多段截骨矫形治疗成骨不全性下肢多骨畸形的临床研究  

Clinical study on multi-segmental osteotomy for severe deformity of lower limbs caused by osteogenesis imperfecta

作　　者：高鹏[1] 李梅[2] 范彧[1] 董玉雷[1] 吕彦泽 陆叶 GAO Peng;LI Mei;FAN Yu;DONG Yulei;LV Yanze;LU Ye(Department of Orthopedics;Department of Endocrinology, Peking Union Medical College Hospital, PUMC＆CAMS, Beijing 100730, China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院骨科,北京100730 [2]中国医学科学院北京协和医学院北京协和医院内分泌科,北京100730

出　　处：《中华骨与关节外科杂志》2018年第6期426-431,共6页Chinese Journal of Bone and Joint Surgery

摘　　要：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)。Wee FIM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Wee FIM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 osteogenesis imperfecta. Methods： Seven patients with severe deformity of lower limbs caused by osteogenesis imperfecta admitted to our hospital between January 2015 and October 2017 were retrospectively analyzed. There were 5 males and 2 females 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 femur and tibia was 63° on average（range： 20°-120°）. Results： The osteotomy was performed in 3.4 sites of one bone on average（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 for17.7 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 liquefaction was found in one incision which was healed by wound dressing. Barthel score increased from 45.7（range： 24-80） preoperatively to 85.7（range： 78-96） after surgery（P〈0.01）. Wee FIM score increased from 50.8（range： 36-70） preoperatively to74.0（range： 60-90） after surgery（P〈0.01）. Conclusions： One-stage multi-segmental osteotomy and intramedullary nail fixation of the femur and tibia c

关 键 词：成骨不全 多段截骨 弹性髓内钉 

分 类 号：R687.3[医药卫生—骨科学]