Surgery for fibrous dysplasia associated with aneurysmal-bone-cystlike changes in right proximal femur:A case report

BACKGROUND Fibrous dysplasia associated with aneurysmal bone cyst(ABC)-like changes in the right proximal femur has a low incidence.It is considered more difficult to make early diagnosis than for single fibrous dysplasia.CASE SUMMARY A 14-year-old woman was admitted because of persistent pain in the right hip and abnormal gait over the previous 2 mo.She had no history of present or past illness.Preoperative photography,enhanced computed tomography,and magnetic resonance imaging showed ground-glass appearance with cortical scalloping and expansion of the right proximal femur and femoral neck.Pathological examination by preoperative puncture biopsy revealed fibrous dysplasia of the right proximal femur.The patient was diagnosed with fibrous dysplasia based on medical history,physical examination,and results of laboratory,imaging and pathological examinations.According to final pathological examination,the patient was diagnosed with fibrous dysplasia of the right proximal femur associated with ABC.Curettage and allograft along with fixation of compression screws was performed for fibrous dysplasia associated with ABClike changes.No obvious allograft absorption,loosening of fixation,or secondary fracture were observed during 6-months’follow-up with re-examination by plain radiography and computed tomography.Fibrous dysplasia associated with ABClike changes in the right proximal femur has a low incidence and early diagnosis is considered more difficult than for single fibrous dysplasia.CONCLUSION We report a cases of fibrous dysplasia associated with ABC-like changes in the right proximal femur treated with curettage and allograft along with hip compression screws.

Low-grade central osteosarcoma of distal femur, resembling fibrous dysplasia

We report a case of a 32 year-old male, admitted for a lytic lesion of the distal femur. One month after the first X-ray, clinical and imaging deterioration was evident. Open biopsy revealed fibrous dysplasia. Three months later, the lytic lesion had spread to the whole distal third of the femur reaching the articular cartilage. The malignant clinical and imaging features necessitated excision of the lesion and reconstruction with a custom-made total knee arthroplasty. Intraoperatively, no obvious soft tissue infiltration was evident. Nevertheless, an excision of the distal 15.5 cm of the femur including 3.0 cm of the surrounding muscles was finally performed. The histological examination of the excised specimen revealed central low-grade osteosarcoma. Based on the morphological features of the excised tumor, allied to the clinical findings, the diagnosis of low-grade central osteosarcoma was finally made although characters of a fibrous dysplasia were apparent. Central low-grade osteosarcoma is a rare, well-differentiated sub-type of osteosarcoma, with clinical, imaging, and histological features similar to benign tumours. Thus, initial misdiagnosis is usual with the condition commonly mistaken for fibrous dysplasia. Central low-grade osteosarcoma is usually treated with surgery alone, with rare cases of distal metastases. However, regional recurrence is quite frequent after close margin excision.

Comprehensive management of malocclusion in maxillary fibrous dysplasia:A case report

BACKGROUND Fibrous dysplasia(FD)is a developmental hamartomatous bone disease characterized by a blend of fibrous and osseous entities.Though rarely malignant,the tumor can vary from being small and asymptomatic,to a fairly large sized lesion,progressing gradually,compromising occlusion and facial esthetics.Treatment approach depends on the stage of skeletal maturity.It primarily involves surgical management for stabilizing the disease process.Post-surgical comprehensive dental treatment is necessary for restoring form and function of the jaws and teeth.This article describes comprehensive orthodontic management of severe malocclusion in a surgically operated case of FD maxilla.CASE SUMMARY A 19-year female presented with a chief complaint of excessive gingival display when smiling.Dental history included swelling of gums around the upper right front teeth,diagnosed at the age of 15 as FD of the right anterior maxillary segment and treated with surgical recontouring of the dysplastic bone.The clinical and radiological examinations showed adequate post-surgical healing.The surgically treated dysplastic area presented with right canting of the maxillary anterior occlusal plane.The maxillary teeth were torqued palatally,with the root of the right maxillary canine exposed clinically.We discuss sequential management of the associated malocclusion with comprehensive fixed orthodontics,along with special precautions taken to prevent reactivation of the quiescent and healed lesion.CONCLUSION The adequate healing of fibro-dysplastic bone post-surgery must be allowed before initiating orthodontic tooth movement in the dysplastic bone.Periodic follow-ups are needed to monitor stability of occlusion and any relapse of the lesion.

Fibula allograft transplantation combined with locking plate for treatment of recurrent monostotic fibular fibrous dysplasia:A case report

BACKGROUND Fibrous dysplasia is a congenital disorder in which normal bone is replaced by fibro-osseous tissue or irregular trabeculae of woven bone intermixed with mature collagenous tissue.A single or multiple bones are affected.This rare bone disorder has three clinical patterns including monostotic,polyostotic,and that associated with McCune-Albright syndrome.Most studies report primary fibrous dysplasia.However,a few cases of recurrent monostotic fibular fibrous dysplasia have been reported.Here,we report a therapeutic strategy for recurrent fibular fibrous dysplasia.CASE SUMMARY A 4-year-old boy was admitted for persistent pain in the left lower limb and abnormal gait over the previous 9 mo.He had no history of present or past illness.Preoperative imaging data showed erosion-like changes with bone expansion of the left middle and lower fibular segment.Tumor tissue in the fibular bone marrow cavity was removed by curettage,and rapid intraoperative pathological examination suggested fibular fibrous dysplasia.An allograft was implanted into the fibular medullary cavity.However,he was readmitted with clinical symptoms including persistent pain,abnormal gait,and local swelling at the age of 6 years.He was diagnosed with recurrent fibular fibrous dysplasia based on the second medical examination.He underwent fibular bone tumor radical resection and longus fibular allograft transplantation combined with fibular bone locking plate and screws.Good host bone to allogenic bone graft fusion was observed by the physician on postoperative regular follow-up.CONCLUSION Radical resection of fibrous dysplasia and longus fibula allograft combined with internal fixation for reconstruction are suitable for the treatment of recurrent monostotic fibular fibrous dysplasia.

Endoscopic Management in Fibrous Dysplasia of Ethmoid Sinus:One Case Report and Literatures Review

Introduction Fibrous dysplasia （FD） is a sporadic real bone structures and marrow are skeletal disorder in which norreplaced by fibro-osseous tissuet. It is classified into three types： monostotic FD, polyostotic FD, and McCune-Albright syndrome. The monostotic is the most common form of FD. The disease primarily affects the long bones of the patients, and may also develop in the craniofacial region. However, FD in the paranasal sinuses is uncommon. The case in this report was diagnosed as FD in the ethmoid sinus which is a rarely seen case.

Surgical Management of 4 Cases of Craniofacial Fibrous Dysplasia

Background: Fibrous dysplasia is an uncommon skeletal disorder in which normal bone and marrow are replaced with fibro-osseous tissue. The disease comprises 2.5% of all bone tumors and 7.5% of all benign bone neoplasm. It is the progressive, slowly developing disease and the optimum treatment remains unclear in many cases. Aim: In this study, the authors report their experience in the surgical treatment of four cases of craniofacial fibrous dysplasia. Cases presentation: The study involved 4 patients with craniofacial fibrous dysplasia. There were 3 men and a woman. The patients were 10, 17, 20 and 8 years old. No patient had a focal neurological deficit. The CT scan appearance was compatible with Fibrous dysplasia in all patients. The site of disease was frontal in one case and parietal in the other 3 cases. Cosmetic surgical treatment was performed in all patients. Cranioplasty was performed in one patient and planned for the other three. Conclusion: Fibrous Dysplasia is a benign slow growing disease that may cause as well as clinical symptom and aesthetical discomfort. Radical resection, if possible, is the only technique to obtain resolution of the disease.

Surgical Treatment of Monostotic Craino-Facial Fibrous Dysplasia: Changing the Narratives

Background: Fibrous dysplasia mainly presents in its monostotic form in the cranio-facial region with serious cosmetic disfigurement and functional derangement of the affected and adjacent structures putting both patient and the attending surgeon in great dilemma. Surgical treatment is the only rewarding and generally accepted treatment option, however, controversy over the surgical technique to be adopted still exists. While in the past, surgeons generally adopted conservative shaving or contouring technique, over the recent years, advocates of radical surgery are winning more disciples. Objective: To highlight the locally destructive, functionally degrading nature of a neglected or poorly excised (shaved) lesion in patients and highlight the outcome of total excision and surgical technique adopted to obviate the need for autologous bone grafting and two-staged surgery. Subjects and Method: We present case series of three patients with giant monostotic fibrous dysplasia of the maxilla, surgically treated in our Centre, who were part of a total of eight cases managed over the past fifteen years in our department of Ear, Nose and Throat-Head and Neck Surgery. The pre-operative clinical assessment, relevant investigations and post-operative outcome are presented. Our surgical technique is highlighted. All the patients had unilateral lesion of the maxilla with gross cosmetic and functional defects. Two of the patients had ischaemic (pressure) atrophy of the cheek soft tissue and skin leading to skin metaplastic changes including leukoplakia, hyperpigmentation. Post-operative follow-up showed satisfactory cosmetic outcome and significant reversal of malocclusion and dental anarchy. There was no recorded recurrence throughout the follow-up period ranging from four to eleven years. Nasal airway was re-established bilaterally in all the cases. Conclusion: Total or near total excision surgical technique with periosteal preservation is our treatment of choice in the management of monostotic cranio-facial fibrous dysplasia. Given the fact that the growth of the tumours often does not cease after puberty against general belief, shaving or contouring technique should be relegated to the background. Our technique of no grafting which reduced cost and morbidity to the patient should be encouraged.

Survival of dental implants in patients with bone dysplasia:A systematic review

Objective:This study used published studies to assess the survival rate of dental implants placed in patients with bone dysplasia of the maxillofacial region.Material and methods:An electronic search without a specified date range was performed using the MEDLINE,PubMed,EMBASE,Web of Science,and Cochrane databases.No gender or age restrictions were applied.Results:Eighteen publications were found that met the study’s criteria,reporting data on 18 patients with bone dysplasia including cleidocranial dysplasia(CDD),fibrous dysplasia(FD),florid cemento-osseous dysplasia(FCOD),and odonto-maxillary segmental dysplasia(SOMD),who received a total of 130 implants,an average of 7.2 implants/patient(range 1 to 16).The mean age of the patients was 36.7 years(range 15 to 70 years).For implants placed in bone dysplasia,the survival rates were 100% for patients with CDD(n=8),FD(n=5),SOMD(n=2),FCOD with implants inserted far from the lesions(n=2)and 0% for dental implants inserted within FCOD(n=1).The mean follow-up was 38.2 months(min 6,max 60).Conclusions:Dental implants placed in patients with dysplastic bone lesions show high survival rates,similar to those in the general population for CDD,FD,and SOMD.For FCOD,the failure rate was 100%.

Revision of Outcomes and Complications Following Open Reduction, and Zigzag Osteotomy Combined with Fibular Allograft for Developmental Dysplasia of the Hip in Children

Background: Reports of the efficacy of open reduction and Zigzag Osteotomy combined Fibular Allograft (ZOFA) for developmental dysplasia of the hip. The purposes of this study were to evaluate the long-term outcomes and complications after surgery. Methods: We performed a retrospective match-controlled study in which 158 patients had 181 hips with developmental dysplasia of the hip. Radiographs were found of acetabular index, height of dislocation, Tönnis grade, abduction angle in the spica cast, and Severin grade. At final follow-up, deformity of femoral head or neck or acetabulum was evaluated according to the Severin. Avascular necrosis was rated according to Kalamchi. Clinical evaluation was made according to modified McKay criteria. Results: Between 2009 and 2012, 133 girls (84.2%) and 25 boys (15.8%) with developmental dysplasia of the hip underwent open reduction and ZOFA;135 (85.4%) were unilateral, and 23 (14.6%) were bilateral. Patients were divided into 2 groups: group 1 included 54 patients (62 hips) aged 12 months - ≤18 months and group 2 included 84 patients (119 hips), aged >18 months - ≤36 months. According to Tönnis system: type 3 appeared in 127 hips (70.2%), and Type 4 in 54 hip (29.8%). The anterior approach was used to expose inner table of the ilium and ZOFA in all cases. Acetabular index was improved;preoperation was 42.95°, and latest follow-up 17.26°. The Kirschner Wires (KW) were not used to fix the fibular allograft at the pelvic osteotomy site. All of the fibular allografts were completely incorporated in mean time of 14 weeks (range, 12 weeks - 17 weeks) post-surgery. Clinical evaluation according to modified McKay criteria: satisfactory result (excellent and good) was achieved in 141 hips (77.9%). Avascular Necrosis (AVN) happened in 61 hips (33.7%), redislocation in 18 hips (9.9%), coxa vara in 4 hips (2.2%), trendelenburg gait in 4 hips (2.2%), and supracondylar femoral fractures in 2 hips (1.1%). Conclusions: On the basis of this study, ZOFA was strength and graft was not resorption, graft problems;without medial displacement of the distal fragment. Acetabular index was improved, without KW problem. Surgical technique with ZOFA did not expose outer table of the illium, limiting abductor muscle injury with negative trendelenburg gait;on the other hand, the blood loss from this procedure is acceptable. Some complications have been seen in this study: AVN, redislocation, coxa magna, coxa vara, trendelenburg gait, and distal femoral fracture.

Enchondromas of Long Bones and Other Skeletal Lesions Found Incidentally Need Critical Evaluation, But Rarely Systematic Follow-Up

Purpose: Incidental bone lesions are a challenge for the specialist, who has to give recommendations for further management. This review of our cases will assist in the decision whether the lesion can be “neglected”, needs further active follow-up or direct initiation of treatment. Patients and Methods: 153 cases of incidental bone findings were presented to our musculoskeletal tumor service for evaluation from July 2008 through June 2021. 73 of them were cartilaginous tumors and 63 of these were diagnosed as enchondroma of a long bone based on X-Ray and MRI. Results: Follow-up imaging of the enchondroma patients was available for 35 patients at 1 to 13 years (mean 4.3 y), with no change in size except for one femoral diaphyseal enchondroma with increasing diameter from age 18 to 20 years. 14 additional patients answered written contact stating that they remained asymptomatic at 2 to 12 years (mean 5.6 y). None of the patients has been reported to the Swiss Confoederation Cancer Registry to have developed malignancy. Among the 10 other cartilaginous tumors were one chondrosarcoma grade II exhibiting different imaging, 3 non-long-bone localizations (pelvis, scapula and rib), 2 Ollier-type enchondromas, and 2 osteochondromas. Incidental findings other than cartilaginous tumors were fibrous dysplasia (n = 31), non-ossifying fibroma (n = 31) and 18 other “sporadic” entities. Conclusions: Incidentally found enchondromas not exhibiting aggressive features need no systematic follow-up and patients can be “discharged” with the advice to present, if symptoms would develop. This also applies to fibrous dysplasia and the other sporadic lesions. 6 cases with other diagnoses needed specific treatment.

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。

经鼻内镜联合右侧眉弓入路治疗右侧额筛窦骨纤维异常增殖症1例并文献复习

目的探讨鼻腔鼻窦骨纤维异常增殖症的治疗方法。方法总结亳州市人民医院2018年5月收治的1例右侧额筛窦骨纤维异常增殖症的临床资料,包括其病史、影像学资料、术中术后情况以及治疗经过。结果病人以头痛1年余、伴右眼胀痛不适半年入院,CT影像诊断为右侧额窦、筛窦区异常密度灶⁃考虑偏良性病变,骨纤维结构发育不良可能,请结合临床;鼻中隔偏曲,双侧下鼻甲肥大。MRI平扫+增强影像诊断为右侧筛窦、额窦窦腔内及右侧眶上壁异常信号,考虑骨瘤,不除外骨纤维异常增殖症或骨化纤维瘤,请结合临床;鼻中隔偏曲。行经鼻内镜联合右侧眉弓入路治疗,彻底切除鼻窦病变组织,手术顺利。术后病理检查示(右侧额筛窦)镜检骨小梁不规则,间质为纤维结缔组织。术后病人临床症状消失。随访半年无复发。结论采取经鼻腔及右侧眉弓联合径路,可以彻底切除鼻窦病灶,具有视野清晰、微创性、恢复快等特点,值得临床应用推广。

鼻内镜手术治疗颅面骨骨纤维异常增殖症13例

目的探讨鼻内镜手术治疗颅面骨骨纤维异常增殖症的优、缺点。方法回顾性分析13例行鼻内镜手术治疗的颅面骨骨纤维异常增殖症患者的病历资料。结果术后症状明显缓解,随访1~5年未见复发和手术并发症。结论鼻内镜手术治疗颅面骨骨纤维异常增殖是一种更有效,更小损伤的手术方法。

眶颅骨纤维异常增殖症病灶手术切除与缺损区钛金属修复

目的：研究眶颅骨纤维异常增殖症手术切除病灶的时机和方法，总结病灶切除后钛金属修复骨缺损的经验，对手术的疗效进行评估。方法：对21例出现视功能障碍或明显外观影响的患者进行术前常规冠状位、水平位、矢状位及三维CT扫描。对眶颅骨病变范围大的患者，将CT扫描图像数据输入计算机，经过处理后制作钛金属三维实体修复体；对于累及眶颅骨病变范围较小的患者，则用手工的方法在手术中制作近似于正常眼眶和颅骨部的修复体。全部病例均采用经颅手术切除病变骨组织，并用钛板和钛网修复缺损区。结果：2l例患者中18例一次性切除干净，3例累及海绵窦区域者进行姑息性切除。13例伴有不同程度的视力下降，11例术后视力明显上升，视力恢复在3-5行。外观畸形的11例患者术后得到明显改善。结论：眶颅骨纤维异常增殖症患者只要影响到视功能或造成眶颅外观畸形，应该尽早进行手术切除。钛金属修复手术后遗留的骨缺损具有牢固、可塑性强、固定简易等优点。

颌面部骨纤维异常增殖行局部骨骼切削塑形术患者围术期护理

目的总结颌面部骨纤维异常增殖行局部骨骼切削塑形术患者的围术期护理措施,为临床规范护理策略的制定提供借鉴。方法对2016年5月~2020年10月在我院接受局部骨骼切削塑形术的72例颌面部骨纤维异常增殖症患者实施健康宣教、心理干预、营养评估、术前准备及呼吸放松训练、术中护理、术后疼痛护理、创面观察及并发症预防护理、康复训练、出院指导等系统化围手术期护理。结果72例患者均顺利完成局部骨骼切削塑形术,面部不对称、眼球移位、视力减退、张口困难等症状均明显改善;术后发生局部出血3例,应用止血药物控制;1例出现面部软组织下垂,经面部悬吊术后纠正;所有患者手术创面均为甲级愈合,无明显瘢痕及色素沉,面部对称良好,患者对塑形效果满意,护理满意度为100%。结论局部骨骼切削塑形术是治疗颌面部骨纤维异常增殖症的首选手段,系统围手术期护理是保证手术顺利完成,降低术后并发症,提升颌面部塑形效果的重要保障措施。

Diagnosis and treatment of McCune-Albright syndrome:A case report

BACKGROUND McCune-Albright syndrome(MAS)is extremely rare clinically.We here report a case of MAS with severe symptoms that have not been reported previously.CASE SUMMARY A 10-year-old boy attended our outpatient clinic due to craniofacial malformations found two years ago.He underwent temporal bone computed tomography and digital radiography photography.Based on a literature review combined with the patient's medical history and imaging examination findings,he was diagnosed with multiple fibrous dysplasia of bone.As the clinical symptoms related to MAS in this patient were not obvious,he was only followed up and not given any special treatment.CONCLUSION The unique clinical manifestations in this MAS patient may be related to mutations in the GNAS gene.

骨纤维结构不良发病机制及外科治疗现状

骨纤维结构不良是一类良性骨病变,其发病机制和外科治疗的预后仍存在争议。骨纤维结构不良的发病机制包括基因突变,染色体结构和数目变异,内分泌异常和骨发育异常。股骨是骨纤维结构不良最常累及的部位,股骨纤维结构不良的分型目前缺乏较为公认的分型方式。植骨材料和手术方式的选择目前仍存在争议。本综述旨在对骨纤维结构不良发病机制和外科治疗的相关研究进行综述,为该疾病的外科治疗提供较为全面的认识。

上颌骨骨纤维异常增殖症的外科治疗

对 １９ 例上颌骨骨异常增殖症患者行手术切除和骨创面冷冻治疗，１７ 例术后颜面畸形矫正满意，随访 ２～１０ 年无复发，２ 例分别在术后 ６ 个月及 １ 年复发，再次手术治愈，无 １ 例发生骨髓炎。此法临床易掌握，疗效满意，是治疗上颌骨骨纤维异常增殖症较理想的方法。

Crowe Ⅳ型发育性髋关节发育不良初次人工全髋关节置换术后翻修手术的研究进展

目的对Crowe Ⅳ型发育性髋关节发育不良(developmental dysplasia of the hip,DDH)患者初次人工全髋关节置换术(total hip arthroplasty,THA)后翻修手术的研究进展进行综述。方法回顾近年来有关Crowe Ⅳ型DDH患者初次THA术后翻修的研究文献,分析翻修手术原因,归纳翻修手术难点、处理方法以及相关假体选择。结果Crowe Ⅳ型DDH患者髋臼前后径小、髋臼及股骨前倾角变异大、软组织挛缩严重,THA及翻修手术难度均较大。导致此类患者初次置换后翻修的原因较多,主要为假体无菌性松动。因此,初次THA时需尽可能恢复异常解剖结构、减少磨损颗粒产生,以避免术后假体松动发生。Crowe Ⅳ型DDH患者由于其解剖结构特殊,翻修术中常存在髋臼侧及股骨侧骨缺损,对于骨缺损的修复重建成为手术关键。其中,髋臼侧通常根据骨缺损程度选择合适的髋臼杯或联合金属块、Cage、定制组件等进行重建,股骨侧则首选S-ROM假体。此外,假体界面应尽可能选择陶瓷-陶瓷或陶瓷-高交联聚乙烯。结论Crowe Ⅳ型DDH患者初次THA术后导致翻修的原因以及手术难点已明确,大量临床研究基于此提出了相应的翻修方式并获得了较好早中期疗效,但远期疗效仍需进一步随访明确。随着技术发展与新型材料的研发,制备此类患者的个性化适配假体有望成为现实。

数字化技术辅助治疗额眶部骨纤维异常增殖症

目的探讨数字化技术在额眶部骨纤维异常增殖症患者手术治疗中应用的效果。方法回顾性分析2016年2月和2018年2月中国医学科学院整形外科医院收治的2例额眶部骨纤维异常增殖症患者的临床资料。患者均为男性,年龄分别为18岁和16岁。术前对患者行CT扫描获取影像学数据,导入Mimics 10.01中进行颅骨三维重建和图像分析,设计骨纤维异常增殖症病灶切除范围,然后三维打印头颅缺损模型,并制作覆盖颅骨缺损的个性化钛修复体,再虚拟设计自体颅骨外板取骨范围并模拟修复眶骨缺损。根据设计方案进行手术。术后观察患者恢复情况和并发症发生情况,行头颅CT扫描,测量眶容积、眼球突出度,检查视力,评估手术效果。结果2例患者术中肉眼所见病灶边界与术前模拟设计相符,术中所见颞骨为正常骨质。术后1例患者恢复良好,另1例术后1 d出现脑脊液鼻漏,未经特殊处理,2周后自愈。术后CT显示个性化修复体与颅骨结合良好,额眶部形态满意,接近正常解剖形态。术后2例患侧眶容积增大,比术前分别增加了2.99、9.99 ml;术后眼球突出度得到改善,比术前分别减小3.32、5.11 mm;术后视力与术前相同,眼球各个方向运动良好。患者及家属对形态满意。结论将数字化技术用于额眶部骨纤维异常增殖症术前的模拟设计,有利于术者精确地制定手术计划,提高移植骨的利用效率,术后可获得满意的效果。