儿童Ⅰ型神经纤维瘤病脊柱侧弯的诊治要点OA北大核心
Clinical diagnostics and interventions of children with neurofibromatosis type 1 associated scoliosis
Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1)的临床表现复杂多样,患者可以出现牛奶咖啡斑、丛状神经纤维瘤、骨骼系统畸形等,其中脊柱侧弯是最常见的骨骼合并畸形.儿童NF1脊柱侧弯常伴随脊柱骨骼营养不良性改变,此类脊柱侧弯发病早、进展快,在造成患者外观畸形的同时,还影响脊柱的正常生长发育,应早期进行干预.可根据脊柱侧弯的部位、类型、程度,结合患者年龄和生长潜力,选择合适的治疗方式.在治疗脊柱侧弯的同时,需要注意合并畸形的处理及预防并发症.NF1脊柱侧弯在整体管理过程中,需要加强多学科联合,力争给患者提供规范化、个体化、精细化的治疗.
Children of type 1 neurofibromatosis may present with a wide range of symptoms,including café au lait spots,plexiform neurofibromas and skeletal system deformities.Among them,scoliosis is the most common skeletal deformity.Children with NF1 scoliosis often have nutritional deficiency-type changes in spinal bones,leading to early onset and rapid progression of scoliosis,causing deformity of appearance and affecting normal growth and development of spine.Thus early intervention is necessary.Based on the location,type,de-gree of scoliosis,and patient age and growth potential,appropriate treatments may be selected.In addition to treating scoliosis,attention should also be paid to managing concomitant deformities and preventing complica-tions.For overall management of NF1,multidisciplinary treatment is needed to provide standardized,individual-ized and meticulous treatment.
曹隽;李海艟
国家儿童医学中心 首都医科大学附属北京儿童医院骨科,北京 100045国家儿童医学中心 首都医科大学附属北京儿童医院骨科,北京 100045
Ⅰ型神经纤维瘤病脊柱侧弯诊断外科手术保守治疗药物疗法多学科诊治儿童
Neurofibromatosis Type 1ScoliosisDiagnosisSurgical Procedures,OperativeConser-vative TreatmentDrug TherapyMulti-disciplinary TeamChild
《临床小儿外科杂志》 2025 (4)
301-306,6
北京市自然科学基金-海淀原始创新联合基金资助项目(L222095)国家重点研发计划资助(2023YFC2507701) Beijing Natural Science Foundation(L222095)National Key R&D Program of China(2023YFC2507701)
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