背景:成骨不全症(osteogenesis imperfecta, OI)系骨骼I型胶原数量减少或结构异常,导致骨强度显著降低、反复骨折、畸形愈合的罕见疾病.截骨矫形术有助于改善肢体功能,提高患者生存率.目的:探讨多段截骨治疗成骨不全性下肢多骨重度畸形的围术期管理、手术方法及短中期临床疗效.方法:回顾性分析2015年1月至2017年10月收治的成骨不全合并重度下肢畸形患者7例,其中男5例,女2例,平均年龄14.7岁(9~27岁).下肢长骨中24骨存在畸形,术前股骨及胫骨畸形成角为63°(20°~120°).结果:18骨行多段截骨弹性髓内钉固定.单骨平均截骨3.4处(3~4处).股骨侧单骨手术时间为1.7 h(1~2.5 h),胫骨侧单骨手术时间为1.2 h(1~1.5 h).股骨侧手术单骨出血量223 ml (150~600 ml).平均随访17.7个月(6~40个月).患肢部分负重时间为术后4.3个月(4~6个月).骨愈合时间5.4个月(4~8个月).无骨不愈合、再骨折发生.1例切口脂肪液化,换药后愈合.Barthel指数评分由术前45.7分(24~80分)提高到术后85.7分(78~96分).WeeFIM评分由术前50.8分(36~70分)提高到术后74.0分(60~90分).手术前后的Barthel评分和WeeFIM评分比较,均有显著统计学差异(P＜0.01).结论:多段截骨内固定术可有效纠正下肢力线,最大限度矫正重度肢体畸形,多骨联合一期手术显著减少手术次数、缩短治疗周期,结合康复训练,可有效恢复下肢功能,提高生活质量.%Background: Osteogenesis imperfecta is a rare disease caused by the decrease and structural abnormality of bone type I collagen. It can lead to the decrease of bone strength, repeated fractures and malunion. Osteotomy can improve limbs fuctions and increase the survival rate of the patients. Objective: To explore the perioperative management, surgical methods and short and mid-term clinical outcomes of multi-segmental osteotomy for severe deformity of lower limbs caused by os-teogenesis imperfecta. Methods: Seven patients with severe deformity of lower limbs caused by osteogenesis imperfecta ad-mitted to our hospital between January 2015 and October 2017 were retrospectively analyzed. There were 5 males and 2 fe-males with a mean age of 14.7 years (range: 9-27 years). Deformity was found in 24 long bones. Of them, 18 bones were conducted multi-segmental osteotomy and intramedullary nail fixation. Preoperative angulation of the deformity of the fe-mur and tibia was 63°on average (range: 20°-120°). Results: The osteotomy was performed in 3.4 sites of one bone on aver-age (range: 3-4 sites). The mean operation time of the femur and tibia was 1.7 h (range: 1-2.5 h) and 1.2 h (range: 1-1.5 h), respectively. The amount of bleeding of one femur was 223 ml (range: 150-600 ml). All the patients were followed up for 17.7 months (range: 6-40 months). The patients began to take partial weight training at 4.3 months postoperatively (range: 4-6 months). The mean healing time was 5.4 months (range: 4-8 months). No nonunion or refracture occurred. Fat liquefac-tion was found in one incision which was healed by wound dressing. Barthel score increased from 45.7 (range: 24-80) preop-eratively to 85.7 (range: 78-96) after surgery (P＜0.01). WeeFIM score increased from 50.8 (range: 36-70) preoperatively to 74.0 (range: 60-90) after surgery (P＜0.01). Conclusions: One-stage multi-segmental osteotomy and intramedullary nail fix-ation of the femur and tibia can effectively correct lower limb alignment, maximize the correction of severe lower limb de-formity, reduce operation frequency and shorten treatment period, and also recover the motor function of lower limbs and improve the quality of life in combination with rehabilitation training.
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