2026, Number 3
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Cir Columna 2026; 4 (3)
Early-onset scoliosis secondary to neurofibromatosis type 1: a case report and literature review
Barraza-Silva JA, Villegas-Esquivel EL, Munguía-León LM, Vilchis-Sámano H
Language: Spanish
References: 14
Page: 246-251
PDF size: 1119.08 Kb.
ABSTRACT
Introduction: early-onset scoliosis involves a curvature greater than 10° in the spinal coronal plane occurring before age 10. This condition has various etiologies, including idiopathic, neuromuscular, congenital, and syndromic causes. Neurofibromatosis Type 1 (NF1) is among the most common syndromic causes of scoliosis, with an estimated 10% to 30% of individuals with NF1 developing this condition.
Objective: to present a case of scoliosis secondary to neurofibromatosis and review the literature regarding its clinical management, epidemiology, and current treatment strategies.
Case report: we report the case of a 10-year-old female diagnosed with neurofibromatosis type 1 (NF1) who presented with severe dorsal pain rated 8/10 on a numeric analog scale and a dorsolumbar deformity. A physical examination revealed right-convex thoracic scoliosis with a positive Adams test. Spine radiographs showed a main thoracic scoliosis with a Cobb angle of 28° and a lumbar curvature of 8.7°. A brace was initially prescribed for conservative management; however, after six months, curvature progression to 36° was noted. Growth rods were subsequently placed, achieving a Cobb angle of 7° in the thoracic curvature. The patient underwent two subsequent lengthenings but lost follow-up due to the COVID-19 pandemic. Two years later, follow-up resumed, leading to an instrumented posterior fusion from T1 to L5, resulting in a residual thoracic curvature of 19°.
Conclusions: scoliosis in neurofibromatosis type 1 can progress rapidly. A combined approach of bracing, growth rods, and fusion is crucial for effective management and stabilization of the spine.
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