China

    Weiyun Chen

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  1. Zhen Wang
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Contributions

WC and JS contributed to conceptualization and project administration; ZW was involved in data curation, formal analysis, visualization and writing—original draft; JS contributed to funding acquisition; YJ was involved in methodology; and EF, YJ, JL and JZ contributed to writing—review and editing.

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Correspondence to Weiyun Chen or Jianxiong Shen.

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Wang, Z., Feng, E., Jiao, Y. et al. Surgical treatment of spinal deformities in spinal muscular atrophy: a single-center experience from China. Eur Spine J 31, 3089–3097 (2022). https://doi.org/10.1007/s00586-022-07347-z

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