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Surgical treatment of early onset scoliosis in neurofibromatosis

机译：神经纤维瘤病早期发病患者的外科治疗

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Case series report of twenty-three patients, aged between 4 and 11 years, were surgically treated at the Authors' Spine Surgery Division in the past 15 years. Mean follow-up is 5 years (range, 18 months to 15 years). Mean age at the time of surgical procedure was 9.1 years (range, 4 years to 11 years). Average scoliosis was 48deg (range, 38deg to 82deg) and skeletal maturity according to Risser sign was 0 in all of the patients. Patients were divided into 2 Groups according to the surgical procedure adopted. Posterior only instrumentation was performed in 16 patients that presented with a thoracic kyphosis lower than 50deg (Group A), in the remaining 7 patients showing thoracic kyphosis exceeding 50deg, combined anterior and posterior instrumented arthrodesis was performed (Group B). One patient, belonging to Group A, was instrumented with growing rod without fusion. Average correction of scoliosis was 60%, overall complication rate 24% and major 7%. Crankshaft phenomenon was observed in 21% (Group A): in these cases, anterior arthrodesis was performed after a mean 15-month from first surgical procedure. Fusion failure was observed in 1 (Group B) patient who underwent revision of posterior instrumentation. Clinical and radiographic evaluation at F-up showed good outcome in terms of deformity progression and quality of life. Early and aggressive surgery is the most effective management for dystrophic curves in neurofibromatosis has been proven to be. Our experience confirms the need for spinal stabilization even in pediatric age in rapidly progressive spinal deformities. .
机译：案例系列报告23名患者，年龄在4至11岁之间，在过去的15年里，在作者的脊柱外科部门进行了外科治疗。平均随访时间为5年（范围，18个月至15年）。手术手术时的平均年龄为9.1岁（范围，4年至11年）。平均脊柱侧凸为48deg（范围，38deg至82deg）和根据味道符号的骨骼成熟度为所有患者。根据采用的外科手术，患者分为2组。仅在16名患者中进行后续仪器，其呈现胸腔脊柱脊髓的患者，剩余的7例显示出现超过50分钟的胸腔脊柱脊柱症的患者，进行了组合的前型和后验证关节术（B组）。一个患者，属于A组，用不含融合的生长棒仪表。脊柱侧凸的平均矫正60％，整体并发症率为24％，主要7％。在21％（A组）中观察到曲轴现象：在这些情况下，在从第一次手术手术开始15个月后进行前部关节瘤。在接受后续仪器修订的1（B组）患者中观察到融合失败。在F-UP的临床和放射线摄影评估在畸形进展和生活质量方面表现出良好的结果。早期和侵略性手术是神经纤维瘤病中最有效的营养不良曲线的管理已被证明是。我们的经验证实，即使在迅速逐步的脊柱畸形中的儿科时代，即使在儿科时代也确认了脊柱稳定的需求。 。

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