Spinal deformities in spinal muscular atrophy in the era of disease-modifying therapy

Czech version

Authors: M. Barna; J. Štulík
Authors‘ workplace: Klinika spondylochirurgie, 1. LF UK a FNMH, Praha
Published in: Cesk Slov Neurol N 2026; 89(S1): 32-36
doi: https://doi.org/10.48095/cccsnn2026S32

Overview

Spinal deformity is one of the most common musculoskeletal complications of spinal muscular atrophy (SMA), particularly in non-ambulant patients. Disease-modifying therapies have profoundly changed survival and motor phenotypes but have not eliminated scoliosis. Early treatment may slow deformity progression in some patients, while improved survival has created a new population with historically severe phenotypes, particularly SMA type 1, who may develop early progressive scoliosis. Surgical decision-making is therefore shifting from algorithms based mainly on SMA type, age and Cobb angle towards individualized assessment of motor function, curve progression, remaining growth, pelvic obliquity and respiratory reserve. Growth-friendly instrumentation can be considered in skeletally immature children, whereas definitive posterior instrumented correction and spinal fusion remain standard in older patients. In patients receiving nusinersen, preservation of long-term intrathecal access is an additional surgical consideration. This review summarizes current evidence and places it in the context of our institutional experience with growth-friendly treatment, definitive scoliosis correction and surgically created access for intrathecal therapy.

Keywords:

spinal muscular atrophy – spinal fusion – scoliosis – nusinersen – disease-modifying therapy – neuromuscular scoliosis

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