【摘要】 目的 探讨先天性巨肢畸形的临床治疗方法与疗效。方法 对8例先天性巨肢畸形病人分别采用病变神经切除及移植术、骨骺阻滞、局部切除减容术、截肢术。结果 手术平均年龄为8岁，术后随访1~5年(平均39个月)，2例术后外形仍显臃肿，功能不理想，1例行截肢术，其余5例术后功能与外形均较满意。结论 应根据患者年龄大小、临床分型、病变程度及患肢功能状况，选择适宜的手术方式。手术方式应个体化、综合性，早期以预防性手术为主，晚期则以矫正畸形为主。

    【关键词】畸形；先天性；巨肢症；显微外科手术；治疗结果

    Clinical therapy andoutcome of congenital macromelia

    ZHANG Xiang-yi，JIANG Chang-qing,WANG Yong-jun,et al.Corresponding Author WAN Sheng-xiang Department of Hand Surgery，Peking University Shen-zhen Hospital，Shenzhen 518036，China

    【Abstract】 Objective To approachclinical therapy and treatment outcome of congenital macromelia.Methods Surgical treatment was performed on 8 cases of macromelia:neurectomy,neurotization,epiphyseal arrest,soft tissue excision,amputation.Results The average age at the time of surgery was 8 years.Over a period of 1-5years following-up(an average of 39 months),the postoperative function and appearance were satisfactory in 5 cases except that of 2 cases had slightly fat forma with poor function.Amputation was used in 1 case.Conclusion The proper surgical operation method was chosen according to the age,clinical classification,abnormal degree and the limb function.The operation method should be individual and comprehensive.The earlier operations are preventive in development of macromelia deformities ......