Low-grade central osteosarcoma mimicking fibrous dysplasia: a report of two cases

Low-grade central osteosarcoma is an unusual variant of conventional osteosarcoma. We present here two rare cases of low-grade central osteosarcoma resembling fibrous dysplasia. A 24-year-old woman diagnosed as fibrous dysplasia was treated with intra-lesional excision and curettage of the tumor but tumor recurred at 4 months after surgery. Distal femoral en-bloc resection was performed followed by arthroplasty with mega-prosthesis of the knee. A 57-year-old man diagnosed as central osteosarcoma was treated with wide excision of the tumor, followed by reconstruction with the vascularized fibula graft combined with an autogenous irradiated bone graft. Because of the difficulty in distinguishing low-grade central osteosarcoma from a benign lesion, open biopsy is needed to obtain a large tumor sample. Careful clinical and pathological evaluation is required to obtain a definite diagnosis. The treatment of low-grade central osteosarcoma is en-block resection with wide surgical margins.     

股骨近端纤维结构不良的手术治疗

目的探讨股骨近端纤维结构不良的手术治疗方式。方法对19例股骨近端纤维结构不良的不同手术治疗方式及术后疗效进行回顾性分析。结果1例术后3d引流管口渗出血清样物质，加强抗感染、营养支持及换药处理后愈合。19例均获随访，时间13—58个月。复查X线片见缺损修复区内有新骨生成改变，骨折处骨愈合；1例术后20个月因外伤致股骨转子下内固定物旁骨折再次手术发现肿瘤复发，行再次刮除植骨内固定术后14个月愈合；除1例未行内固定的病例外，余患者术后患肢功能均得到良好恢复，8—12个月可弃拐行走。结论股骨近端纤维结构不良应积极手术治疗，在彻底刮除病变和充分植骨的基础上，强调内固定的应用。     

股骨近段纤维结构不良内翻畸形的手术疗效

目的 评估和分析股骨近段纤维结构不良内翻畸形手术治疗的临床疗效.方法 2000年8月至2009年5月,采用截骨矫形、病灶刮除、打压植骨、股骨重建钉内固定治疗21例(24个)股骨纤维结构不良伴重度内翻畸形患者,男12例,女9例;年龄14～39岁,平均22.7岁.其中15例为单纯髋内翻畸形,6例同时伴髋内翻及股骨内翻畸形.颈干角55°～105°,平均75°;股骨内翻角18°～45°,平均30°;患侧股骨长度较对侧短缩2.0～4.5 cm,平均3.4cm.其中13例患者因发生病理性骨折而行手术治疗.结果 21例患者均获得随访,随访时间21个月至7年,平均3年4个月.24个股骨共30处截骨面中,28处于术后3～6个月骨性愈合,2例双段远端截骨面各有1处在术后12个月时仍不愈合,经再次植骨3个月后骨性愈合.21例内翻畸形股骨力线均完全矫正,术后颈干角矫正为95°～135°,平均118°;股骨内翻角完全纠正.股骨长度较术前延长1.8～3.6 cm,平均2.8cm.术后无一例患者发生感染、畸形进展及再骨折.17例患者疼痛视觉模拟评分(visual analogue scales,VAS)由术前7～10分降至术后0分,4例VAS评分由术前8～10分降至术后3～4分.Harris髋关节功能评分优12例,良6例,可3例.结论 外翻截骨可有效矫正内翻畸形,股骨重建钉可稳定股骨,大量打压式植骨可有效恢复骨量.

Abstract：

Objective To evaluate and analyse the effectiveness of surgical treatment for fibrous dysplasia in proximal femur with severe varus deformity.Methods A retrospective study was performed in 21 patients (24 femora)of fibrous dysplasia who were treated in our hospital between August 2000 and May 2009.All patients had severe femoral varus deformity.The four-step procedures were performed orderlv as valgus osteotomy,lesion curettage,impacting of massive bone allograft,and fixation by femoral intramedullary nail.There were 6 patients with monostotic disease,15 with polyostotic diseases,including 12 males and 9 females with a mean age of 22.7 years(range,14-39 years).The average neck-shaft angle and femoral varus angle was 75°(range 55°-105°)and 30°(range,18°-45°),respectively.The average length of thigh shortened 3.4 cm(range,2.0-4.5 cm)compared with the contralateral thigh.Results All of the 21 patients were followed up from 21 months to 7 years with an average period of 3 years and 4 months.There were 30 osteotomy sites in 24 femurs,28 osteotomy sites showed bone union after 3-6 months from surgery.Two distal location of the double-level osteotomy showed nonunion,which received bone grafting again and got bone union after 3 months finally.The femoral mechanical alignments of the 21 patients had been recriticd.The average neck-shaft angle was 118°(range,95°-135°)postoperatively,the femoral varus angle disappeared.The average extremity lengthening was 2.8 cm(range,1.8-3.6 cm)postoperatively.There were no infection,recurrent fracture and progression of deformity.The visual analogue scales(VAS)score of 17 patients decreased to zero postoperatively from 7-10 preoperatively,and that of the other 4 patients decreased to 3-4 postoperatively from 8-10 preoperatively.The result of Harris hip functional score was excellent in 12 cases,good in 6,and fair in 3.Conclusion The valgus osteotomy can rectify varus deformity effectively.The reconstract nail of the fumer can support the stability of femur.Impacting of massive bone allograft can improve the capacity of the femur.     

Changes in bone mineral density of the distal femur after revision total knee arthroplasty with metaphyseal press-fit stem

Background  

The effect of postoperative adaptive bone remodeling following a stemmed femoral implant in revision total knee arthroplasty (rTKA) is unknown. The aim of this study was to evaluate bone mineral density (BMD) changes of the distal femur following cemented rTKA with a 100-mm press-fit stem.     

截骨矫形钢板固定治疗股骨近段纤维结构不良伴髋内翻畸形

目的 探讨股骨转子下截骨矫形、动力髋或髁螺钉系统固定治疗股骨近段纤维结构不良(fibrous dysplasia,FD)伴髋内翻畸形的临床疗效.方法 回顾性分析2001年4月至2010年5月收治的26例股骨FD伴髋内翻畸形患者的临床资料,男9例,女17例;年龄10～53岁,中位年龄19岁.单骨型14例,多骨型12例.病灶长度为多骨型9～36 cm(平均19 cm),单骨型7～15 cm(平均9 cm);17例合并病理性骨折;术前颈干角65°～110°(平均92°),患肢短缩1.5～4.5cm(平均2.8 cm).21例病灶累及股骨颈者采用髋螺钉固定,5例未累及股骨颈者采用髁螺钉固定.结果 手术时间80～170 min(平均120min),出血量280～1650ml(平均960ml).术后颈干角为119°～140°(平均127°);患肢平均延长2.3 cm(1.5～3.6 cm).随访9～118个月(平均39个月),截骨面均愈合.除1例颈干角从术后126°减小到术后56个月的115°,其余患者无髋内翻畸形复发,无内固定断裂或松动.1例股骨颈变短、髋螺钉沿滑槽滑动,但螺钉未切割股骨头;1例术后7年外伤后钢板远端处股骨干骨折.根据Guille的功能评价标准,24例满意、2例不满意.结论 转子下截骨矫形、动力髋或髁螺钉系统内固定能有效地纠正股骨上段FD伴髋内翻畸形,改善患肢功能.

Abstract：

Objective To investigate the clinical effect of subtrochanteric osteotomy plus dynamic hip/condyle screw (DHS/DCS)fixation to treat the fibrous dysplasia of the proximal femur with coxa vara.Methods Twenty-six clinical cases of femoral fibrous dysplasia with coxa vara were retrospectively analysed from April 2001 to May 2010.There were 9 males and 17 females,with a median age of 19(10 to 53).Forteen patients presented with monostotic disease,and 12 with polyostotic disease.The length of the lesion for polyostotic disease was from 9 cm to 36 cm,while for monostotic disease was from 7 cm to 15 cm.Seventeen cases were merged with pathologic fracture.The neck shaft angles were from 65 to 110 degree preoperation.The shortage of limbs was from 1.5 cm to 4.5 cm.Twenty-one patients involved in femoral neck were fixed with DHS and the other 5 cases with DCS.Results The duration of operation was from 80 to 170 min.The amout of bleeding was from 280 to 1650 ml with the average of 960 ml.The average postoperative neck shaft angles were 127 degree(119 to 140).The shortened limbs were extended 2.3 cm for average (1.5 to 3.6 cm).The follow-up time was from 9 to 118 months with the average of 39 months.All patients with osteotomy were healed.The neck shaft angle of 1 case decreased from post-operative 126°to 115°56months post-operatively,no coxa adducta recurrented and all internal fixations were in position.Hip screw backed out through the barrel in one case with the shortage of femoral neck.One case had femoral fracture after an injury.According to Guille function standard,24 cases were regarded as satisfied and 2 cases as unsatisfied.Conclusion Subtroehanteric osteotomy plus DHS/DCS fixation can effectively correct the fibrous dysplasia of the proximal femur with coxa vara,and significantly improve the function.     

Secondary osteosarcoma arising in fibrous dysplasia,case report

Malignant transformation of fibrous dysplasia is very rare. The frequency is increased in polyostotic forms, in McCune–Albright and Mazabraud’s syndromes and previously irradiated cases. Pain, which is rapidly becoming worse over a short period unrelated to trauma is the most alarming symptom. Early radiological features of sarcomatous transformation are moth-eaten or cystic areas of osteolysis, cortical destruction and gradual formation of a soft tissue mass. The prognosis is unfavorable as most of the cases are in an advanced stage in the time of diagnosis. We present an unusual case of unsuspected secondary osteosarcoma arising in a previously unirradiated, monostotic fibrous dysplasia. A 46-year-old woman was admitted with hip pain, which worsened after a minor trauma occurred 1 year ago. Plain graphies of left femur showed a well-delineated lesion with endosteal scalloping and areas having a ground-glass appearance. The MRI revealed minimal contrast enhancement but no heterogenous signal intensity, cortical destruction, periost reaction or accompanying soft tissue component was noted. The lesion was initially curetted. But being diagnosed as osteosarcoma histologically, classical osteosarcoma protocol pre and postoperative chemotherapy was applied. Resected femur showed areas of fibrous dysplasia admixed with osteosarcoma having fibroblastic, chondroblastic and osteoblastic areas that were focally invading the soft tissue. Tumor viability was estimated as 95%. The clinical course worsened rapidly after the operation. She did not respond to postoperative chemotherapy and lost with pulmonary metastases less than a years’ time after the operation. The case is presented to increase awareness on the possibility of malignant transformation in an otherwise unsuspected fibrous dysplasia.     

Nephronophthisis-like nephritis associated with fibrous dysplasia of bone

Nephronophthisis is a chronic tubulointerstitial nephritis with autosomal recessive inheritance whose evolution to end-stage renal disease is insidious but constant. Fibrous dysplasia of bone is characterized by focal replacement of normal bone and marrow with abnormal bone and fibrous tissue. We report on a young boy initially diagnosed with fibrous dysplasia of bone, who underwent renal investigation because of treatment with pamidronate. He presented with mild proteinuria (albuminuria/creatininuria 19 mg/mmol) and decreased glomerular filtration rate (GFR) (79 ml/min per 1.73 m(2) body surface area) leading to kidney biopsy, which showed nephronophthisis-like lesions, but neither NPHP1 gene deletion nor UMOD (uromodulin) mutation were identified. No association between fibrous dysplasia of bone and nephronophthisis has yet been described. Nephronophthisis-like nephritis associated with fibrous dysplasia of bone might represent a possible new syndrome in the nephronophthisis and medullary cystic kidney disease complex. However, a fortuitous association between these two conditions is also possible.     

The longitudinal split technique for narrow canal tibia in a case of distal femur replacement for osteosarcoma

IntroductionThere are currently no available prostheses that can be used for extremely small femurs or tibias of adult patients in countries where allograft is unavailable.Case reportWe report the case of a 17-year-old girl requiring special limb salvage technique for distal femur reconstruction following resection of malignant tumor. This technique was needed because of a very narrow canal of the tibia. We split bilateral tibial cortices longitudinally with osteotomies to enlarge tibial canal enough to insert conventional endoprosthesis. After insertion of the implant, split tibia reduced with titanium cables and bands, and the split clefts were filled with cancellous bone. Fifteen months after the operation, proper fixation was achieved with bony fusion of bilateral split clefts and ambulation without assistance was achieved.DiscussionAny proper surgical procedure for our patient were reported previously. We developed a conventional non-expandable endoprosthesis for distal femur replacement using the double longitudinal split technique as a solution to this difficult problem.ConclusionOur longitudinal split technique should be applicable to adult patients with skeletal immaturity requiring resection of juxta-articular malignant tumors and in other situations complicated by the presence of a narrow canal.     

Cutting error of the distal femur in total knee arthroplasty by use of a navigation system

The aim of this study was to evaluate the alignment of the distal femoral cutting surface using a navigation system to determine the accuracy of bone cutting. We evaluated 20 knees in 20 patients. After cutting the distal femur, the cutting surface was validated using the navigated cutting block adapter, and the angular difference between the cutting surface and that preoperatively planned in the sagittal and coronal planes was recorded. The average error of all knees was 1.6° ± 2.2° in extension, and 14 (70%) of 20 knees were cut in an extended position. Our tendency is to cut the distal femur in an extended position with the first femoral cut in the sagittal plane.     

股骨纤维结构不良伴重度内翻畸形的外科治疗

[目的]探索一种治疗股骨纤维结构不良伴重度髋或及股内翻畸形的有效外科方法。[方法]自2000年8月~2005年7月共收治13例14个股骨纤维结构不良伴重度髋或及股内翻畸形患者,均采用股骨单处或双处外翻截骨矫形、病灶刮除、打压植骨、重建钉内固定治疗。[结果]所有病人随访8~41个月(平均21个月)。X线显示,14个股内翻畸形股骨力线几乎完全矫正;髋内翻畸形的颈干角由术前平均75°(55°~100°)矫正为术后120°(95°~130°);股骨相对长度由术前平均短缩3.4 cm(2.0~4.5 cm),矫正后延长2.8 cm(1.8~3.6 cm);14个股骨共19处截骨面均达骨性愈合。所有患者植骨区于术后3个月均有不同程度的吸收,术后10~14个月最明显。术前4例患者扶双拐行走,2例扶单杖行走,5例病理性骨折不能行走,2例不扶杖跛行;术后11例患者不扶拐行走,2例扶单杖行走。11例患者疼痛消失,2例疼痛明显减轻。所有患者无感染、畸形进展及再骨折。[结论](1)该治疗方法能够有效的矫正股骨畸形,恢复股骨生物力学力线,改善患肢功能;(2)能有效的去除病变,防止复发;(3)股骨髓腔内大量打压式植骨是促进骨愈合及防止病理性骨折的关键。     

Is bone density in the distal femur affected by use of cement and by femoral component design in total knee arthroplasty?

The objective of this study was to evaluate changes in bone density in the distal femur 2 years after total knee arthroplasty with four different implant designs using cemented or cementless femoral components. Bone density was measured retrospectively from radiographs of 114 knees, using a densitometer. A decrease in bone density of up to 57% was identified in the distal femora with a cemented femoral component 2 years after surgery, compared with a decrease of up to 28% with a cementless, porous-coated component of the same design. The differences between the four implant designs in the changes in bone density in the anterior distal femur were significant at 2 years (P < 0.001). A possible cause of this result may be a difference in load transfer due to the different methods of fixation. The surgeon should expect decreased bone density in the distal femur at the time of revision surgery, especially with a cemented femoral component. Received for publication on June 5, 1998; accepted on Dec. 11, 1998     

确定股骨远端旋转力线在膝关节置换中的意义

目的应用MRI测量探讨股骨远端各旋转轴线的关系,为人工全膝关节置换术(TKA)股骨远端旋转力线提供参考。方法选取苏浙地区健康汉族成人106例,膝关节共197个。对所选膝关节行MRI平扫,在MRI横断面图像上进行定位参照轴线,包括外科经股骨上髁轴线(STEA)、临床经股骨上髁轴线(CTEA)、前后轴线(APL)及股骨后髁轴线(PCL)。对轴线参数股骨后髁角(PCA)、髁扭转角(CTA)及前后轴线的垂线与后髁轴线的夹角(PAPA)进行测量。比较各角度不同性别、不同侧别均数的差异是否具有统计学意义。结果PCA平均为3.56°±0.26°,CTA平均为6.35°±0.72°,PAPA平均为4.08°±0.22°。不同性别、侧别的PCA均数差异无统计学意义(P0.05),不同性别、侧别的CTA、PAPA均数差异有统计学意义(P0.05)。结论应用MRI测量确定股骨远端轴线参数是一种可靠的方法,不同性别、侧别PCA相对恒定,STEA可作为TKA中股骨远端假体旋转参照力线。     

股骨远端前后轴线的影像学研究及其临床意义

目的探讨股骨远端前后轴线在全膝关节置换术(TKA)中的临床应用价值。方法对86例106个无疾病的膝关节应用CT对股骨远端垂直于膝关节的机械轴进行薄层扫描,将获取的横断面图像输入电脑,用AutoCAD软件标识并测量、记录前后轴线与外科髁上轴的外侧夹角(ATA),前后轴线与后髁轴线的外侧夹角(APA),前后轴线的垂线与后髁轴线的夹角(A-PA),外科髁上轴与后髁轴线的夹角(PCA)。按性别和侧别分组,分析两组间各角度、比较ATA、APA与常数90°间以及A-PA与PCA、常数3°间有无统计学差异。结果 ATA为89.79°±1.22°,男性89.89°±1.10°,女性89.70°±1.34°,左侧89.63°±1.11°,右侧89.96°±1.32°。APA为84.84°±1.83°,男性84.96°±1.66°,女性84.72°±1.99°,左侧84.50°±2.03°,右侧85.19°±1.54°。A-PA为5.16°±1.83°,男性5.04°±1.66°,女性5.28°±1.99°,左侧5.50°±2.03°,右侧4.81°±1.54°。PCA为4.80°±1.23°,男性4.94°±1.24°,女性4.67°±1.21°,左侧4.87°±1.24°,右侧4.73°±1.22°。4角度性别间及侧别间比较差异均无统计学意义(P0.05)。常数90°与ATA比较差异无统计学意义(P0.05),与APA比较差异有统计学意义(P0.05);A-PA与PCA比较差异无统计学意义(P0.05),但二者与常数3°比较差异均有统计学意义(P0.05)。结论股骨远端的前后轴线可以作为股骨假体旋转对线的可靠标志。TKA中,股骨后髁的截骨在参照APL旋转定位时,应依据PCL外旋5°的方法才能获得满意的股骨假体旋转对线。     

Aggressive aneurysmal bone cyst in association with polyostotic fibrous dysplasia: A case report

IntroductionAneurysmal bone cyst occurring in the setting of previously diagnosed fibrous dysplasia is rare. While both are benign processes, pain, compression of nearby structures and risk of fracture can require treatment.Presentation of caseIn this report, we describe a 56 year old male who developed an aggressive aneurysmal bone cyst secondary to fibrous dysplasia in the proximal tibia over a period of 8 months. He required an above knee amputation for disease and symptom control due to the aggressive nature of disease and medical comorbidities.DiscussionThe diagnosis of a secondary lesion can prove difficult. It is important to exclude a malignant disease process, particularly when imaging demonstrates an aggressive appearance. In this case, repeat imaging, CT guided biopsies and an open biopsy were performed to exclude malignancy prior to definitive surgical management.ConclusionIn order to exclude secondary lesions, we suggest further investigation for new onset pain in the setting of a benign lesion.     

Monostotic fibrous dysplasia of the spine: report of a case involving a cervical vertebra

Monostotic fibrous dysplasia of the spine is a rare entity. Only 26 cases, of which 11 were located in the cervical spine, are to be found in the literature. We report a 56-year-old male patient with cervicobrachialgia of half year’s duration. Radiographs showed a diffuse destruction of the vertebral body and the spinous process of C₄. A biopsy of the spinous process confirmed histopathologically a fibrous dysplasia. Due to minor symptoms, no surgical treatment was performed or is planned unless in case of increasing pain, an acute instability or neurological symptoms.     

Osteolysis of the distal femur after total knee arthroplasty

An 11.1% incidence of femoral osteolysis (30 cases in 28 patients) was identified in a series of 271 primary total knee arthroplasties. Two minimally constrained total knee designs (Synatomic [Depuy, Warsaw, IN] and Porous-Coated Anatomic [PCA, Howmedica, Rutherford, NJ]) were used in this patient population. Femoral osteolysis was observed in 26 Synatomic and 4 PCA knees. The average follow-up period was 52 months (range, 24–96 months). Osteolytic lesions were identified radiographically, adjacent to the nonporous-coated (smooth) regions of the anterior and posterior flanges of the Synatomic and PCA femoral components. The average time to the diagnosis of femoral osteolysis was 31 months (range, 7–96 months). The average patient age at the time of primary total knee arthroplasty was 63 years (range, 43–83 years) and the average weight was 180 lb. (range, 107–278 lb.). Sixteen of the 30 cases were in men. All of the cases with femoral osteolysis had cementless implantation. Tissue specimens were obtained from the 18 cases requiring revision. Implants remained in situ an average of 66 months (range, 15–96 months) prior to revision. In 16 of the 18 cases revised, the femoral component was clinically and radiographically stable. Six of 18 cases were revised for severe osteolysis. The remaining 12 cases were revised for failed metal-backed patellae, failed cementless tibial fixation, or advanced polyethylene wear. Wear of the thin tibial inserts and patellar components were the two sources of particulate polyethylene. Polyethylene debris was observed in all tissue specimens. In cases with failed metal-backed patellae or impingement of the tibial locking pin-and-clip, fine metallic debris was also noted in tissue specimens. Microscopic evaluation of the osteolytic tissue revealed a florid histiocytic response with occasional giant cells. Intracellular submicron particulate polyethylene was identified with polarized light microscopy and oil-red-O staining techniques. In the study population, statistically significant factors associated with femoral osteolysis included male gender (P < .05), younger age (P < .05), increased patient weight (P < .05), positive tibial osteolysis (P < .001), osteoarthritis (P < .07), and length of time in situ (P < .001). In addition, patients with a Synatomic prosthesis were at increased risk for osteolysis compared to patients with a PCA device (P < .02). The authors postulate that histiocytic granuloma gained access to the proximal femur primarily via the unbonded interface between the smooth metal and bone.     

带血运骨膜内组合双段腓骨移植治疗下肢长骨骨纤维结构不良致大段骨缺损

目的 探讨下肢长骨大段骨纤维结构不良切除后骨缺损的修复方法.方法 1995年5月至2003年8月,6例骨纤维结构不良患者.男5例,女1例.年龄14～40岁(平均25.5岁).胫骨干2例,股骨干4例.肿瘤切除后骨缺损的长度为15～22 cm.移植双腓骨段长度17～24 cm.均从双侧切取带腓血管的腓骨,在一端将腓血管吻合,使成一条腓血管相连的双段腓骨.将双段腓骨的前内侧骨膜从中央纵行切开并向两侧剥离至骨嵴.双段腓骨折叠,其前内侧面相对,两断端同定,将相对缘游离的骨膜纵行缝合,使其组合为一个骨膜包裹的粗骨.将与双段腓骨相连的腓血管襻在移植体一端制成"U"形,勿形成锐角.能保证血管供血不受影响.解剖出受区血管.将组合的腓骨插入骨折两断端间,如不稳定,加用钢丝或螺钉固定.精确无误地将腓血管与受区血管进行吻合,通过移植体骨膜出血情况判断血管吻合口情况.结果 随访6～10年,平均7.4年.1年后骨性愈合;5年后5例移植骨髓腔再通.移植的双腓骨未发生肿瘤,X线片显示为正常骨骼;1例股骨干于正常骨干远段复发,但移植骨部分未复发.骨移植体愈合好,无发生骨不连、骨感染等并发症.膝关节伸屈功能恢复正常,逐渐恢复日常活动与工作.结论 骨膜内组合双腓骨移植足修复下肢长骨大段缺损的良好方法 .     

病灶清除、植骨联合内固定治疗下肢长管状骨纤维结构不良的疗效和并发症观察

背景：骨纤维结构不良临床上可表现为单发或多发的骨骼畸形、疼痛和病理性骨折。目前该病的治疗方法以手术清除病灶、植骨内固定为主。目的：探讨病灶清除、植骨联合髓外固定治疗下肢长管状骨纤维结构不良的疗效和可行性。方法方法：选取2010年2月至2012年11月我院收治的15例骨纤维结构不良患者的病例资料进行回顾性分析,男8例,女7例;年龄26-48岁,平均（32.1±1.2）岁;股骨9例,胫骨6例。均采用病灶清除,取髂骨植骨,动力髋螺钉或锁定接骨板固定。围手术期予抗炎、补液对症处理,术后石膏固定,指导患者功能锻炼。髋关节功能按Harris评分系统评定。膝关节功能按Merchan评分系统评定。术后定期摄X线片,观察肿瘤复发情况,植骨融合情况和不良反应发生情况。结果：所有伤口均甲级愈合,按期拆线。所有患者均完成18个月随访,无1例复发,植骨均融合,术后部分负重下地时间（4.3±0.6）个月,X线片未见骨溶解,术后（4.0±0.8）个月可见新生骨长入,2例患者残留手术区疼痛,对症治疗后缓解。术后髋关节功能Harris评分为97.2分,膝关节功能Merchan评分为95.8分,均较术前显著提高（P〈0.05）。结论：病灶清除、植骨联合髓外固定在下肢长管状骨纤维结构不良患者中固定可靠,预后佳,是一种可行的治疗方法。     

肋骨骨纤维异常增殖症2例

病例1．患者男．33岁。发现左侧前胸壁肿块,运动后自觉胸闷、不适10余年。触诊肿块不活动．有轻压痛。实验室检查未见异常。CT：左侧第3肋骨前端呈囊状膨胀性改变,约4．12cm×8．30cm,其内可见纤细的骨嵴（图1）,向胸内生长,相邻肺组织受压．周围皮肤隆起;骨皮质变薄周边硬化,未见骨膜反应及骨质破坏改变。行左侧第3前肋骨切除术。病理：镜下可见大量增生的纤维组织、骨样组织和新生骨小梁。诊断：肋骨骨纤维异常增殖症。