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Background <p>Spinocerebellar ataxia (SCA) and Friedreich’s ataxia (FA) frequently give rise to progressive scoliosis, which impairs respiratory and postural function. Although spinal fusion is the most reliable method for correcting deformities, perioperative neurological vulnerability and monitoring failures complicate decision-making. This study combines a systematic review with a detailed pediatric case to clarify current evidence on surgical management, complications, and outcomes.</p> Methods <p>We present a 12-year-old girl who underwent posterior fusion (T4–L4) for presumed adolescent idiopathic scoliosis. Early proximal junctional kyphosis (PJK) prompted planned revision, which was terminated after the intraoperative loss of somatosensory evoked potentials (SSEPs). Subsequent imaging revealed cerebellar atrophy consistent with SCA. Definitive surgery extended the construct to T2 and incorporated a T4 pedicle subtraction osteotomy. Persistent gait and balance deficits were managed with intensive rehabilitation. In parallel, a PRISMA-compliant systematic search of PubMed and Embase (2000–May 2025) identified pediatric studies reporting scoliosis surgery in SCA or FA. Four studies (105 patients) met the inclusion criteria and were analyzed for surgical strategy, neuromonitoring performance, and complications.</p> Results <p>In the index case, the unreliability of the SSEP necessitated staged correction; transient unilateral lower limb weakness resolved partially over 6 months. A literature synthesis revealed PJK in 23–25% of patients and high SSEP failure rates (up to 91%); nevertheless, no permanent neurological injuries were reported. Infection (≤ 4%) and pseudarthrosis were infrequent, whereas one cardiopulmonary death occurred. Motor evoked potential (MEP) is seldom employed but may offer greater sensitivity. Disease severity, rather than surgical variables alone, influences long-term functional outcomes.</p> Conclusions <p>Spinal fusion can be performed safely in pediatric SCA/FA patients when meticulous multidisciplinary planning, individualized neuromonitoring strategies, and aggressive postoperative rehabilitation are adopted. Early recognition of underlying ataxia is critical for risk stratification and the timing of intervention. Prospective, longitudinal studies are warranted to refine guidelines and optimize neurological and orthopedic results in this complex population.</p>

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Progressive scoliosis in a pediatric patient with cerebellar ataxia: surgical challenges and literature review

  • 期刊论文

  • 发表日期：

    2025年12月15日

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  • Emanuela Asunis,
  • Francesca Vitulli,
  • Roberto Nicotra,
  • Alfonso Rubino,
  • Domenico Cicala,
  • Giuseppe Cinalli,
  • Gianluca Colella

摘要

Background

Spinocerebellar ataxia (SCA) and Friedreich’s ataxia (FA) frequently give rise to progressive scoliosis, which impairs respiratory and postural function. Although spinal fusion is the most reliable method for correcting deformities, perioperative neurological vulnerability and monitoring failures complicate decision-making. This study combines a systematic review with a detailed pediatric case to clarify current evidence on surgical management, complications, and outcomes.

Methods

We present a 12-year-old girl who underwent posterior fusion (T4–L4) for presumed adolescent idiopathic scoliosis. Early proximal junctional kyphosis (PJK) prompted planned revision, which was terminated after the intraoperative loss of somatosensory evoked potentials (SSEPs). Subsequent imaging revealed cerebellar atrophy consistent with SCA. Definitive surgery extended the construct to T2 and incorporated a T4 pedicle subtraction osteotomy. Persistent gait and balance deficits were managed with intensive rehabilitation. In parallel, a PRISMA-compliant systematic search of PubMed and Embase (2000–May 2025) identified pediatric studies reporting scoliosis surgery in SCA or FA. Four studies (105 patients) met the inclusion criteria and were analyzed for surgical strategy, neuromonitoring performance, and complications.

Results

In the index case, the unreliability of the SSEP necessitated staged correction; transient unilateral lower limb weakness resolved partially over 6 months. A literature synthesis revealed PJK in 23–25% of patients and high SSEP failure rates (up to 91%); nevertheless, no permanent neurological injuries were reported. Infection (≤ 4%) and pseudarthrosis were infrequent, whereas one cardiopulmonary death occurred. Motor evoked potential (MEP) is seldom employed but may offer greater sensitivity. Disease severity, rather than surgical variables alone, influences long-term functional outcomes.

Conclusions

Spinal fusion can be performed safely in pediatric SCA/FA patients when meticulous multidisciplinary planning, individualized neuromonitoring strategies, and aggressive postoperative rehabilitation are adopted. Early recognition of underlying ataxia is critical for risk stratification and the timing of intervention. Prospective, longitudinal studies are warranted to refine guidelines and optimize neurological and orthopedic results in this complex population.