髂骨植骨尺骨延长治疗多发性骨软骨瘤前臂畸形的疗效评价

目的：探讨髂骨植骨尺骨延长治疗遗传性多发性骨软骨瘤所致前臂畸形的疗效。方法：保留尺骨远端骨骺部分，使尺骨一次性延长1．5～4cm，根据临床分型辅加环状韧带重建术和桡骨截骨术以矫正前臂弯曲畸形。结果：除1臂外，全部随访病例前臂畸形均获明显改善，肘腕关节活动与正常侧比较无明显差异。X线片示尺骨发育与桡骨近于匹配。结论：保留尺骨远端骨骺，行尺骨远端骨软骨瘤切除，同时髂骨植骨，一次性延长尺骨治疗遗传性多发性骨软骨瘤所致的前臂弯曲短缩畸形、下尺桡关节脱位及尺偏手效果良好，可使患儿获得近于正常的发育，提高生活质量。

The effect of ilium grafting and ulna lengthening on the treatment of forearm deformity of heredity multiple osteochondroma

Objective:To discuss the effect of ilium grafting and ulna lengthening on the treatment of forearm deformity of heredity multiple osteochondroma.Method:Fifteen patients(including 12 male and 3 female) were treated,and there were totally 20 arms including 11 arms in left and 9 arms in right.The mean age of patients was 11.1 years.In the operation,the distal epiphyses of ulnas were preserved and the ulnas were lengthened from 1.5 to 4 centimeters.At the same time,the reconstruction of the annular ligaments or radius osteotomies were performed according to the clinical classification.All patients were followed up for an averaged period of 2 years and 2 months.Result:The deformities and the wrist and elbow joints motion range improved significantly in all cases except 1 arm.The ulnar development well matched the radii from the result of X-ray checking.Conclusion:The resection of distal ulnar osteochondrona,the preserving of the epiphyses,and the ilium grafting for the ulnar lengthening can correct the bowing and shortening deformities of forearm.And the dislocation of distal ulnoradial joints and ulnar clubhand can be prevented as well.The normal development of affected part and a good quality of life can be obtained.