四肢长骨大段纤维结构不良的临床特征和治疗

Fibrous dysplasia in large segments of long bones:clinical features and treatment

目的研究四肢长骨大段纤维结构不良(FD)的临床特征和有效治疗方法。方法我院自2001年1月至2008年12月收治33例四肢长骨大段FD患者(病变超过单一长骨总长度2/3),其中男性23例、女性10例,平均年龄42.5岁(8～72岁)。病变位于股骨12例,胫骨5例,腓骨2例,肱骨6例,尺骨5例,桡骨3例,伴发其他部位病变3例,发生病理性骨折18例。平均病程6.4年(6～20年)。手术治疗方法为单纯病灶切除+打压式植骨内固定术(17例)、病灶切除+带血管腓骨重建胫骨术(3例)、病灶清除+带血管腓骨重建肱骨术(3例)、人工关节置换术(5例)和截肢术(5例)。结果 33例患者均未出现感染或皮缘坏死等手术并发症。其中28例获随访,平均随访时间8.4年(23个月～10年)。2例单纯病变经刮除、打压式植骨术后复发,经再次病灶刮除+植骨重建钉内固定后治愈,5例关节置换术患者肢体活动度恢复理想。结论四肢长骨大段FD的病程长,骨质破坏严重,分步骤逐个治疗可获得病灶治愈。

Objective To study the clinical features and treatment tactics of fibrous dysplasia（FD） in large segments of long bones.Methods Summarize 33 cases of FD involving limbs with lesion exceeds 2/3 of single limb,which were treated in our hospital from Jan.2001 to Dec.2008.There were 23 males and 10 females.The average age was 42.5 years（8 to 72 years）. Twelve cases of the FD lesions were located in femur,5 in tibia.2 in fibula.6 in humerus.5 in ulna and 3 in radius.There were 3 cases for multiple FD lesions.18 cases for pathological fracture.The course of FD lesions was 6 to 20 years,average of 6.4 years.Surgical treatment included;focus resection,bone grafting and internal fixation（17 cases）,focus resection,tibia reconstruction with vascularized fibula（3 cases）,focus resection,humerus reconstruction with vascularized fibula（3 cases）. arthroplasty（5 cases） and amputation（5 cases）.Results No complications such as infection,skin edge necrosis were observed after surgery in all patients.Twenty-eight cases were followed up with average of 8.4 years（23 months to 10 years）.Two recurred cases of bone grafting were eventually healed with reoperation.Five cases of arthroplasty got satisfactory function. Conclusions The course of FD develops slowly and causes severe bone destruction.It can be cured by thoroughly debridement, internal fixation,and should be treated step by step.