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[目的]对多发性骨软骨瘤病进行治疗、分析,总结临床经验,提高对该病的认识。[方法]回顾分析2007年5月~2013年2月收治的12例多发性骨软骨瘤病患者的临床资料。[结果]男7例,女5例;年龄6~42岁,7例有家族史。其中10例全身多发,合并畸形包括2例前臂尺桡关节脱位,1例手指关节畸形,2例下胫腓关节畸形。瘤体大小0.6 cm×1.0 cm×1.2 cm~5cm×7.6 cm×8.5 cm。所有患者行局部肿瘤切除术,手术史:1例6次,2例4次,2例2次,7例1次;每次手术切除部位:2例5个,4例3个,6例2个。1例术后发生右腓总神经损伤,经营养神经保守治疗6个月后神经功能部分恢复,发生足下垂、内翻畸形,行肌腱转位移植后功能明显改善。术后随访时间12~60个月,3例患者术后1年复发再次行局部肿瘤切除术,年龄在6~12岁。1例术后42个月发生恶变,行截肢术。[结论]多发性骨软骨瘤病诊断容易,早期一般无明显临床症状,若影响关节功能及骨骼发育应尽早手术治疗,局部肿瘤切除术效果确切,恶变率小。
[Objective] To treat, analyze and summarize the clinical experience of multiple osteochondromatosis to improve the understanding of the disease. [Methods] The clinical data of 12 patients with multiple osteochondromatosis admitted to our hospital from May 2007 to February 2013 were retrospectively analyzed. [Results] There were 7 males and 5 females with a family history of 6 ~ 42 years and 7 with family history. Of which 10 cases of multiple body multiple, combined deformity, including 2 cases of forearm ulnar dislocation, 1 case of finger joint deformity, 2 cases of tibiofibular joint deformity. Tumor size 0.6 cm × 1.0 cm × 1.2 cm ~ 5 cm × 7.6 cm × 8.5 cm. All patients underwent local tumor resection and operation history: 1 case was 6 times, 2 cases were 4 times, 2 cases were 2 times, 7 cases were 1 time; each operation site was removed: 2 cases 5 cases, 4 cases 3 cases and 6 cases 2 A One case of right common peroneal nerve injury occurred after operation, conservative neurotrophic therapy 6 months after partial recovery of nerve function, the occurrence of foot drooping, varus deformity, line tendon transposition after transplantation significantly improved function. The follow-up time ranged from 12 to 60 months. Three patients relapsed after one year and underwent local tumor resection with a mean age of 6 to 12 years. One case had malignant transformation 42 months after operation and performed amputation. [Conclusion] Multiple osteochondromatosis is easy to diagnose. There are no obvious clinical symptoms in early stage. If the effect on joint function and bone development should be treated as soon as possible, the effect of local tumor resection is definite and the rate of malignant transformation is small.