骨纤维发育不良致股骨近端畸形的手术治疗进展  

The surgical treatment progress of proximal femoral deformity caused by fibrous dysplasia of bone

作　　者：蔡成阔 张净宇 邓书贞[1] 刘艳成[1] 胡永成[1] Cai Chengkuo;Zhang Jingyu;Deng Shuzhen;Liu Yancheng;Hu Yongcheng(Department of Bone and Soft Tissue Oncology,Tianjin Hospital,Tianjin 300211,China)

机构地区：[1]天津市天津医院骨与软组织肿瘤科,天津300211

出　　处：《中华骨科杂志》2024年第15期1040-1047,共8页Chinese Journal of Orthopaedics

摘　　要：骨纤维发育不良(fibrous dysplasia of bone,FD)是一种以髓内纤维性病变为特征的类肿瘤样疾病,病变区域骨的发育停滞在未成熟的编织骨阶段,无法形成正常的骨小梁,导致骨的结构改变和力学强度降低;在负重时常出现反复的病理性骨折,进而继发受累骨的弯曲畸形、肢体短缩及步态异常。股骨近端是FD肢体畸形常累及的部位,畸形类型和程度复杂多样,多表现为逐渐加重的髋关节内翻及骨干弯曲。矫正畸形的目的是恢复股骨正常的机械轴线和长度,从而恢复肢体的功能,避免畸形进展以及缓解因反复的病理性微骨折而导致的疼痛症状,这较病灶本身的治疗更加重要。术前应根据病变的位置、范围以及病变类型个体化制定每一例患者的治疗方案,术后需对患者长期随访,进行矫形方案的调整。是否应进行病灶刮除植骨以及使用植骨材料的种类仍存在争议,应基于矫形原则对FD股骨进行畸形分析,确定畸形类型及畸形顶点位置,设计截骨方案,并进行术前模拟。虽然截骨后髓内或髓外固定方式均可提供足够的生物学稳定,但应根据术中具体情况决定使用何种固定装置。FD患者骨的愈合以及再生均无明显异常,但是生成的骨痂中包括发育不良的骨组织。FD患者在肢体畸形治疗后容易出现畸形复发,需长期密切随访以便进行矫形方案的调整。Fibrous dysplasia of bone(FD)is a tumorlike disease characterized by intramedullary fibrosis,in which the development of the bone in the lesion area stops at the stage of immature braided bone,with the inability to form a normal bone trabecula,resulting in structural changes and reduced mechanical strength of the bone.Repeated pathological fractures often occur with weight bearing,followed by curvature of the affected bone,limb shortening,and abnormal gait.The proximal femur is often involved in FD limb malformations,with complex types and degrees,most of which are manifested as gradually aggravating hip varus and diaphysial curvature.The proximal femur is a common site of limb deformity caused by FD,the types and severity of malformations are complex and varied,which is usually manifested as gradually aggravated varus hip joint and diaphysis bending deformity.The purpose of deformity correction is to restore the normal mechanical axis and length of the femur,thereby restoring the function of the limb,avoiding the progression of deformity and relieving the pain symptoms caused by repeated pathological microfractures,which is more important than the treatment of the lesion itself.The preoperative treatment plan should be made individually for each patient according to the location and extent of the lesion and the type of the lesion.The patients need to be followed up for a long time to adjust the correction plan.Whether the lesion should be curette and bone graft and the type of bone graft material used are still controversial.The femoral deformity of FD should be analyzed based on the principles of deformity correction,the type of deformity and the location of the apex of the deformity should be determined,the osteotomy plan should be designed,and the preoperative simulation should be performed.Both intramedullary and extramedullary fixation after osteotomy can provide sufficient biological stability.The choice of fixation device should be determined according to the specific situation during the operation.There

关 键 词：骨纤维发育不良 肌肉骨骼畸形 截骨术 

分 类 号：R687.3[医药卫生—骨科学]