成骨不全股骨畸形的截骨矫形

Corrective osteotomy for the treatment of femoral deformity in children with osteogenesis imperfecta

目的探讨多段截骨矫形治疗成骨不全儿童股骨畸形的手术特点和临床效果。方法2004年1月至2007年1月，采用多段截骨矫形＋Rush钉内固定，治疗成骨不全股骨畸形10例（共14根股骨）。其中男7例，女3例，年龄5～16岁，平均年龄10．3岁。术后进行临床评价（活动方式、股骨长度和感染）和放射学评价（Rush钉的位置、移位、弯曲、断裂和再骨折）。结果随访3～5年，平均3．5年，所有病例均骨性愈合，愈合时间6～9周，平均7周；首次手术的平均年龄为6．1岁，4例需要二次手术更换Rush钉；首次与第二次手术的时间间隔为1．5～4年，平均2．7年。所有病例未发生骨折、血管神经损伤、感染、骨不连等并发症；股骨畸形和下肢功能获得明显改善（P〈0．01）。其中1例未及时更换Rush钉，其远端自骨干穿出，局部成角畸形，给予截骨矫形、更换Rush钉后治愈。结论术前个性化设计，采取多段截骨矫形治疗成骨不全儿童的股骨畸形，可以加速骨愈合，减少再骨折；最大可能的矫正畸形，改善下肢功能，提高生活质量。

Objective To evaluate the surgical strategies and outcomes of multiple corrective osteotomy for the treatment of femoral deformity in children with osteogenesis imperfecta. Methods Between 2004 and 2007, 14 femora of 1（1 patients with osteogenesis imperfect, who underwent recurrent fractures and/or deformities in the femora at this center, were recruited in this study. They included 7 males and 3 females, aged from 5 to 16 years old （mean age, 10. 3 years old）. The femoral deformities were corrected by multiple corrective osteotomy and Rush pins. After surgery, the clinical conditions of these patients regarding mobility status, growth and limb-length, and infection were followed up. Radiological images were taken to monitor pin placement, migration, bending, breakage of pins, and refracture of femora. Results The average duration of follow-up was 3.5 years （ranged from 3 to 5 years）. Union was achieved in all cases within 7 weeks （ranged from 6 to 9 weeks）. The mean age at primary surgery was 6. 1 years old. The pin revision surgeries were performed on 4 patients. No complication was noted. Postoperative mobility and lower extremity function were improved in all patients （P〈0.01 ）. Conclusions Multiple corrective osteotomy is effective for the treatment of femoral deformity in children with osteogenesis imperfect.