【摘要】 目的探讨尺骨远端遗传性多发性骨软骨瘤病的病变特点和治疗效果。方法回顾性地分析了14例23侧累及尺骨远端的多发性骨软骨瘤病例,有2侧采用非手术治疗,5侧接受了瘤体切除手术,17侧接受了尺骨延长术,5侧接受了桡骨截骨术。比较了手术前后的前臂功能和外观变化。并对桡骨头脱位和尺骨弯曲的相关性进行了统计学分析。结果本组病例术后前臂短缩、弯曲和时关节畸形改善较为明显。部分病例切除部分瘤体后,使尺骨远端的部分骺板开放。在尺骨延长后,部分病例桡骨远端的尺偏角也显著减少。但肘、腕关节主动屈、伸活动度、前臂旋前、旋后活动度、以及握力在术前和术后的测量值差异均无显著性意义。手术也不能使脱位的桡骨头良好复位。在桡骨头脱位的病例中,尺骨弯曲度为24．2°±7．5°,在没有桡骨头脱位的病例中,尺骨的弯曲度为6．5°±5．1°,两者差异有非常显著性意义。结论桡骨头脱位和尺骨弯曲有密切的相关性,手术治疗对外观改善较为明显,但无助于患肢功能改善,应依据病变的具体情况制定个性化的手术方案,如果决定采用手术治疗,早期手术疗效更好。更多还原

【Abstract】 Objective To explore the clinical patterns and the treatments of hereditary multiple exostoses at the distal end of the ulna in children.Methods Twenty-three sides in 14 cases were stud- ied retrospectively for their hereditary multiple exostoses at the distal end of the ulna,Among them,2 sides were treated conservatively,5 sides received exostoses removal,17 sides got ulnar elongation, and the other 5 sides underwent radius osteotomy.All patients were followed up.The functional sta- tus and appearance improvement were evaluated.The relationship between radial head dislocation and the ulnar curvature was also analyzed.Results The forearm and elbow deformities were almost cor- rected by the surgical procedures.In some cases,partial exostosis removal was of help for the re-open- ing of ulnar epiphysis.For young patients,ulnar elongation could reduce the ulnar tilt of distal radius. But operations did not contribute to the improvement of the range of motion for the flexion and exten- sion of both wrists and elbows,as well as for the pronation and supination of the forearms.Neither could operations stably well reduce the dislocated radial head.The ulnar curvatures were 24.2°±7.5°in the cases with radial head dislocation and 6.5°±5.1°in the cases without radial head dislocation. Conclusions Surgical procedures have some benefits for the deformity correction and aesthetic purpo- ses,but of little help for functional improvement of the involved extremities in children with multiple exostoses at the distal end of the ulna.更多还原