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Tratado de Cirurgia da Coluna Vertebral
SECTION 4 • Spinal Deformities
Chapter30

Neuromuscular Scoliosis

Vancouver: Aguiar CA, Cordeiro LEB📖 Pages: 411-422
Full reading of this chapter is available exclusively in the printed edition of the Treatise.
Sec. 4Spinal Deformities
Cap. 30Clinical Chapter
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Chapter Summary

• Context: Neuromuscular scoliosis is a spinal deformity resulting from underlying neurological or muscular disorders that disrupt postural control and the trunk balance of forces. Unlike idiopathic scoliosis, it typically manifests earlier, progresses more rapidly, and continues to deteriorate even after skeletal maturity. The curves frequently involve extensive thoracolumbar segments and propagate to the sacropelvis, generating severe pelvic obliquity, coronal and sagittal trunk decompensation, and major difficulties with sitting balance, positioning, personal hygiene, and skin integrity. The condition is intimately linked with respiratory, nutritional, and systemic impairment, the magnitude of which varies according to the underlying disorder. Consequently, therapeutic decision-making cannot rely solely on the radiographic Cobb angle. Etiology, gross motor function, ambulatory status, pulmonary reserve, comorbidities, patient quality of life, and caregiver burden must be evaluated in an integrated fashion. The chapter emphasizes a highly individualized, multidisciplinary decision-making model.
• Chapter Objective: To present the core foundations for the clinical assessment, multidisciplinary optimization, and surgical management of neuromuscular scoliosis. The reader will learn to distinguish neuropathic from myopathic etiologies, apply structural and functional classification systems (such as GMFCS and FMS), recognize the biomechanical and functional impact of pelvic obliquity, conduct comprehensive preoperative risk assessments, understand the indications for growth-friendly versus definitive posterior fusion techniques, and anticipate major perioperative complications and realistic functional expectations.
• Etiology and functional status define the pathologyThe chapter divides neuromuscular scoliosis into neuropathic and myopathic forms. Neuropathic etiologies arise from central, spinal, peripheral, or mixed neural compromise, including cerebral palsy, myelomeningocele, and spinal muscular atrophy. Myopathic etiologies stem from primary muscle disorders, notably Duchenne and other muscular dystrophies. This distinction is critical because curve behavior, trunk muscle balance, pulmonary decline, and the degree of functional dependence vary substantially according to the underlying disease.
• Classification beyond plain radiographsThe Lonstein and Akbarnia classification categorizes curve patterns and trunk balance in patients with cerebral palsy. In parallel, the chapter emphasizes functional scales. The Gross Motor Function Classification System (GMFCS), shown in Figure 2, categorizes gross motor impairment. The Functional Mobility Scale (FMS), presented in Figure 3, provides granular data on assisted mobility across home, school, and community settings. These tools connect radiographic deformity to the patient's real-world functional capacity.
• Multidisciplinary preoperative optimizationSurgical planning must address the spine and pelvis, but equally prioritize the respiratory system, nutritional status, cardiac function, seizure control, bone mineral density, and caregiver support. Plain radiographs in sitting and supine traction positions evaluate trunk balance and flexibility; CT and MRI are utilized according to etiology and instrumentation requirements. Preoperative pulmonary evaluation is paramount due to the high incidence of postoperative respiratory complications. However, the chapter notes that moderate-to-severe pulmonary restriction is not an absolute contraindication if risks are thoroughly optimized and communicated.
• Pelvic obliquity and functional reconstructive goalsPelvic obliquity represents one of the most critical elements in surgical planning, particularly in non-ambulatory patients. Its clinical sequelae include unstable sitting balance, iliocostal impingement, ischial pressure sores, and increased caregiver physical strain. The decision to extend instrumentation to the pelvis must weigh functional level, magnitude and rigidity of obliquity, primary diagnosis, and the risk of revision surgery. The chapter discusses ongoing controversies and stresses that pelvic fixation should not be decided by a single radiographic parameter.
• Surgical strategies and reconstructionIn very young children, growth-friendly instrumentation may be considered, although the chapter highlights high complication and reoperation rates, recommending stringent indications. Whenever feasible, definitive fusion near the adolescent growth spurt is preferred to avoid repeated surgical interventions in clinically fragile patients. Rigid posterior segmental pedicle screw fixation forms the foundation of modern deformity correction. Severe, rigid curves may require posterior column osteotomies (PCO), three-column osteotomies (PSO/VCR), preoperative halo-gravity traction, or combined approaches. The primary surgical objective is not a 'normal' radiograph, but establishing a balanced, stable trunk over a level pelvis to enhance sitting comfort, respiratory mechanics, skin care, and overall quality of life.
• Clinical Application: In clinical practice, the evaluation of a patient with neuromuscular scoliosis must start with what the patient can actually do, rather than the Cobb angle alone. Sitting tolerance, transfer ability, self-feeding, hygiene, upper extremity functional use, and wheelchair positioning must be systematically documented alongside GMFCS and FMS scores. Pelvic obliquity should be examined directly in the patient's customized wheelchair and functional posture. Before indicating surgery, the multidisciplinary team must establish whether progressive deformity is actively compromising pulmonary function, sitting stability, skin integrity, comfort, or daily caregiving. The operative risk must be weighed against the natural history of unchecked deformity progression. Preoperative workup mandates nutritional optimization (often via gastrostomy), pulmonary conditioning, and cardiac evaluation (particularly in muscular dystrophies and syndromic conditions). In severe, neglected curves, preoperative halo-gravity traction can safely loosen the deformity, improve nutritional and pulmonary status, and reduce intraoperative neurological risk. In non-ambulatory patients, sacropelvic fixation (such as S2AI screws) is frequently required, whereas in ambulatory patients, preserving lumbar mobility and avoiding fusion to the pelvis protects gait mechanics. Family and caregivers must be aligned from the outset with realistic goals: surgery aims to restore sitting balance, improve head and trunk control, alleviate pain, and simplify caregiving, but cannot restore motor function lost to the underlying neuromuscular disease.
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Keywords

Preferred DeCS/MeSH Descriptors:
ScoliosisNeuromuscular DiseasesCerebral PalsySpinal Muscular Atrophies of ChildhoodSpinal FusionActivities of Daily LivingQuality of Life
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Why this chapter matters

A technically dramatic radiographic correction offers little real-world benefit if the patient remains unable to sit comfortably, develops pressure ulcers, or suffers respiratory failure. Conversely, establishing a stable, balanced trunk over the pelvis can profoundly improve daily life even without altering motor function. This chapter shifts the decision-making focus from the Cobb angle to the patient's functional and systemic reality. By integrating mobility, respiration, nutrition, pelvic obliquity, and caregiver realities, it provides a comprehensive framework to determine not just how to operate, but who, when, and why to treat.

“In neuromuscular scoliosis, functional capacity and the primary disease process are just as decisive as the radiographic deformity. Surgical decision-making must synthesize curve progression, trunk balance, pelvic obliquity, ambulatory status, pulmonary and nutritional reserve, comorbidities, and the daily burden on caregivers. Although surgery is complex and carries significant perioperative risks, appropriately indicated and multidisciplinarily planned spinal reconstruction provides transformative improvements in sitting stability, comfort, and patient/caregiver quality of life.”
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Chapter Highlights

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Card 1 — Core Concept
The Curve Expresses the Disease

Neuromuscular scoliosis is the structural consequence of muscle weakness and impaired central/peripheral postural control. Thus, two radiographically identical curves have entirely distinct natural histories, pulmonary risks, and functional needs depending on the underlying diagnosis and GMFCS motor level.

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Card 2 — Clinical Decision
Plan Around the Seated Patient

In non-ambulatory patients, trunk balance and pelvic obliquity must be evaluated in their habitual sitting posture. Reconstructive span and pelvic fixation should be decided based on sitting comfort, iliocostal impingement, skin breakdown risk, and caregiver ease, rather than radiographic numbers alone.

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Card 3 — Pearl / Alert
Optimize Before Correcting

Pulmonary function, nutritional status, cardiac clearance, seizure management, and social support directly dictate perioperative morbidity. In this high-risk population, true surgical planning begins well before skin incision: converting a medically fragile patient into an optimized surgical candidate is an integral part of deformity care.

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How to Cite this Chapter (Vancouver Format)

Official bibliographic indexing and citation guidelines
📖 Pages: 411-422Vancouver Style
Authors (Vancouver):Aguiar CA, Cordeiro LEB

Aguiar CA, Cordeiro LEB. Escoliose neuromuscular. In: Pudles E, Defino H, Risso M, editors. Tratado de Cirurgia da Coluna Vertebral (Treatise of Spine Surgery). 1st ed. Rio de Janeiro: Dilivros Editora; 2026. p. 411-422.

ISBN: 978-85-8053-292-0 • 1.ª Edição • Dilivros Editora
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Episode 06 – Early-Onset Scoliosis
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Episode 06 – Early-Onset Scoliosis

Lungs and the Growing Spine: thoracic development, C-EOS classification, and growth-friendly surgical techniques