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目的 探讨Marfan’s综合征合并小儿脊柱畸形的发病机制及治疗原则。方法 30例Marfan’s综合征合并脊柱畸形的患儿在我院得到矫治及随访。 1 3例采用单纯后路矫形融合 ,1 7例采用前路松解加后路矫形。其中 2 0例曾采用支具等保守疗法。通过对手术前后的顶椎偏移 (AVT) ,顶椎旋转度 (AVR) ,Cobb’s角 ,躯干移位程度 (TS)及身高的变化比较 ,分析总结治疗效果并探讨治疗原则。结果 单纯后路矫形术后并发症较多 ,前后路较大范围的融合疗效较佳。结论 Marfan’s综合征合并小儿脊柱畸形应早期治疗 ,前后路融合可获得较好的治疗效果
Objective To investigate the pathogenesis and treatment of Marfan’s syndrome combined with pediatric spinal deformity. Methods Thirty patients with Marfan’s syndrome complicated with spinal deformity were treated and followed up in our hospital. Thirteen cases were treated with pure posterior orthopedic fusion, and 17 cases were treated with anterior approach plus posterior correction. Of these, 20 had conservative treatment such as braces. AVT, AVR, Cobb’s angle, trunk displacement (TS) and height changes before and after surgery were compared to analyze and summarize the treatment effect and explore the treatment principle. Results The posterior simple orthopedic surgery more complications, a wide range of anterior and posterior fusion better. Conclusion Marfan’s syndrome combined with pediatric spinal deformity should be treated early, and the anterior and posterior fusion can achieve better therapeutic effect