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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。
Objective To investigate the clinical treatment and efficacy of congenital giant limb deformity. Methods Eight cases of congenital giant limb deformity were treated by pathological nerve resection and transplantation, epiphyseal block, partial resection and volume reduction and amputation. Results The average age of the operation was 8 years. The postoperative follow-up ranged from 1 to 5 years (average 39 months). The shape of the two patients was still bloated after operation. The function was unsatisfactory. One patient underwent amputation. The other five patients had postoperative function and appearance More satisfied. Conclusion According to the patient’s age, clinical type, degree of lesion and functional status of limbs, the appropriate surgical method should be selected. The mode of operation should be individualized and comprehensive, mainly in the early stage of preventive surgery, while in the later stage, the correction of deformity is the mainstay.