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Early onset scoliosis in syndromic and neuromuscular disorders: A multidisciplinary approach
Aaron J Wey1, Alexander J Schüpper1, Travis S CreveCoeur1
1Shriners Children's Philadelphia, 3551 N Broad St, Philadelphia, PA, 19140, USA.
Insights
Pediatric scoliosis in children with neuromuscular and syndromic disorders requires specialized, multidisciplinary care. Coordinated treatment improves outcomes and patient satisfaction for these medically fragile children.
Area of Science:
- Pediatric Orthopedics
- Spine Surgery
- Genetics
Background:
- Children with neuromuscular and syndromic disorders face high risks of early-onset scoliosis.
- Scoliosis in these populations is complex, involving systemic issues beyond spinal curvature.
Purpose of the Study:
- To review clinical and surgical challenges in pediatric scoliosis associated with specific disorders.
- To emphasize the critical role of multidisciplinary care in managing these complex cases.
Main Methods:
- Review of clinical and surgical challenges in pediatric scoliosis.
- Emphasis on multidisciplinary care approaches for various conditions.
Main Results:
- Specific challenges identified for cerebral palsy, myelomeningocele, spinal muscular atrophy, neurofibromatosis type 1, Marfan syndrome, and Prader-Willi syndrome.
- Standardized multidisciplinary pathways reduce hospitalizations and complications.
Conclusions:
- Optimal management of neuromuscular and syndromic scoliosis necessitates coordinated, comprehensive, lifelong care.
- Integrated care models enhance patient trust and satisfaction.
Abstract:
Children with neuromuscular and syndromic disorders are among the most medically fragile patients in pediatric spine care. Conditions such as cerebral palsy, myelomeningocele, spinal muscular atrophy, neurofibromatosis type 1, Marfan syndrome, and Prader-Willi syndrome carry a high risk of early-onset scoliosis during critical periods of thoracic and pulmonary development. In these populations, scoliosis is rarely an isolated problem; it reflects the cumulative burden of neurologic impairment, connective tissue fragility, cardiopulmonary compromise, nutritional deficiency, and systemic disease. This review highlights the clinical and surgical challenges unique to each condition while emphasizing the central role of multidisciplinary care. In cerebral palsy, scoliosis risk correlates with motor severity and is compounded by respiratory and nutritional compromise. In myelomeningocele, neurosurgical and urologic coordination is essential given the prevalence of shunt dependence, Chiari malformation, tethered cord, and neurogenic bladder. In spinal muscular atrophy, perioperative pathways center on airway clearance, noninvasive ventilation, and nutritional optimization. Neurofibromatosis type 1 is distinguished by dystrophic scoliosis and dural ectasia, requiring advanced imaging and tailored instrumentation, while families often seek broader clinic support from genetics, oncology, and ophthalmology. In Marfan syndrome, connective tissue fragility complicates fixation, and combined management with cardiology and chest wall specialists is often required. For Prader-Willi syndrome, obesity, endocrine dysfunction, and severe sleep apnea demand careful optimization. Evidence demonstrates that standardized multidisciplinary pathways shorten hospitalizations, reduce pulmonary and infectious complications, and improve consistency of care. Beyond measurable outcomes, families report greater trust and satisfaction when diverse specialties are integrated. Together, these findings reinforce that optimal management of neuromuscular and syndromic scoliosis depends not only on surgical expertise, but also on coordinated, comprehensive, and lifelong care.
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