尺骨延长联合瘤体切除治疗儿童遗传性多发性骨软骨瘤尺骨短缩畸形近期效果观察

Short-term Clinical Observation of Gradual Ulnar Lengthening Combined with Excision of Exostoses to in Treatment of Pediatric Ulnar Shortening Deformity

目的探讨尺骨延长联合瘤体切除治疗儿童遗传性多发性骨软骨瘤（hereditary multiple exostoses,HME）尺骨短缩畸形的效果及安全性。方法 2011年6月—2014年8月我院收治7例HME尺骨短缩畸形,尺骨短缩平均2.6 cm,根据前臂HME临床分型标准,4例为Ⅱa型,3例为Ⅰ1b型。均采用Orthofix外固定器尺骨延长联合瘤体切除治疗,术后患肢按计划进行延长及适当功能锻炼。结果本组平均随访22个月,尺骨延长2.5～4.0 cm,平均3.2 cm,均达骨性愈合。腕关节尺偏畸形得到纠正,前臂旋转功能明显恢复,腕关节及肘关节活动功能明显改善。7例均未出现围术期及远期并发症。结论应用Orthofix外固定器尺骨延长联合瘤体切除治疗HME尺骨短缩畸形,临床近期疗效显著,具有创伤小、并发症少、操作简便灵活等优点。

Objective To explore the effect of gradual ulnar lengthening combined with excision of exostoses to treat ulnar shortening deformity in children with hereditary multiple exostoses （HME）. Methods 7 children with multiple exosto- ses shortening of the ulnar deformity, admitted to our hospital during June 2011 and August 2014 underwent gradual ulnar lengthening combined with excision of exostoses. The average of ulnar shortening was 2.6 cm. According to the type of stand- ard clinical HME of the forearm, 4 cases were type I1 A and 3 cases were type Ilb. The technology of Orthofix external fixa- tion combined with excision of exostoses was used. According to the plan, the postoperative limb was extended with appropri- ate functional exercises. Results The mean follow-up time was 22 months. 7 cases in middle piece of ulnar extended 2.5 ~ 4.0 cm （average 3.2 cm） , and all the patients were healed. Wrist ulnar deviation was corrected, forearm rotation function was restored, wrist and elbow joint function was improved. All the patients had no peri operational period and long-term com- plications. Conclusion Short-term clinical effect of the application of Orthofix external fixation combined with excision of ex- ostoses in the treatment of ulnar shortening deformity in children with HME is obvious with smaller invasion and fewer compli-cations.