Curve evolution during bracing in children with scoliosis secondary to early-onset neurofibromatosis type 1:indicators of rapid curve progression

Background:Scoliosis secondary to neurofibromatosis type 1(NF1)in children aged<10 years is an important etiology of earlyonset scoliosis(EOS).This study was performed to investigate the curve evolution of patients with EOS secondary to NF1 undergoing bracing treatment and to analyze high-risk indicators of rapid curve progression.Methods:Children with EOS due to NF1 who underwent bracing treatment from 2010 to 2017 were retrospectively reviewed.The angle velocity(AV)at each visit was calculated,and patients with rapid curve progression(AV of>10°/year)were identified.The age at modulation and the AV before and after modulation were obtained.Patients with(n=18)and without rapid curve progression(n=10)were statistically compared.Results:Twenty-eight patients with a mean age of 6.5±1.9 years at the initial visit were reviewed.The mean Cobb angle of the main curve was 41.7°±2.4°at the initial visit and increased to 67.1°±8.6°during a mean follow-up of 44.1±8.5 months.The overall AV was 6.6°±2.4°/year for all patients.At the last follow-up,all patients presented curve progression of>5°,and 20(71%)patients had progressed by>20°.Rapid curve progression was observed in 18(64%)patients and was associated with younger age at the initial visit and a higher incidence of modulation change during follow-up(t=2.868,P=0.008 and<0.001,respectively).The mean AV was 4.4°±1.2°/year before modulation and 11.8°±2.7°/year after modulation(t=11.477,P<0.010).Conclusions:Curve progression of>10°/year is associated with younger age at the initial visit,and modulation change indicated the occurrence of the rapid curve progression phase.