骨的纤维结构不良的临床特点和处理(附38例分析)

Fibrous dysplasia of bone-Clinical characteristics and management tactics

目的 研究骨的纤维结构不良的临床特征和治疗方法。方法 骨纤维异样增殖症临床不少见 ,多发病例治疗困难 ,此症有其临床特点 :1 发病年龄 ,本组 3 8例中儿童 1 0例 ,青壮年 2 8例 ;2 就诊症状 ,疼痛 2 5例 ,病理骨折 1 2例 ,发生畸形及功能障碍者 1 7例 ;3 好发部位 ,单骨病变 3 3例 ,多骨病变 5例 ,大粗隆部病变 1 4骨 ,骨干全长病变股骨 1 0例 ,胫骨 8例 ,肱骨 3例 ,尺桡骨 4例 ,其他短骨 6例 ;4 病变进展缓慢 ,出现症状后观察 5～ 9年 ,无明显改变。病灶全长截骨 ,清除病灶 ,嵌插接骨 ,少量植骨 ,对多发病例逐个治疗。结果 1 股粗隆部病变 ,髋内翻 ,嵌插接骨矫正内翻 ,植碎骼骨 ,随诊 2～ 7年 ,9骨均愈 ;2 长骨干病变 ,长形带肌蒂或骨膜骨瓣截骨 ,清除病灶 ,嵌插接骨 ,植自体肋骨细条 ,治疗股骨 5例 ,胫骨 4例 ,随诊 2～ 5年均愈合 ;3 多骨病变 ,对有症状者按前述治疗 ,1年 1骨 ,4例近年先胫骨 ,后股骨 ,半年愈合 ,共 1例。结论 骨纤维异样增殖症 ,发展缓慢 ,对多发病例 ,可逐个治疗 ,自体肋骨适于长骨干植骨 ,可达病灶治愈。

Objective To study the clinical features and the treatment tactics of fibrous dysplasia(FD) of bone.Methods Fibrous dysplasia of bone is a chronic disease and not rare to encounter in clinical setting.The FD with multiple bone lesions is difficult to treat.We reviewed 38 cases with FD of bone.The clinical features of cases with FD involved:①Age of onset:Of 38 cases in this series,10 cases were children,28 cases were young adults.②Symptoms:Local pain was found in 25 cases;Pathologic fracture was seen in 12 cases;Developed deformity and impairment of function were noticed in 17 cases.③Location site:FD with single bone lesions were seen in 33 cases and that with multiple bone lesions were found in 5 cases.The FD lesions that located in trochanteric regions were seen in 14 bones,that in whole length of femur in 10 bones,that in tibia in 8 bones,that in humerus in 3 bones,that in ulna and radius in 4 bones,that in other short bones in 6 bones.④The course of FD developed slowly,significant changes were not seen in X ray in many cases that had followed-up for 5-9 years since the onset of symptom.Results Treatment tactics include:resection of the whole length of bone lesion,debridement of focus,osteosynthesis by impaction with a little bone graft.①The lesions in trochanteric region with varus deformity were treated by osteosynthesis by impaction with cancellus bone graft.Nine cases with varus deformities were all corrected in this series.After 2-7 years follow-up,all sites of osteotomy were healed.②The lesions of long bone shaft were treated by long bone osteotomy with muscle or periosteum or bone flap with vascular pedicle after debridement.By using above method with osteosynthesis by impaction and rib long chip bone graft,5 femurs and 4 tibias weretreated and followed up for 2-5 years,all bone were healed in this period.③The multiple bone lesion were treated one by one within one year according to the regimen that we used before,and now all lesions were treated within half a year in sequence of femur and then tibia.By using this method,5 cases with multiple lesions were healed.Conclusion The course of FD develops very slowly.It can be cured by thoroughly debridement,osteosynthesis by impaction with bone graft.For multiple FD lesions,rib autograft was good bone graft for long bone shaft lesion,the bone lesions should be treated one by one.