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Luo K, Yang M, Dai W, Teng H, Huang W, Liu S, Xiao D, Hu J, Deng J, Quan H, Song E, Liu Y. Arthroscopic-assisted uni-portal non-coaxial endoscopic surgery treatment of fibrous dysplasia of the femur: a minimally invasive alternative to open surgery. BMC Musculoskelet Disord 2025; 26:360. [PMID: 40221733 PMCID: PMC11992799 DOI: 10.1186/s12891-025-08617-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2025] [Accepted: 04/03/2025] [Indexed: 04/14/2025] Open
Abstract
BACKGROUND Fibrous dysplasia of bone (FD) is a rare skeletal disorder. Traditional surgical options may have disadvantages such as greater trauma, incomplete curettage and recurrence risk. In this report, we introduce the technique for the treatment of fibrous dysplasia of the femur with Arthroscopic-assisted uni-portal non-coaxial endoscopic surgery (AUNES) and evaluate its feasibility. CASE PRESENTATION We described a 49-year-old female patient with an elliptical lesion measuring approximately 4.3 cm × 4.2 cm in the proximal left femur. Due to the limitations of traditional open surgery-significant trauma, incomplete curettage, and a high risk of recurrence-we performed tumor curettage and bone graft fusion with AUNES assistance for the first time. The AUNES endoscopy provided a clear surgical field, precise lesion boundaries, thorough curettage, and minimal blood loss. Postoperative pathology confirmed fibrous dysplasia. At the 1-month and 3-month postoperative follow-ups, the patient's surgical wound healed well without complications. DR and CT scans showed satisfactory graft placement and bone fusion. CONCLUSION This case report demonstrates that AUNES-assisted tumor lesion curettage and bone grafting fusion surgery for FD is safe and feasible. It offers several advantages, including minimal invasiveness, a wide surgical field, thorough lesion curettage, and a low postoperative recurrence rate.
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Affiliation(s)
- Kai Luo
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Mingxiu Yang
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Wei Dai
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Hongcai Teng
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Wenxian Huang
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Shangyu Liu
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Danting Xiao
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Jianming Hu
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Jingxin Deng
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - Haiyi Quan
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China
| | - En Song
- Department of Sports Medicine, The First Affiliated Hospital of Kunming Medical University, Kunming, 650032, China.
| | - Yun Liu
- Department of Spine and Osteopathy, The First Affiliated Hospital of Guangxi Medical University, Nanning, 530021, China.
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Soveral Pereira T, Malheiro N, Gonçalves D, Rodrigues C, Gonçalves PJ. Surgical treatment of fibrous dysplasia in the proximal femur: a literature review. EUROPEAN JOURNAL OF ORTHOPAEDIC SURGERY & TRAUMATOLOGY : ORTHOPEDIE TRAUMATOLOGIE 2025; 35:148. [PMID: 40186713 DOI: 10.1007/s00590-025-04271-y] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/20/2024] [Accepted: 03/18/2025] [Indexed: 04/07/2025]
Abstract
PURPOSE Fibrous dysplasia is an uncommon disease with heterogeneous presentations and patterns of bone involvement. Lesions around the proximal femur are the most common. The article reviews the different surgical options described in the literature and summarizes recent recommendations in managing proximal femoral lesions. METHODS A comprehensive review of the literature was conducted to analyze surgical techniques, including curettage with bone grafting, osteotomies for deformity correction, fixation methods for fractures, and total hip arthroplasty (THA). RESULTS Curettage and bone grafting, while traditionally used, are largely discouraged due to high recurrence rates and limited efficacy. Osteotomies, particularly valgus intertrochanteric and subtrochanteric osteotomies, are effective for correcting deformities such as the Shepherd's crook deformity, with intramedullary fixation currently being considered the gold standard. Fracture fixation remains individualized, with a range of implants, including intramedullary nails and fixed-angle devices, chosen according to the fracture pattern, localization of the lesion within the proximal femur and patient age. THA is a viable option for end-stage osteoarthritis as well as select cases of acute fractures, with cementless components, augmented by bone grafts when necessary, showing favorable results. CONCLUSIONS Surgical management of proximal femoral FD is multifaceted and tailored to individual patient presentations. Despite advancements, there is still no standardized approach, emphasizing the need for high-quality studies to establish consensus guidelines.
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Affiliation(s)
| | - Nuno Malheiro
- Unidade Local de Saúde do Alto Minho (ULSAM), Viana do Castelo, Portugal
| | - Daniel Gonçalves
- Unidade Local de Saúde do Alto Minho (ULSAM), Viana do Castelo, Portugal
| | - Catarina Rodrigues
- Unidade Local de Saúde do Alto Minho (ULSAM), Viana do Castelo, Portugal
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Paris E, De Marco G, Vazquez O, Steiger C, Boudabbous S, Dayer R, Ceroni D. A narrative review of the literature on the pediatric orthopedic management of fibrous dysplasia. Front Pediatr 2025; 12:1502262. [PMID: 39981406 PMCID: PMC11841395 DOI: 10.3389/fped.2024.1502262] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/26/2024] [Accepted: 12/31/2024] [Indexed: 02/22/2025] Open
Abstract
Fibrous dysplasia is a congenital, non-inherited, benign intramedullary bone lesion in which the normal bone marrow is replaced by abnormal fibro-osseous tissue. The disorder can be monostotic (involving a single bone) or polyostotic (involving multiple bones). As the abnormal fibro-osseous tissue compromises the mechanical strength of bone, it can result in pain, deformity, fractures, or abnormalities in bone mechanics with inappropriate bone alignment. This narrative review attempts to summarize more than 20 years of observations of patients with FD to help pediatric orthopedists establish a care framework that can improve its identification, understand the impact that endocrinopathies can have on its clinical presentation, and optimize the management of bone disorders. Our focus is specifically on orthopedic manifestations of FD and modern management alternatives. The past 20 years have provided major advances in understanding of fibrous dysplasia (FD), and it is clear that the pediatric orthopedist's role remains highly relevant in the management of all types of FD. Surgical treatment remains appropriate when pain is unresponsive to other medical treatments, when a pathological fracture is impending or has happened, when a deformity is worsening or has formed, or when there is a suspicion of malignant transformation. The pediatric orthopedist must be aware, therefore, of the particularities of the different bones on which they may be called to intervene, and they should give very careful consideration to their operative strategy, which must be adjusted to the biological and static characteristics of the bone.
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Affiliation(s)
- Elio Paris
- Faculty of Medicine, University of Geneva, Geneva, Switzerland
| | - Giacomo De Marco
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, Switzerland
| | - Oscar Vazquez
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, Switzerland
| | - Christina Steiger
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, Switzerland
| | - Sana Boudabbous
- Radiology Department, Geneva University Hospitals, Geneva, Switzerland
| | - Romain Dayer
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, Switzerland
| | - Dimitri Ceroni
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospitals, Geneva, Switzerland
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Paris E, De Marco G, Vazquez O, Boudabbous S, Steiger C, Dayer R, Ceroni D. Polyostotic fibrous dysplasia with epiphyseal involvement of the proximal femur in a child: a case report and review of the literature. Front Pediatr 2025; 12:1505766. [PMID: 39902226 PMCID: PMC11788400 DOI: 10.3389/fped.2024.1505766] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/08/2024] [Accepted: 12/31/2024] [Indexed: 02/05/2025] Open
Abstract
Fibrous dysplasia (FD) is a benign medullary fibro-osseous anomaly that compromises the mechanical strength of bones, especially the long bones that bear strong mechanical stresses. It can lead to an inability to remodel immature bone into mature lamellar bone, resulting in inappropriate bone alignment in response to mechanical stresses. This case study describes a rare case of polyostotic FD presenting with an epiphyseal lesion of the proximal femoral head in its weight-bearing zone, accompanied by an unconventional femoral malrotation. The present case leads us to recommend that clinicians should not underestimate the occurrence of other deformities, such as the retrotorsion or flexion deformities that can compromise bone structure and the hip's biomechanics. Finally, the involvement of the epiphysis is probably more common than usually thought, introducing an additional complexity since juxta-articular lesions in weight-bearing joints may collapse, compromising articular congruence and function. To minimise this risk, bone scintigraphy and MRI should play a critical role in the patient's workup, evaluation, prognosis and follow-up.
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Affiliation(s)
- Elio Paris
- Faculty of Medicine, University of Geneva, Geneva, Switzerland
| | - Giacomo De Marco
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, Switzerland
| | - Oscar Vazquez
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, Switzerland
| | - Sana Boudabbous
- Radiology Department, Geneva University Hospital, Geneva, Switzerland
| | - Christina Steiger
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, Switzerland
| | - Romain Dayer
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, Switzerland
| | - Dimitri Ceroni
- Pediatric Orthopedic Unit, Pediatric Surgery Service, Geneva University Hospital, Geneva, Switzerland
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Jayasoorya A, Pisulkar G, Samal N, Taywade S, Vasavada SN. A Rare Case of Monostotic Fibrous Dysplasia of the Femoral Neck With Pathological Fracture: A Case Report. Cureus 2023; 15:e49085. [PMID: 38125230 PMCID: PMC10731395 DOI: 10.7759/cureus.49085] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/28/2023] [Accepted: 11/20/2023] [Indexed: 12/23/2023] Open
Abstract
Fibrous dysplasia is a rare benign bone disorder characterized by the replacement of normal bone with fibroblastic and osteoblastic tissue. We present a case of monostotic fibrous dysplasia in a 25-year-old male patient. The case highlights the clinical presentation, radiographic features, and management approach for this condition. This report aims to contribute to the understanding of fibrous dysplasia and its management options. A 25-year-old male presented with a chief complaint of persistent left hip pain. The pain was described as a dull ache, associated with difficulty in weight-bearing activities. There was no history of trauma or constitutional symptoms. Physical examination revealed externally rotated left lower limb. Range of motion of the left hip could not be assessed due to pain, with no neurological deficits noted. Initial imaging included plain radiographs of the right femur, which demonstrated a radiolucent lesion with a ground-glass appearance and cortical thinning. Magnetic resonance imaging of both hip joints reveals an irregular T2 hyperintense and T1 hypointense lesion involving the left femoral neck; moreover, few tiny cystic spaces are seen within the lesion. Part of the lesion is extending into the superior-lateral aspect of the femoral head and surrounding bone marrow edema with minimal left hip joint effusion, features suggestive of a primary bony tumour. Plain computed tomography (CT) of the hip joint and pelvis was suggestive of an expansile lytic lesion with thin bony septation within and thick sclerotic margin in the left femoral head and greater trochanter associated with sub-capital femoral neck fracture suggestive of bone neoplasm (? giant cell tumour > simple bone cyst). A bone biopsy was performed, and histopathological examination confirmed the diagnosis of fibrous dysplasia, with characteristic woven bone and fibrous stroma. In this case, after confirming the diagnosis, the patient was managed with total hip arthroplasty on the left side. Monostotic fibrous dysplasia is a rare benign bone disorder that can present with various clinical manifestations. Timely diagnosis through a combination of clinical, radiographic, and histopathological assessments is crucial. Management should be tailored to the patient's symptoms.
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Affiliation(s)
- Adarsh Jayasoorya
- Department of Orthopedics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Gajanan Pisulkar
- Department of Orthopaedic Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Nitin Samal
- Department of Orthopedics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Shounak Taywade
- Department of Orthopaedic Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
| | - Shrut N Vasavada
- Department of Orthopedics, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND
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Yang Q, Liu J, Tan L, Jiang Y, Zhu D. Polyostotic Fibrous Dysplasia Complicated by Pathological Fracture of Right Femoral Shaft with Nonunion: A Case Report. Front Surg 2022; 9:879550. [PMID: 35495762 PMCID: PMC9039455 DOI: 10.3389/fsurg.2022.879550] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/19/2022] [Accepted: 03/28/2022] [Indexed: 11/23/2022] Open
Abstract
Introduction Fibrous dysplasia is a benign fibrous bone tumor that accounts for 5% to 10% of benign bone tumors. It can manifest as simple fibrous dysplasia (70%–80%), polyostotic fibrous dysplasia (20%–30%), with approximately the same incidence in men and women. We report a patient with a rare case of multiple fibrous dysplasia combined with proximal femoral shepherd deformity with pathological fracture of the femoral shaft complicated by nonunion. It is necessary to understand the disease in more detail to avoid overtreatment of benign lesions or misdiagnosis of malignant tumors and other diseases. Case presentation A 58-year-old man with polyostotic fibrous dysplasia, bilateral proximal femur deformity, Shepherd’s angle deformity, right femoral shaft pathological fracture complicated by nonunion, we under fluoroscopy, in the obvious proximal fracture, take osteotomy, and process the shape of the cut bone fragment to adapt it to the corrected force line, and then restore it back to its original position, using intramedullary nailing technology complete the operation. Three months after the operation, he came to the hospital for re-examination, and an X-ray of the right femur was taken. It was found that the fractured end had a tendency to heal. The patient was instructed to gradually bear weight. After six months of re-examination, the patient could walk with a walker. One year after the operation, the patient could walk without a walker and take care of himself at home. However, there was still stretch-like pain in the right lower back, but it was tolerable. Conclusions For patients with polyostotic fibrous dysplasia combined with proximal femoral shepherd deformity and pathological fracture of the femoral shaft with nonunion, osteotomy combined with intramedullary nailing is a simple and convenient way to correct the deformity and obtain correct fracture alignment.
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Affiliation(s)
- Qifan Yang
- Department of Orthopedics, The First Hospital of Jilin University, Changchun, China
| | - Jing Liu
- The First Clinical Medical College of Bin Zhou Medical College, Binzhou, China
| | - Lei Tan
- Department of Orthopedics, The First Hospital of Jilin University, Changchun, China
| | - Ye Jiang
- Department of Orthopedics, The First Hospital of Jilin University, Changchun, China
| | - Dong Zhu
- Department of Orthopedics, The First Hospital of Jilin University, Changchun, China
- Correspondence: Dong Zhu zhu
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Liu Z, Yin Y, Wang Z, Xie L, Deng P, Wang D, Ji N, Zhao H, Han X, Chen Q, Chung CH, Bai D, Zhao X. RANKL inhibition halts lesion progression and promotes bone remineralization in mice with fibrous dysplasia. Bone 2022; 156:116301. [PMID: 34952228 DOI: 10.1016/j.bone.2021.116301] [Citation(s) in RCA: 11] [Impact Index Per Article: 3.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/23/2021] [Revised: 11/07/2021] [Accepted: 12/13/2021] [Indexed: 02/08/2023]
Abstract
Fibrous dysplasia (FD) is a rare bone disease caused by GNAS mutation in skeletal stem cells, typically originating from and worsening in childhood. Till now, no cure for FD exists despite the well-recognized etiology. Studies have demonstrated that osteoclastogenesis hyperactivity is caused by elevated RANKL expression, making RANKL inhibition a potential therapy. Although a human monoclonal anti-RANKL antibody, denosumab, has been used in FD patients, the effects and mechanisms of RANKL inhibition for FD treatment require assessment. Denosumab is expensive and can only be injected. Therefore, formulating an oral-administered, cost-effective medicine is encouraged. In the current study, we evaluated the effects of a small-molecule RANKL inhibitor, AS2676293, on a transgenic FD mouse model. AS2676293 effectively suppressed osteoclastogenesis and halted FD progression. The pre-existing bone defects were primarily replaced by newly formed mineralized bone after two weeks of AS2676293 administration. The potent RANKL inhibitory effect and easier route of delivery make AS2676293 a promising target therapy of FD. Results from our study suggested that RANKL inhibition is effective in halting FD progression and promoting bone remineralization, which could benefit the patients with early onset of FD.
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Affiliation(s)
- Zhongyu Liu
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Yijia Yin
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Zheng Wang
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Liang Xie
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Peng Deng
- Laboratory of Molecular Signaling, Division of Oral Biology and Medicine, School of Dentistry, UCLA, Los Angeles, CA 90095, USA
| | - Donghui Wang
- School of Materials Science and Engineering, Hebei University of Technology, Tianjin 300130, China
| | - Ning Ji
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Hang Zhao
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Xianglong Han
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Qianming Chen
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China
| | - Chun-Hsi Chung
- Department of Orthodontics, School of Dental Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA
| | - Ding Bai
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.
| | - Xuefeng Zhao
- State Key Laboratory of Oral Diseases, National Clinical Research Center for Oral Diseases, West China Hospital of Stomatology, Sichuan University, Chengdu 610041, China.
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Vyas A, Godara A, Kumar N, Singhal S, Bhattacherjee D. Fibrous Dysplasia of Proximal Femur: A Case Report of Treatment With Single-Stage Valgus Osteotomy With Dynamic Hip Screw and Fibular Strut Graft. Cureus 2022; 14:e21496. [PMID: 35223273 PMCID: PMC8860356 DOI: 10.7759/cureus.21496] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/21/2022] [Indexed: 11/29/2022] Open
Abstract
Fibrous dysplasia is a rare benign intramedullary fibro-osseous lesion characterized by fibro-osseous proliferation with intervening areas of normal or immature bone. It can either be a monostotic or a polyostotic presentation. The etiology of fibrous dysplasia has been linked with a missense mutation in the GNAS1 gene on chromosome 20. The most common fibrous dysplasia is first diagnosed in children or young adults. There is no gender predilection. Overall, fibrous dysplasia constitutes 5% of all benign bone lesions.The monostotic form is the most frequent, accounting for 75% to 80% of fibrous dysplasia cases. We report a case of unilateral monostotic fibrous dysplasia in a 30-year-old male in the proximal femur with Shepherd's crook deformity. The patient underwent a single-stage procedure of curettage of lesion and valgus osteotomy with dynamic hip screw (DHS) fixation and fibular strut graft. The lesion resulted in alteration of hip joint anatomy with a decrease in the neck-shaft angle to 114 degrees and leading to coxa vara. After surgical correction, the neck-shaft angle was restored to 130 degrees. The patient was followed up in the outpatient department (OPD), x-rays were taken, and signs of radiological healing were seen at three months. Partial weight-bearing was allowed at three months postoperatively and full weight-bearing at six months with no restriction in the activity. After six months, the patient was able to perform all activities without any difficulty, and shortening of 1.5 cm was compensated with footwear modification. No evidence of recurrence was noted in the follow-up x-ray. Fibrous dysplasia of proximal femur treated with curettage and bone grafting and supported with an osteotomy to correct mechanical alignment provides excellent results. DHS, though old hardware, provides a versatile option to support osteotomy and helps in maintaining the correction. To support the neck femur after curettage, the fibula strut graft provides an excellent option. When the procedure is done in a single stage, it gives good functional and radiological outcomes along with early rehabilitation.
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Hampton MJ, Weston-Simmons S, Giles SN, Fernandes JA. Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience. Strategies Trauma Limb Reconstr 2021; 16:41-45. [PMID: 34326901 PMCID: PMC8311743 DOI: 10.5005/jp-journals-10080-1523] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Abstract
Introduction Fibrous dysplasia (FD) of bone can be present with pain, deformity and pathological fractures. Management is both medical and surgical. Little literature exists on the surgical management of both monostotic and polyostotic FD. We present our experience of limb reconstruction surgery in this pathological group of bone disease. Materials and methods A retrospective cohort of children who underwent limb reconstruction surgery at a single high-volume paediatric centre was identified from a prospective database. Case notes and radiographs were reviewed. Surgical techniques, outcomes and difficulties were explored. Results Twenty-one patients were identified aged between 7 and 13 at presentation to the limb reconstruction unit. Eleven were female, nine had McCune-Albright syndrome, seven had polyostotic FD and five had monostotic. Proximal femoral varus procurvatum deformity was the most common site requiring surgical intervention. The distal femur, tibia, humerus and forearm were also treated. Methods include deformity correction with intramedullary fixation including endo-exo-endo techniques, elastic nailing, guided growth, circular fixator technique and fixator-assisted plating. Correction of deformity and leg length discrepancies was common. The osteotomies went on to heal with no nonunions or delayed healing. We encountered secondary deformity at distal end of nails as the children grew as expected. These were managed with revision nailing techniques and in some cases external fixation. There was one implant failure, which did not require revision surgery. Conclusion The surgical management of pathological bone disease is challenging. Corrective osteotomies with intramedullary fixation can be very successful if appropriate limb reconstruction principles are adhered to. Deformity correction, guided growth and lengthening can all be successfully achieved in bone affected by FD. Polyostotic FD can be present with secondary deformities, and these can be difficult to manage. How to cite this article Hampton MJ, Weston-Simmons S, Giles SN, et al. Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience. Strategies Trauma Limb Reconstr 2021;16(1):41–45.
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Affiliation(s)
- Matthew J Hampton
- Department of Paediatric Orthopaedics and Trauma Surgery, Sheffield Children's NHS Foundation Trust, Sheffield, South Yorkshire, United Kingdom
| | - Samuel Weston-Simmons
- Department of Paediatric Orthopaedics and Trauma Surgery, Sheffield Children's NHS Foundation Trust, Sheffield, South Yorkshire, United Kingdom
| | - Stephen N Giles
- Department of Paediatric Orthopaedics and Trauma Surgery, Sheffield Children's NHS Foundation Trust, Sheffield, South Yorkshire, United Kingdom
| | - James A Fernandes
- Department of Paediatric Orthopaedics and Trauma Surgery, Sheffield Children's NHS Foundation Trust, Sheffield, South Yorkshire, United Kingdom
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10
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Dheenadhayalan J, Avinash M, Lakhani A, Rajasekaran S. Shepherd's crook deformity: How to set it straight. A five-step surgical guide. J Orthop Surg (Hong Kong) 2020; 27:2309499019834362. [PMID: 30852946 DOI: 10.1177/2309499019834362] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/27/2022] Open
Abstract
PURPOSE Fibrous dysplasia (FD) of the proximal femur can result in severe deformity and disability. The results of surgical management in such situations have been reported to be poor. We present a novel, five-step surgical treatment to correct proximal femoral deformity in FD. MATERIAL AND METHODS This study is a review of prospectively collected data of cases of proximal femur polyostotic FD managed at our institute from 2012 onward. We managed three patients with FD involving four femora (one patient had bilateral disease). Mean age at presentation was 16 years with an average follow-up of 46 months. All underwent five steps, that is, (1) curettage/excision of the lesion, (2) allograft in intramedullary region, (3) lateral closing wedge valgus osteotomy, (4) fixation with extramedullary implant, and (5) augmentation of osteotomy site with autograft. Functional outcome was assessed using Harris Hip Score preoperatively and at the final follow-up. RESULTS The neck shaft angle was corrected from an average of 91.7° to 152.1°, while the Harris Hip Score improved from an average of 59 to 95. There was no clinical or radiological evidence of recurrence of disease or deformity in any patient till the last follow-up. CONCLUSION This five-step technique ensures good functional and radiological outcomes in the management of proximal femur FD.
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Affiliation(s)
| | - M Avinash
- Department of Orthopaedics and Traumatology, Ganga Hospital, Coimbatore, Tamil Nadu, India
| | - Azhar Lakhani
- Department of Orthopaedics and Traumatology, Ganga Hospital, Coimbatore, Tamil Nadu, India
| | - S Rajasekaran
- Department of Orthopaedics and Traumatology, Ganga Hospital, Coimbatore, Tamil Nadu, India
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11
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A Novel Technique for Single-stage Correction of Severe Shepherd Crook Deformity of a Femur With the Large Cystic Lesion and Pathologic Fracture. Tech Orthop 2019. [DOI: 10.1097/bto.0000000000000401] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
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12
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Zhang P, Kang L, Hu Q, Xia C, Yu H, Wang L, Lian K, Lin D. Treatment of diaphyseal pathological fractures in children with monostotic fibrous dysplasia using cortical strut allografts and internal plating: A retrospective clinical study. Medicine (Baltimore) 2019; 98:e14318. [PMID: 30702610 PMCID: PMC6380873 DOI: 10.1097/md.0000000000014318] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Children with fibrous dysplasia (FD) chronically suffer from pain, pathological fractures, and limb deformities. The most effective methods for managing the associated pathological fractures remain controversial. The purpose of this study was to evaluate the clinical results of the treatment of diaphyseal pathological fractures in children with monostotic fibrous dysplasia (MFD) using cortical strut allografts and internal plating.We retrospectively analyzed outcomes in nine children (5 boys, 4 girls) with diaphyseal pathological fractures due to MFD, who were treated with cortical strut allografts and internal plating (6 femoral fractures and 3 humeral fractures) between July 2007 and November 2012. The median age of patients in our study was 10 years (range 6-14 years). The fracture healing time, pain, extremity function, refracture, graft resorption, and complications were recorded to evaluate treatment effects.The median time of follow-up was 69 months (range 60-75 months). All patients had good postoperative fracture healing with a median healing time of 14 weeks (range 12-16 weeks). None experienced refracture, graft resorption, nerve injury, or limitation of extremity function or other complications. The fixation remained stable in all patients, with no evidence of loosening screws after surgery.In pediatric patients, the described surgical approach is an effective and reliable treatment method for diaphyseal pathological fractures caused by MFD. Cortical strut allografts, which act as biological bone plates, can provide good mechanical support while increasing the rate of fracture union.
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Affiliation(s)
- Peng Zhang
- The First Clinical College of Zhejiang Chinese Medical University, Hangzhou, Zhejiang
| | - Liangqi Kang
- Department of Orthopaedic Surgery, The Affiliated Southeast Hospital of Xiamen University, Zhangzhou
| | - Qimiao Hu
- Department of Neurobiology and Acupuncture Research, The Third Clinical Medical College, Zhejiang Chinese Medical University, Hangzhou, Zhejiang, China
| | - Chenjie Xia
- The First Clinical College of Zhejiang Chinese Medical University, Hangzhou, Zhejiang
| | - Huan Yu
- The First Clinical College of Zhejiang Chinese Medical University, Hangzhou, Zhejiang
| | - Lei Wang
- Department of Orthopaedic Surgery, The Affiliated Southeast Hospital of Xiamen University, Zhangzhou
| | - Kejian Lian
- Department of Orthopaedic Surgery, The Affiliated Southeast Hospital of Xiamen University, Zhangzhou
| | - Dasheng Lin
- Department of Orthopaedic Surgery, The Affiliated Southeast Hospital of Xiamen University, Zhangzhou
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13
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Luo S, Jiang T, Yang X, Yang Y, Zhao J. Treatment of tumor-like lesions in the femoral neck using free nonvascularized fibular autografts in pediatric patients before epiphyseal closure. J Int Med Res 2019; 47:823-835. [PMID: 30556444 PMCID: PMC6381492 DOI: 10.1177/0300060518813510] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/12/2018] [Accepted: 10/24/2018] [Indexed: 01/18/2023] Open
Abstract
OBJECTIVES Surgical resection of benign bone tumors and tumor-like lesions at the femoral neck presents a difficult reconstructive challenge. However, the safety and efficacy of free nonvascularized fibular autografts (FNFAs) in the treatment of femoral neck tumor-like lesions before epiphyseal closure in young patients remain unknown. METHODS Sixteen pediatric patients who had not yet undergone epiphyseal closure were treated with FNFAs after resection of tumor-like lesions in the femoral neck from August 2012 to September 2016. All patients underwent supplementary skeletal traction through the supracondylar femur for 4 to 6 weeks after resection. Demographic data were recorded and clinical and radiological outcomes were evaluated during the follow-up. RESULTS All patients could walk with partial weight bearing 4 weeks postoperative, and full weight bearing was permitted after a mean of 8 weeks. Graft union was attained in all 16 patients at a mean of 2 months. The donor site of the fibular cortical strut showed good regeneration in all patients. The Harris hip score significantly improved from 65% to 95%. CONCLUSIONS Application of an FNFA is a feasible method in the treatment of tumor-like lesions in the femoral neck before epiphyseal closure in pediatric patients. LEVEL OF EVIDENCE Level IV.
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Affiliation(s)
- Shanchao Luo
- Yulin Orthopedics Hospital of Chinese and Western Medicine,
Yulin, China
- Postdoctoral Innovation Practice Base, The Ninth Affiliated
Hospital of Guangxi Medical University, Beihai, China
- Guangxi Engineering Center in Biomedical Materials for Tissue
and Organ Regeneration & Guangxi Collaborative Innovation Center for
Biomedicine, Life Sciences Institute, Guangxi Medical University, Nanning,
China
- *These authors contributed equally to this
work
| | - Tongmeng Jiang
- Guangxi Engineering Center in Biomedical Materials for Tissue
and Organ Regeneration & Guangxi Collaborative Innovation Center for
Biomedicine, Life Sciences Institute, Guangxi Medical University, Nanning,
China
- Department of Orthopaedics & Guangxi Key Laboratory of
Regenerative Medicine, International Joint Laboratory on Regeneration of Bone
and Soft Tissue, The First Affiliated Hospital of Guangxi Medical University,
Nanning, China
- School of Materials Science and Engineering, Zhejiang
University, Hangzhou, China
- *These authors contributed equally to this
work
| | - Xiaoping Yang
- Yulin Orthopedics Hospital of Chinese and Western Medicine,
Yulin, China
| | - Yingnian Yang
- Yulin Orthopedics Hospital of Chinese and Western Medicine,
Yulin, China
| | - Jinmin Zhao
- Postdoctoral Innovation Practice Base, The Ninth Affiliated
Hospital of Guangxi Medical University, Beihai, China
- Guangxi Engineering Center in Biomedical Materials for Tissue
and Organ Regeneration & Guangxi Collaborative Innovation Center for
Biomedicine, Life Sciences Institute, Guangxi Medical University, Nanning,
China
- Department of Orthopaedics & Guangxi Key Laboratory of
Regenerative Medicine, International Joint Laboratory on Regeneration of Bone
and Soft Tissue, The First Affiliated Hospital of Guangxi Medical University,
Nanning, China
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Liu YB, Zou TM. Giant monostotic osteofibrous dysplasia of the ilium: A case report and review of literature. World J Clin Cases 2018; 6:830-835. [PMID: 30510951 PMCID: PMC6264999 DOI: 10.12998/wjcc.v6.i14.830] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/03/2018] [Revised: 10/30/2018] [Accepted: 11/01/2018] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Osteofibrous dysplasia (OFD) is a developmental skeletal disorder, and cases with a giant affected area in the pelvis are rare.
CASE SUMMARY In this case report, a 48-year-old man presented with a large tumor in the right iliac region that turned out to be OFD. The patient had rebound tenderness in his right hip. After radiography examination, magnetic resonance imaging examinations and some physical examination, extensive bone destruction in the right ilium was confirmed. Moreover, changes in bone mineral density and peripheral cortical bone sclerosis with surrounding soft tissue swelling were observed. Thus, this patient was considered to have giant monostotic OFD of the ilium. The tumor-related area was removed completely by surgery, and the remaining cavity was filled by artificial bones from the opposite ilium. According to the results of follow-up, the patient had normal flexion and extension activities of the right hip joint, and there was no evidence of recurrence of the tumor.
CONCLUSION Suture of iliopsoas and gluteus medius muscle following focus curettage and bone grafting is a promising and effective method to treat giant OFD of the ilium. It is a feasible way to fill a large cavity after removing a lesion like the one is this case.
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Affiliation(s)
- Yu-Bo Liu
- Department of Orthopedics, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215002, Jiangsu Province, China
| | - Tian-Ming Zou
- Department of Orthopedics, the Affiliated Suzhou Hospital of Nanjing Medical University, Suzhou 215002, Jiangsu Province, China
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15
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Zekry KM, Yamamoto N, Hayashi K, Takeuchi A, Alkhooly AZAA, Abd-Elfattah AS, Fouly EH, Ahmed AR, Tsuchiya H. Treatment of the benign lytic lesions of the proximal femur with synthetic bone graft. J Orthop Surg Res 2018; 13:270. [PMID: 30373674 PMCID: PMC6206894 DOI: 10.1186/s13018-018-0982-z] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/24/2017] [Accepted: 10/19/2018] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND Benign bone tumors and tumor-like conditions are commonly located in the proximal femur. The main indications for surgical treatment are lesions with impending or actual pathological fractures, or with aggressive or recurrent lesions. However, patients complaining of persistent pain, limping, or abnormal gait patterns are also considered for surgical treatment. In this study, we describe the outcomes of the surgical treatment of benign lytic lesions of the proximal femur by curettage followed by implantation of synthetic bone graft. METHODS This retrospective study included 27 patients (22 females and 5 males) with benign lytic lesions of the proximal femur. The average age was 25.5 years (6-65 years), and the mean follow-up period was 54.5 months (9-145 months). The histopathological diagnoses were fibrous dysplasia (8 patients), simple bone cyst (8 patients), chondroblastoma (7 patients), giant cell tumor (3 patients), and eosinophilic granuloma (1 patient). These lesions were managed with curettage followed by implantation of the bone defects with alpha tricalcium phosphate in 14 patients, beta tricalcium phosphate granules in 11 patients, hydroxyapatite granules in 1 patient, and combined beta tricalcium phosphate and hydroxyapatite granules in 1 patient. Internal fixation was performed in three patients. RESULTS The mean operative time was 143 min (80-245 min). Patients had regained normal unrestricted activity without pain at the operation site. Patients treated with beta tricalcium phosphate achieved radiographic consolidation of the bone defects within 1 year after the surgery, and those treated with alpha tricalcium phosphate or hydroxyapatite experienced no progression nor recurrence of the lesions. Local tumor recurrence was observed in one patient with giant cell tumor 5 years after the surgery. Post-operative pathological fracture was occurred in one patient with a simple bone cyst of the subtrochanteric region 1 month after surgery. No post-operative infection was observed. CONCLUSION We concluded that the treatment of benign lytic lesions of the proximal femur, either primary or recurrent, using synthetic bone graft is a safe and satisfactory method and the addition of internal fixation should be carefully planned.
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Affiliation(s)
- Karem M Zekry
- Department of Orthopaedic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan.,Department of Orthopaedic Surgery, Faculty of Medicine, Minia University, El-Minya, Egypt
| | - Norio Yamamoto
- Department of Orthopaedic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan.
| | - Katsuhiro Hayashi
- Department of Orthopaedic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
| | - Akihiko Takeuchi
- Department of Orthopaedic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
| | - Ali Zein A A Alkhooly
- Department of Orthopaedic Surgery, Faculty of Medicine, Minia University, El-Minya, Egypt
| | | | - Ezzat H Fouly
- Department of Orthopaedic Surgery, Faculty of Medicine, Minia University, El-Minya, Egypt
| | - Adel Refaat Ahmed
- Department of Orthopaedic Surgery, Faculty of Medicine, Alexandria University, Alexandria, Egypt
| | - Hiroyuki Tsuchiya
- Department of Orthopaedic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan
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Management of Fibrous Dysplasia of Proximal Femur by Internal Fixation Without Grafting: A Retrospective Study of 19 Patients. JOURNAL OF THE AMERICAN ACADEMY OF ORTHOPAEDIC SURGEONS GLOBAL RESEARCH AND REVIEWS 2018; 2:e057. [PMID: 30656248 PMCID: PMC6324889 DOI: 10.5435/jaaosglobal-d-18-00057] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/11/2022]
Abstract
Introduction: This paper assesses whether treating patients with fibrous dysplasia of proximal femur by internal fixation with correction of the deformity, if present, without grafting is enough or not. Methods: This study is a retrospective study using patient's medical records, including analysis of 19 patients with fibrous dysplasia of proximal femur treated by internal fixation only and followed up between 2000 and 2017 for at least 2 years. Epidemiologic data, clinical manifestations, radiologic and histologic investigations, surgery, functional outcomes, and complications in these patients were analyzed. Results: The study included 12 male patients and 7 female patients, with a mean age of 20.74 years. Of these, two patients presented with pathologic fractures, two presented with shepherd's crook deformity, and the remaining presented with hip pain and limping. Implants used for internal fixation were intramedullary nail in four patients, dynamic hip screw in eight patients, cannulated screws in four patients, broad dynamic compression plate in two patients, and narrow dynamic compression plate in one patient. The mean follow-up period was 53.58 months. Four patients had postoperative complications. The mean Musculoskeletal Tumor Society score was 27.63 points. Discussion: The optimal management option for fibrous dysplasia of proximal femur is debatable. We suggest that internal fixation without grafting has a good local control and satisfactory functional long-term outcome.
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17
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Yung D, Kikuta K, Sekita T, Asano N, Nakayama R, Nakamura M, Matsumoto M. Conservative surgical management of simple monostotic fibrous dysplasia of the proximal femur in a 19-year-old basketballer: a case report. J Med Case Rep 2018; 12:240. [PMID: 30165901 PMCID: PMC6117941 DOI: 10.1186/s13256-018-1763-3] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/18/2017] [Accepted: 07/07/2018] [Indexed: 12/02/2022] Open
Abstract
Background Fibrous dysplasia is a rare benign, intramedullary, fibro-osseous lesion. It is thought to be a developmental disorder of bone maturation where normal lamellar bone is replaced by irregular trabecular bone ensnared with fibrous dysplastic tissue that is unable to complete maturation resulting in significant loss of mechanical strength. This, together with the inability to mineralize sufficiently, leads to deformity, pain, and pathological fractures. It typically presents in young adults, with an equal representation in both genders. Surgical intervention is necessary in mild cases with chronic symptoms to prevent pathological fractures or to correct deformities. Case presentation A 19-year-old Chinese woman presented with non-traumatic, nonspecific left hip pain during basketball training. X-rays demonstrated a ground glass lesion, 10 cm in length, in her left femoral neck, which is a classic sign of fibrous dysplasia. No other deformities were noted. She was managed conservatively with analgesia for 6 months; however, her condition did not improve and a decision was made for surgical intervention. The lesion was a type 1 lesion according to the Ippolito radiological classification of fibrous dysplasia, which is a lesion with mild deformities. Therefore, we performed minimal curettage and insertion of a free autologous fibula strut harvested from her left leg, for structural stability. No implants were used. The operation was successful and her postoperative course was uneventful. Histology confirmed the diagnosis of fibrous dysplasia. She achieved partial weight bearing at 4 weeks postoperation, and full weight bearing at 8 weeks, and returned to basketball at 12 weeks. At 1-year follow-up, she returned to competitive basketball and remained pain free with no complications. Conclusions Fibrous dysplasia is a rare and benign fibrous tumor of the bone that presents mostly in a young patient population. From our case, we have shown that it is possible to treat young patients with uncomplicated Ippolito type 1 fibrous dysplasia with a minimally invasive approach of using a cortical bone graft for structural augmentation of the affected area, without the use of implants. They are able to fully return to an active and vigorous lifestyle without restriction of activities or long-term risks of orthopedic implant complications.
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Affiliation(s)
- David Yung
- Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo, 160-8582, Japan.,Royal College of Surgeons in Ireland, 123 St Stephen's Green, Dublin 2, Ireland
| | - Kazutaka Kikuta
- Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo, 160-8582, Japan.
| | - Tetsuya Sekita
- Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo, 160-8582, Japan
| | - Naofumi Asano
- Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo, 160-8582, Japan
| | - Robert Nakayama
- Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo, 160-8582, Japan
| | - Masaya Nakamura
- Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo, 160-8582, Japan
| | - Morio Matsumoto
- Department of Orthopaedic Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjuku-ku, Tokyo, 160-8582, Japan
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18
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Fang X, Liu H, Lang Y, Xiong Y, Duan H. Fibrous dysplasia of bone: Surgical management options and outcomes of 22 cases. Mol Clin Oncol 2018; 9:98-103. [PMID: 29977545 DOI: 10.3892/mco.2018.1636] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/02/2018] [Accepted: 05/15/2018] [Indexed: 02/07/2023] Open
Abstract
The surgical treatment for fibrous dysplasia (FD) of bone is problematic due to its variable clinical courses. And multifarious surgical treatment options have been reported while no consistent view can be reached. Therefore, we reviewed a series of 22 patients (11 males and 11 females; mean age 28.4 years, range 15-48 years) with FD between December 2011 and July 2015. Fourteen patients had monostotic fibrous dysplasia (MFD) and eight patients had polyostotic fibrous dysplasia (PFD) with nine lesions. All patients were followed up from 15 to 58 months with an average of 26.0 months. Functional and radiographic outcomes were recorded. In the MFD group, four patients were treated with curettage and bone grafting without internal fixation and nine were treated with curettage and bone grafting with internal fixations. Osteotomy and intramedullary (IM) nail was applied in one patient with serious deformity. In the PFD group, three deformity lesions were treated with osteotomy and proximal femoral nail anti-rotation (PFNA). IM were also applied in six large lesions to treat fracture or prevent deformity. One lesion in tibia were treated with only curettage and bone graft. No complication was observed in MFD group and satisfactory union and functional outcomes acquired during follow-up period. In the PFD group, the spiral blade cutting out from femoral head in PFNA was observed in one patient and treated with revision surgery. No other complication occurred, and satisfactory radiological and functional outcome were observed. The severity of both MFD and PFD are related to size, site and of the lesion. The goal of the surgery is to eliminate pain, correct deformity and treat pathological fracture. Curettage, bone grafting with internal fixation is recommended for treating large lesions with deformity or high pathological fracture risk. PFNA or IM nail is prior in osteotomy with better clinical outcome.
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Affiliation(s)
- Xiang Fang
- Department of Orthopedics, West China School of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan 610064, P.R. China
| | - Hongyuan Liu
- Department of Orthopedics, West China School of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan 610064, P.R. China
| | - Yun Lang
- Department of Orthopedics, West China School of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan 610064, P.R. China.,Department of Orthopedics, People's Hospital of Deyang City, Deyang, Sichuan 618000, P.R. China
| | - Yan Xiong
- Department of Orthopedics, West China School of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan 610064, P.R. China
| | - Hong Duan
- Department of Orthopedics, West China School of Medicine, West China Hospital, Sichuan University, Chengdu, Sichuan 610064, P.R. China
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Individualized approach to the surgical management of fibrous dysplasia of the proximal femur. Orphanet J Rare Dis 2018; 13:72. [PMID: 29720212 PMCID: PMC5932767 DOI: 10.1186/s13023-018-0805-7] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/09/2017] [Accepted: 04/10/2018] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND Fibrous dysplasia of the proximal femur presents with heterogeneous clinical manifestations dictating different surgical approaches. However, to date there are no clear recommendations to guide the choice of surgical approach and no general guidelines for the optimal orthopedic management of these lesions. The objective of this study was to evaluate treatment outcomes of angled blade plates and intramedullary nails, using as outcome indicators revision-free survival, pain, function and femoral neck-shaft-angle. Based on a review of published literature and our study findings, we propose a treatment algorithm, taking into account different factors, which may play a role in the selection of one surgical approach over another. METHODS Data were evaluated in thirty-two patients (18 male) from a combined cohort from the Netherlands and Austria, who had a surgical intervention using an angled blade plate (n = 27) or an intramedullary nail (n = 5) between 1985 and 2015, and who had a minimal follow-up of one year. The primary outcome was success of the procedure according to the revised Henderson classification. Secondary outcomes, which were assessed at one year and at the end of follow-up included: function (as measured by walking ability), pain and change in femoral neck-shaft angle over time. RESULTS Analysis of data showed that revision-free survival was 72% after a median follow-up of 4.1 years. Revision was necessary in two patients for structural failure due to a fracture distal to an angled blade plate and in 7 patients due to angled blade plate-induced iliotibial tract pain. At the end of follow-up 91% of all patients had good walking ability and 91% were pain free. There was no significant postoperative change in femoral neck shaft angle. CONCLUSION Our data show that fibrous dysplasia of the proximal femur can be adequately and safely treated with angled blade plates or intramedullary nails, providing these are used according to specific characteristics of the individual patient. Based on published literature and our own experience, we propose an individualized, patient-tailored approach for the surgical management of fibrous dysplasia of the proximal femur.
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20
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Rosario MS, Hayashi K, Yamamoto N, Takeuchi A, Miwa S, Taniguchi Y, Tsuchiya H. Functional and radiological outcomes of a minimally invasive surgical approach to monostotic fibrous dysplasia. World J Surg Oncol 2017; 15:1. [PMID: 28057011 PMCID: PMC5217401 DOI: 10.1186/s12957-016-1068-1] [Citation(s) in RCA: 21] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2016] [Accepted: 12/14/2016] [Indexed: 11/13/2022] Open
Abstract
Background Reports showing high recurrence rates for intralesional curettage and bone grafting have made the current treatment principle for fibrous dysplasia controversial. This study aimed to report the postoperative clinical outcomes from three minimally invasive surgical strategies we use for monostotic fibrous dysplasia (MFD). Patients and methods Twelve patients with MFD presenting with no pathologic fracture or deformity and treated with one of three surgical strategies—plain open biopsy, plain alpha-tricalcium phosphate (ATP) reconstruction, and prophylactic bridge plating—were included. There were nine men and three women, with median age of 38 years. Mean follow-up was 88 weeks. Five cases involved the proximal femur, two each involved the femoral and tibial diaphyses, and one each involved the distal humerus, radial diaphysis, and proximal tibia. All cases were reviewed for functional and radiological outcomes. Results Median time to full activity was 1 day (range 1 to 3) for the plain open biopsy group, while the prophylactic bridge-plating and plain ATP reconstruction groups had longer median recovery times (59 days, range 3 to 143, and 52 days, range 11 to 192, respectively). Musculoskeletal Tumor Society scores at last follow-up were excellent for all the cases (mean 29.6, range 25 to 30). Radiological analysis using Gaski et al.’s criteria showed plain open biopsy resulted in partial resolution of proximal femoral lesions, while ATP reconstruction and prophylactic plating resulted in no change and progression in this lesion site, respectively. For femoral diaphyseal lesions, prophylactic plating resulted in partial resolution, while ATP reconstruction resulted in no change. In the tibial diaphysis, prophylactic plating resulted in partial resolution, while plain open biopsy resulted in no change. For the lesions involving the distal humerus and the proximal tibia, plain open biopsy resulted in partial resolution, while for the radial diaphyseal lesion, ATP reconstruction resulted in no change. Radiological progression was limited in 11 (92%) cases, and none had postoperative complications. Conclusion Plain open biopsies for asymptomatic lesions; prophylactic bridge plating for symptomatic, large diaphyseal lytic lesions; and plain ATP reconstructions for both small and large nondiaphyseal symptomatic lytic lesions may be acceptable alternatives to curettage-incorporating procedures for MFD.
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Affiliation(s)
- Mamer S Rosario
- Department of Orthopaedic Surgery, Kanazawa University School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8640, Japan.,Department of Orthopaedics, East Avenue Medical Center, East Avenue, Diliman, Quezon City, 1101, Metro Manila, Philippines
| | - Katsuhiro Hayashi
- Department of Orthopaedic Surgery, Kanazawa University School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8640, Japan.
| | - Norio Yamamoto
- Department of Orthopaedic Surgery, Kanazawa University School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8640, Japan
| | - Akihiko Takeuchi
- Department of Orthopaedic Surgery, Kanazawa University School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8640, Japan
| | - Shinji Miwa
- Department of Orthopaedic Surgery, Kanazawa University School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8640, Japan
| | - Yuta Taniguchi
- Department of Orthopaedic Surgery, Kanazawa University School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8640, Japan
| | - Hiroyuki Tsuchiya
- Department of Orthopaedic Surgery, Kanazawa University School of Medicine, 13-1 Takara-machi, Kanazawa, Ishikawa, 920-8640, Japan
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21
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Leet AI, Boyce AM, Ibrahim KA, Wientroub S, Kushner H, Collins MT. Bone-Grafting in Polyostotic Fibrous Dysplasia. J Bone Joint Surg Am 2016; 98:211-9. [PMID: 26842411 PMCID: PMC4732545 DOI: 10.2106/jbjs.o.00547] [Citation(s) in RCA: 40] [Impact Index Per Article: 4.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
BACKGROUND Polyostotic fibrous dysplasia is a skeletal disease that results from somatic activating mutations in the gene GNAS in skeletal stem cells, leading to proliferation of immature osteogenic cells with replacement of normal marrow and bone with fibro-osseous tissue. Lesions may cause bone deformity or fracture. In the surgical care of polyostotic fibrous dysplasia, the role of grafting and the optimal grafting material are not clear. The purpose of this study was to evaluate the long-term survival of bone-grafting procedures in subjects with polyostotic fibrous dysplasia over time. METHODS The operative reports and radiographs of a cohort of subjects with polyostotic fibrous dysplasia followed in a natural history study were reviewed. Twenty-three subjects (mean age at the time of enrollment, thirteen years [range, two to forty years]) with fifty-two bone-grafting procedures had a mean follow-up time of 19.6 years (range, twenty-nine months to forty-seven years). Kaplan-Meier life table estimates, Cox proportional hazard models, and t tests comparing means were performed to assess various aspects of graft survival. RESULTS Kaplan-Meier curves showed a 50% estimate of survival of 14.5 years. Cox proportional hazards models showed no advantage comparing allograft with autograft or structural with nonstructural graft materials. The mean age of the patients was significantly greater (p < 0.001) in the subgroup of subjects in whom grafts were maintained over time (20.9 years) compared with the subgroup of patients whose grafts were resorbed over time (9.8 years). CONCLUSIONS Bone-grafting, including both allograft and autograft, is of limited value in ablating the lesions of fibrous dysplasia. The expectations of patients and surgeons should include the high probability of graft resorption over time with return of bone characteristics of fibrous dysplasia, particularly in younger patients. This suggests the maintenance of normal bone mechanics with implant support should be the priority of any surgical intervention.
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Affiliation(s)
| | - Alison M. Boyce
- Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland,Division of Endocrinology and Diabetes, Children’s National Health System, Washington, D.C.,Bone Health Program, Division of Orthopaedics and Sports Medicine, Children’s National Health System, Washington, D.C
| | - Khalda A. Ibrahim
- Department of Orthopedics, Johns Hopkins University, Baltimore, Maryland
| | - Shlomo Wientroub
- Department of Pediatric Orthopedics, Dana Children’s Hospital, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Harvey Kushner
- Biomedical Computer Research Institute, Philadelphia, Pennsylvania
| | - Michael T. Collins
- Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Bethesda, Maryland,E-mail address for M.T. Collins:
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Carvallo PI, Griffin AM, Ferguson PC, Wunder JS. Salvage of the proximal femur following pathological fractures involving benign bone tumors. J Surg Oncol 2015; 112:846-52. [PMID: 26482583 DOI: 10.1002/jso.24072] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2015] [Accepted: 10/08/2015] [Indexed: 12/15/2022]
Abstract
BACKGROUND AND OBJECTIVES To describe the surgical treatment of patients with a pathologic fracture through a benign tumor of the proximal femur to determine if there is a difference in local recurrence, complications or functional outcome compared to patients with tumors in the same location without pathologic fractures. METHODS From 1989-2010, of 97 patients, 29 presented with a pathologic fracture (PF) through a proximal femoral benign bone tumor and 68 presented without a pathologic fracture (NPF). Outcomes of the two groups were compared in terms of surgical management, postoperative complications, local recurrence and functional scores. RESULTS Fibrous dysplasia, giant cell tumor of bone and chondroblastoma were the most common subtypes. Most patients were managed with joint preservation in both PF (86.2%) and NPF (98.5%) groups (P = 0.03). Local recurrence risk was similar for patients in the PF (10.3%) and NPF (8.8%) groups. Mean follow-up was 105.7 months (P = 0.8). Functional outcome scores were high in both groups and not statistically significantly different. CONCLUSIONS The majority of pathologic fractures through a benign bone tumor of the proximal femur can be successfully treated with curettage, burring, bone grafting and internal fixation without increasing the risk of local recurrence or negatively impacting functional outcome.
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Affiliation(s)
- Pedro I Carvallo
- University Post-Graduate Orthopaedic Oncology Program, Universidad Central de Venezuela, Servicios Oncológicos Hospitalarios, Seguro Social, Caracas, Venezuela
| | - Anthony M Griffin
- University Musculoskeletal Oncology Unit, Mount Sinai Hospital, Toronto, Ontario, Canada
| | - Peter C Ferguson
- University Musculoskeletal Oncology Unit, Mount Sinai Hospital, Toronto, Ontario, Canada.,Division of Orthopedic Surgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada
| | - Jay S Wunder
- University Musculoskeletal Oncology Unit, Mount Sinai Hospital, Toronto, Ontario, Canada.,Division of Orthopedic Surgery, Department of Surgery, University of Toronto, Toronto, Ontario, Canada
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Nakamura T, Matsumine A, Asanuma K, Matsubara T, Sudo A. Treatment of the benign bone tumors including femoral neck lesion using compression hip screw and synthetic bone graft. SICOT J 2015; 1:15. [PMID: 27163071 PMCID: PMC4849251 DOI: 10.1051/sicotj/2015009] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/15/2022] Open
Abstract
PURPOSE The proximal femur is one of the most common locations for benign bone tumors and tumor like conditions. We describe the clinical outcomes of the surgical treatment of benign lesions of the proximal femur including femoral neck using compression hip screw and synthetic bone graft. METHODS Thirteen patients with benign bone tumors or tumor like conditions of the proximal femur including femoral neck were surgically treated. Their average age at the time of presentation was 35 years and the average follow-up time was 76 months. RESULTS The average intraoperative blood loss was 1088 mL and intraoperative blood transfusion was required in eight patients. The average operative time was 167 minutes. All patients required one week and 12 weeks after surgery before full weight-bearing was allowed. All patients had regained full physical function without pain by the final follow-up. No patient sustained a pathological fracture of the femur following the procedure. All patients achieved partial or complete radiographic consolidation of the lesion within one year except one patient who developed a local tumor recurrence in 11 months. Post-operative superficial wound infection was observed in one patient, which resolved with intravenous antibiotics. Chronic hip pain was observed in one patient due to the irritation of tensor fascia lata muscle by the tube plate. CONCLUSION We suggest that the treatment of benign bone lesion of the proximal femur using compression hip screw and synthetic bone graft is a safe and effective method.
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Affiliation(s)
- Tomoki Nakamura
- Department of Orthopaedic Surgery, Mie University Graduate School of Medicine Tsu Japan
| | - Akihiko Matsumine
- Department of Orthopaedic Surgery, Mie University Graduate School of Medicine Tsu Japan
| | - Kunihiro Asanuma
- Department of Orthopaedic Surgery, Mie University Graduate School of Medicine Tsu Japan
| | - Takao Matsubara
- Department of Orthopaedic Surgery, Mie University Graduate School of Medicine Tsu Japan
| | - Akihiro Sudo
- Department of Orthopaedic Surgery, Mie University Graduate School of Medicine Tsu Japan
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Nishida Y, Tsukushi S, Hosono K, Nakashima H, Yamada Y, Urakawa H, Ishiguro N. Surgical treatment for fibrous dysplasia of femoral neck with mild but prolonged symptoms: a case series. J Orthop Surg Res 2015; 10:63. [PMID: 25958111 PMCID: PMC4429415 DOI: 10.1186/s13018-015-0208-6] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/04/2015] [Accepted: 03/14/2015] [Indexed: 11/11/2022] Open
Abstract
Background The proximal femur is one of the most common sites involved by fibrous dysplasia. In cases with mild deformity that does not require corrective surgery, occasional patients suffer sustained pain because of repeated microfractures. This study aimed to clarify the outcomes of surgery with autogenous fibular cortical strut grafting and compression hip screw fixation. Methods Since 2002, eight consecutive patients (nine hips) with femoral neck fibrous dysplasia without severe deformity were prospectively treated with autogenous fibular strut grafting and compression hip screw fixation. Results Mean age of patients was 35 years. Mean follow-up of patients after surgery was 75 months. Most of the patients could walk with full weight-bearing 2 weeks after surgery. Functional score of lower extremity was significantly improved from 65 % to 95 % (P = 0.001). Femoral neck angle was increased from 127 to 130. Donor site of strut cortical fibula showed good regeneration with β-tricalcium phosphate. Conclusions Autogenous fibular cortical strut grafting and compression hip screw fixation achieved good post-operative function and provided an early return to work for adult patients with fibrous dysplasia of the femoral neck with mild but prolonged symptoms. Morbidity in the donor site of fibula strut is minimal with the use of β-tricalcium phosphate.
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Affiliation(s)
- Yoshihiro Nishida
- Department of Orthopaedic Surgery, Nagoya University Graduate School and School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan.
| | - Satoshi Tsukushi
- Department of Orthopaedic Surgery, Nagoya University Graduate School and School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan.
| | - Kozo Hosono
- Orthopaedic Surgery, Aichi Cancer Center Aichi Hospital, 18 Kuriyado, Kake-machi, Okazaki City, Aichi, Japan.
| | - Hiroatsu Nakashima
- Orthopaedic Surgery, Aichi Cancer Center Aichi Hospital, 18 Kuriyado, Kake-machi, Okazaki City, Aichi, Japan. .,Present address: Orthopaedic Surgery, Gifu Prefectural Tajimi Hospital, 5-161, Maehata, Tajimi, Gifu, Japan.
| | - Yoshihisa Yamada
- Orthopaedic Surgery, Nagoya Memorial Hospital, 4-305 Hirabari, Tenpaku-ku, Nagoya, Japan.
| | - Hiroshi Urakawa
- Department of Orthopaedic Surgery, Nagoya University Graduate School and School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan.
| | - Naoki Ishiguro
- Department of Orthopaedic Surgery, Nagoya University Graduate School and School of Medicine, 65 Tsurumai, Showa, Nagoya, Aichi, 466-8550, Japan.
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Hagiwara Y, Iwata S, Yonemoto T, Ishii T. Rotational valgus osteotomy for shepherd's crook deformity: a case report. J Orthop Sci 2015; 20:422-4. [PMID: 24005584 DOI: 10.1007/s00776-013-0463-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/20/2013] [Accepted: 08/22/2013] [Indexed: 02/09/2023]
Affiliation(s)
- Yoko Hagiwara
- Department of Orthopaedics, Tokyo Women's Medical University, Tokyo, Japan
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Interim Clinical Outcomes in Nanocomposite Bone Material Repairing Large Proximal Femoral Defect of Fibrous Dysplasia. JOURNAL OF NANOMATERIALS 2015. [DOI: 10.1155/2015/385612] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Background and Objectives. To evaluate the clinical effectiveness and safety of using nanocomposite bone material in the repair of large proximal femoral defects that are due to fibrous dysplasia.Method. Thirty-one patients were analyzed retrospectively, including 13 males and 18 females, and the mean age was 30.9 years (13–59). The median follow-up period was 50 months (30–78) and the masses of artificial bone transplants were in the range of 15~40 g (average of 23.4 g). Functional and radiographic outcomes were evaluated.Results. All wounds healed to grade A. There were no infections, nonspecific inflammatory reactions, rejection reactions, or fractures. One case had fat liquefaction and healed after dressing. All patients had no recurrence until the last follow-up. At the last follow-up, the mean Musculoskeletal Tumor Society’s (MSTS) 93 score was 28.42 ± 1.31, the mean Harris hip score was 84.23 ± 8.97, and mean radiopaque density ratio was 0.78 ± 0.09. Radiologic analysis indicated that nanocomposite bone material had been completely incorporated with the host bone within a year.Conclusions. This study indicated that the nanocomposite bone material had biological safety and good biocompatibility. In conclusion, the nanocomposite bone material is an ideal artificial bone substitute and worthy of promotion in the field of orthopedics.
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Radiographic classification of coronal plane femoral deformities in polyostotic fibrous dysplasia. Clin Orthop Relat Res 2014; 472:1558-67. [PMID: 24249535 PMCID: PMC3971252 DOI: 10.1007/s11999-013-3380-1] [Citation(s) in RCA: 43] [Impact Index Per Article: 3.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/04/2013] [Accepted: 11/05/2013] [Indexed: 01/31/2023]
Abstract
BACKGROUND Fibrous dysplasia of bone is a skeletal dysplasia with a propensity to affect the femur in its polyostotic form, leading to deformity, fracture, and pain. The proximal femur is most commonly involved with a tendency to distal progression, thereby producing the typical shepherd's crook deformity. However, there are few data on the spectrum and progression of femoral deformities in polyostotic fibrous dysplasia. QUESTIONS/PURPOSES The purposes of this study were (1) to develop a radiographic classification for polyostotic fibrous dysplasia; (2) to test this classification's intra- and interobserver reliability; and (3) to characterize the radiographic progression of polyostotic fibrous dysplasia in a population of patients with the condition who were treated with a variety of approaches at several centers. METHODS We retrospectively reviewed radiographs of 127 femurs from 84 adult patients affected by polyostotic fibrous dysplasia. Fifty-nine femurs had undergone one or more operations. The radiographs were evaluated in the coronal plane for neck-shaft angle and angular deformities along the whole femoral shaft down to the distal epiphysis. Four observers evaluated each film two times at intervals; intra- and interobserver reliability testing was performed using the kappa statistic. Eighty-nine femurs (70%) were available for followup to evaluate for progression at a mean of 10 years (range, 6-20 years). RESULTS Six reproducible patterns of deformity were identified in both untreated and operated femurs: type 1 (24%), normal neck-shaft angle with altered shape of the proximal femur; type 2 (6%), isolated coxa valga with neck-shaft angle > 140°; type 3 (7%), isolated coxa vara with neck-shaft angle < 120°; type 4 (20%), lateral bowing of the proximal half of the femur associated with normal neck-shaft angle; type 5 (14%), like in type 4 but associated with coxa valga; and type 6 (29%), like in type 4 but associated with coxa vara. Interobserver and intraoberver kappa values were excellent, ranging from 0.83 to 0.87. In 46 of the 89 femurs (52%) for which longitudinal radiographic documentation was available, there was progressive worsening of the original deformity, although the pattern remained the same; types 1 and 2 tended not to progress, whereas types 3 to 6 did. CONCLUSIONS A reproducible radiographic classification of polyostotic fibrous dysplasia-associated femoral deformities is proposed, which can serve as a tool for assessing and treating these deformities. After reviewing the radiographs of 127 femurs, we identified six reproducible patterns of femoral deformities. LEVEL OF EVIDENCE Level III, diagnostic study. See Guidelines for Authors for a complete description of levels of evidence.
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Abstract
Monostotic fibrous dysplasia of the proximal femur has a variable clinical course, despite its reported limited tendency to progress. We investigated the natural history and predisposing factors for progression of dysplasia in a group of 76 patients with a mean follow-up of 8.5 years (2.0 to 15.2). Of these, 31 (41%) presented with an asymptomatic incidental lesion while 45 (59%) presented with pain or a pathological fracture. A group of 23 patients (30%) underwent early operative treatment for pain (19: 25%) or pathological fracture (4: 5%). Of the 53 patients who were initially treated non-operatively, 45 (85%) remained asymptomatic but eight (15%) needed surgery because of pain or fracture. The progression-free survival of the observation group was 81% (sd 6.4%) at five-years follow-up. An initial presentation of pain (p < 0.001), a limp (p < 0.001), radiological evidence of microfracture (p = 0.001) and younger age (< 17 years) (p = 0.016) were significant predisposing factors for disease progression. The risk of experiencing pain or pathological fracture is considerable in monostotic fibrous dysplasia of the proximal femur. Patients presenting with pain, a limp or radiological evidence of microfracture have a high chance of needing surgical treatment. Cite this article: Bone Joint J 2014;96-B:673–6.
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Affiliation(s)
- I. Han
- Seoul National University Hospital, Department
of Orthopaedic Surgery, 101 Daehak-ro Jongno-gu, Seoul, 110-744, South
Korea
| | - E. S. Choi
- Seoul National University Hospital, Department
of Orthopaedic Surgery, 101 Daehak-ro Jongno-gu, Seoul, 110-744, South
Korea
| | - H-S. Kim
- Seoul National University Hospital, Department
of Orthopaedic Surgery, 101 Daehak-ro Jongno-gu, Seoul, 110-744, South
Korea
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Tank JC, Vignos MF, Wetzel RJ, Weiner SD. Allograft cortical strut for reconstruction of space-occupying bone lesions. Orthopedics 2014; 37:e66-70. [PMID: 24683659 DOI: 10.3928/01477447-20131219-19] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/03/2023]
Abstract
Space-occupying bone lesions present orthopedic surgeons with clinical and operative challenges. Multiple reconstructive procedures have proven successful for small bone lesions but lack the structural support necessary for reconstruction of larger lesions. This study reports the clinical outcomes of patients undergoing excision and reconstruction of large bone lesions with allograft cortical struts without additional internal fixation. This retrospective outcomes study reviewed patients who underwent surgical curettage and cortical strut allograft reconstruction of any space-occupying bone lesion. Clinical, surgical, and imaging data were collected. The primary outcome measures were lesion healing, graft incorporation, long-term pain, return to activity, and presence of complications/recurrences. Seventeen patients met the inclusion criteria. At least partial lesion healing and allograft incorporation was identified in 15 of 17 lesions. Of the 15 patients who did not sustain a recurrence, only 1 did not return to full activities. Mean lesion volume was 107 cc. Average follow-up was 19.6 months. Two recurrences were identified, and no other major complications were identified.
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Al-Mouazzen L, Rajakulendran K, Ahad N. Fibrous dysplasia, shepherd's crook deformity and an intra-capsular femoral neck fracture. Strategies Trauma Limb Reconstr 2013; 8:187-91. [PMID: 24036944 PMCID: PMC3800513 DOI: 10.1007/s11751-013-0174-7] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/22/2012] [Accepted: 08/20/2013] [Indexed: 12/18/2022] Open
Abstract
Fibrous dysplasia (FD) is a rare bone disorder in which normal medullary bone is replaced by fibro-osseous tissue. It typically presents in childhood with pain, skeletal deformities, gait abnormalities and occasionally, fatigue fractures. The management of FD remains a challenge. Surgical procedures have been developed to provide symptom relief, correct skeletal deformity and offer mechanical support in cases at risk of fracture. However, there is a paucity of data on the management of acute femoral neck fractures in the adult population with FD. We report the case of a 23-year-old man with a shepherd’s crook deformity secondary to FD, who sustained an intra-capsular femoral neck fracture whilst playing football. The patient initially underwent closed reduction and internal fixation with cannulated screws. However, during the procedure, a guide wire broke whilst inside the femoral head. The patient was referred to the senior author, who undertook a second operation to remove the metalwork and correct the varus deformity using a closing-wedge femoral osteotomy, whilst achieving osteosynthesis at the fracture site. At 1-year follow-up, the patient is pain-free and demonstrates a full range of movement. These cases can be technically demanding and carry a greater risk of complication. It is important that preoperative planning is undertaken and surgery performed by individuals with experience in managing FD and complex femoral neck fractures. Correction of the skeletal deformity whilst fixing the fracture will help restore the mechanical axis and reduce the risk of a recurrent fracture.
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Affiliation(s)
- Louay Al-Mouazzen
- ST3 Trauma & Orthopaedics Cheltenham General Hospital, Gloucestershire, UK,
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31
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Tong Z, Zhang W, Jiao N, Wang K, Chen B, Yang T. Surgical treatment of fibrous dysplasia in the proximal femur. Exp Ther Med 2013; 5:1355-1358. [PMID: 23737878 PMCID: PMC3671783 DOI: 10.3892/etm.2013.987] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/04/2012] [Accepted: 02/11/2013] [Indexed: 12/16/2022] Open
Abstract
The aim of this study was to summarize oncological and functional results and to investigate surgical treatment methods and efficacies by conducting a retrospective study of patients with fibrous dysplasia (FD) in the proximal femur. A total of 15 patients with FD in the proximal femur were selected. Among them, 12 cases were monostotic and 3 cases were polyostotic. In addition, 2 cases were accompanied by shepherd’s crook deformity. All cases received internal fixation following focus curettage and impaction grafting. Among them, valgus osteotomy was conducted for 2 cases of shepherd’s crook deformity. All patients were followed up for 12–32 months. For 2 patients with shepherd’s crook deformity in the proximal femur, the collodiaphysial angle was recovered after an orthopedic procedure following osteotomy. In addition, no patient presented with postoperative recurrent lesions. At 3 months following surgery, local bone resorption was visible in the bone grafting area. Between 8 and 12 months after surgery, the bones in the bone grafting area had healed, pain had disappeared and gaits were nearly normal. An effective internal fixation following thorough focus curettage and allograft bone transplantation is an effective method of treating FD in the proximal femur. For patients with shepherd’s crook deformity, it is also necessary to perform valgus osteotomy to recover hip joint function.
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Affiliation(s)
- Zhichao Tong
- Department of Osteopathy, Xi'an Red Cross Hospital, Xi'an, Shaanxi 710054
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Valgus osteotomy in combination with dynamic hip screw fixation for fibrous dysplasia with shepherd's crook deformity. Arch Orthop Trauma Surg 2013; 133:147-52. [PMID: 23161149 DOI: 10.1007/s00402-012-1633-1] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2012] [Indexed: 02/07/2023]
Abstract
BACKGROUND AND OBJECTIVES The treatment of fibrous dysplasia with shepherd's crook deformity is a big challenge. The purpose of this study was to investigate the clinical effect of valgus osteotomy in combination with dynamic hip screw (DHS) fixation to treat fibrous dysplasia with shepherd's crook deformity. METHOD Twenty-one clinical cases of femoral fibrous dysplasia with shepherd's crook deformity treated between April 2001 and May 2010 were retrospectively analyzed. The valgus osteotomy and internal fixation were performed for these patients. Six patients underwent DHS and trochanter stabilizing plate internal fixation, and the other 15 cases were stabilized by DHS fixation. RESULTS Patients were followed for 19-128 months. The neck-shaft angle was corrected from 89° (range 65°-107°) preoperatively to 129° (range 119°-140°) postoperatively. Limb-length discrepancy was corrected from 3.0 (range 1.8-4.5) cm preoperatively to 0.7 (range 0-1.9) cm postoperatively. All osteotomies had healed at the final follow-up examination. The clinical scores, which were evaluated by the modified criteria of Guille, improved from an average of 2.9 (range 1-7) to 8.5 (range 6-10). CONCLUSION Our results demonstrate that valgus osteotomy in combination with DHS internal fixation is an easy and effective method for the treatment of fibrous dysplasia with shepherd's crook deformity. It can restore the neck-shaft angle and re-establish the mechanical alignment of the femur to improve function.
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33
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Knapik JA. Fibro-Osseous Lesions. Surg Pathol Clin 2012; 5:201-29. [PMID: 26837922 DOI: 10.1016/j.path.2011.07.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/14/2022]
Abstract
This article describes the clinical, radiographic, gross, microscopic, and histologic features; differential diagnosis; molecular pathology; treatment; and prognosis of fibrous dysplasia, osteofibrous dysplasia, and adamantinoma of long bones.
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Affiliation(s)
- Jacquelyn A Knapik
- Department of Pathology, University of Florida, 1600 South West Archer Road, Gainesville, FL 32610, USA
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Valgus osteotomy combined with intramedullary nail for Shepherd's crook deformity in fibrous dysplasia: 14 femurs with a minimum of 4 years follow-up. Arch Orthop Trauma Surg 2010; 130:497-502. [PMID: 19629503 DOI: 10.1007/s00402-009-0943-4] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2009] [Indexed: 02/07/2023]
Abstract
BACKGROUND AND OBJECTIVES To investigate the effectiveness of valgus osteotomy combined with intramedullary nail in treatment of Shepherd's crook deformity of fibrous dysplasia. METHOD A retrospective study was performed in 13 patients (14 femora) of fibrous dysplasia who were treated in our hospital between August 2000 and February 2005. All patients had Shepherd's crook deformity. Six patients had monostotic disease, and seven had polyostotic disease. There were seven males and six females. The four-step procedure was performed orderly as valgus osteotomy, curettage lesion, massive impaction allograft, and insert intramedullary nail with neck cross pinning. RESULTS All patients were followed up from 4 to 7 years with an average duration of 75.3 months. The average neck-shaft angle was corrected from preoperative 75 degrees (range 55 degrees -100 degrees ) to postoperative 120 degrees (range 95 degrees -130 degrees ). The average extremity lengthening was 3.4 cm (range 2.0-4.5 cm). 19 location of osteotomy showed good union. There were no infection and recurrent fracture and progression of deformity. CONCLUSION The valgus osteotomy can correct Shepherd's crook deformity, prevent recurrent fracture, and restore alignment, thus improve functioning of limb. The intramedullary nail with neck cross pinning should be the first consideration of internal fixation. Massive impaction allograft is the key technique to improve full incorporation of allograft and to prevent pathological fracture.
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Marshman LAG, David KM, O'Donovan DG, Chawda SJ. Fibrous dysplasia of the cervical spine presenting as a pathological fracture. Br J Neurosurg 2009; 18:527-33. [PMID: 15799160 DOI: 10.1080/02688690400012525] [Citation(s) in RCA: 16] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/26/2022]
Abstract
Fibrous dysplasia (FD) is an uncommon benign fibro-osseous abnormality of bone, of unknown aetiology and equal sex incidence, which is most commonly restricted to one bone (monostotic FD: MFD, 70%). Less commonly multiple bones are affected (polyostotic FD: PFD, 27%). Vertebral involvement is uncommon (4%), but more common with PFD (7 - 24%) than MFD (1%). Of 20 cases of FD involving the cervical spine, only three have represented MFD. Unlike cases associated with PFD, all cases presented with acute neck pain without significant neurological impairment after minor trauma. We present the case of a 35-year-old male with MFD who developed a pathological fracture of C3 following minor trauma. Radiographs showed collapse and typical 'ground glass' lucency of C3. CT revealed replacement of C3 cancellous bone by hypodense tissue extending into the right lateral mass. The cortex was thinned and fractured, and encroached upon the right foramen transversarium and spinal canal. Magnetic resonance imaging demonstrated hypo-intensity on both T1 and T2, with uniform contrast enhancement. Subtotal excision was achieved via an anterior C3 corpectomy, with residual FD left within the right lateral mass. Stability was achieved utilizing an iliac crest strut autograft, C2-4 plate-and-screws, and mobilization in a halo frame for 3 months. At 18 months, he remained asymptomatic and without deficit. Radiography, CT and MRI confirmed graft fusion without FD invasion, but with residual right lateral mass FD unchanged in size.
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Affiliation(s)
- L A G Marshman
- Department of Neurosurgery, Oldchurch Hospital, Romford, Essex RM7 0BE, UK.
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36
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Kataria H, Sharma N, Kanojia RK. One-stage osteotomy and fixation using a long proximal femoral nail and fibular graft to correct a severe shepherd's crook deformity in a patient with fibrous dysplasia: a case report. J Orthop Surg (Hong Kong) 2009; 17:245-7. [PMID: 19721165 DOI: 10.1177/230949900901700229] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
We report a case where a one-stage osteotomy and fixation, using a long proximal femoral nail and fibular graft, was performed to correct a severe shepherd's crook deformity (70-degree varus and 50-degree retroversion) of the femoral neck with a pathological stress fracture in a patient with fibrous dysplasia. The neck shaft angle was corrected to 125 degrees. At the 57-month follow-up, the patient was free of pain and had no limp or evidence of recurrence.
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Affiliation(s)
- Himanshu Kataria
- Department of Orthopaedics, Lady Hardinge Medical College, New Delhi, India
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Abstract
Fibrous dysplasia is a developmental anomaly of bone formation that accounts for approximately 7% of benign bone tumors. It exists in a monostotic or polyostotic form, commonly occurs in the proximal femur, and may require total hip arthroplasty (THA) for its management in a select group of patients. The results of THA in patients with this disorder is not known. Of the 27,543 primary THAs performed at our institution between 1969 and 2001, 10 were in patients with fibrous dysplasia (prevalence of 0.04%). Two other patients (2 hips) with the same diagnoses were referred after a failed primary THA. The study group comprised 7 men and 4 women (1 bilateral) with an average age at primary THA of 44 years (range, 23-66 years). Four patients had the monostotic form and 7 patients had the polyostotic form. A cemented stem was used in 7 hips and cementless stem in the rest (N=12). Average follow-up was 15.7 years (range, 2-30 years). Seven hips in 6 patients were revised for loosening of components at an average of 12.5 years, and 2 of these were re-revised for the same reason. Loosening of a cementless femoral component led to early revision in 3 hips. The surgical outcomes for patients with monostotic was better than those with polyostotic disease. Long-term fixation of the femoral component is of concern in patients with fibrous dysplasia. Total hip arthroplasty provided these patients with long-lasting pain relief and function despite the higher rate of complications at the time of surgery.
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Affiliation(s)
- Rafael J Sierra
- Department of Orthopedic Surgery, Mayo Clinic College of Medicine, 200 First St SW, Rochester, MN 55905, USA
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George B, Abudu A, Grimer RJ, Carter SR, Tillman RM. The treatment of benign lesions of the proximal femur with non-vascularised autologous fibular strut grafts. ACTA ACUST UNITED AC 2008; 90:648-51. [PMID: 18450634 DOI: 10.1302/0301-620x.90b5.20330] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
We report our experience of treating 17 patients with benign lesions of the proximal femur with non-vascularised, autologous fibular strut grafts, without osteosynthesis. The mean age of the patients at presentation was 16.5 years (5 to 33) and they were followed up for a mean of 2.9 years (0.4 to 19.5). Histological diagnoses included simple bone cyst, fibrous dysplasia, aneurysmal bone cysts and giant cell tumour. Local recurrence occurred in two patients (11.7%) and superficial wound infection, chronic hip pain and deep venous thrombosis occurred in three. Pathological fracture did not occur in any patient following the procedure. We conclude that non-vascularised fibular strut grafts are a safe and satisfactory method of treating benign lesions of the proximal femur.
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Affiliation(s)
- B George
- The Royal Orthopaedic Hospital, Bristol Road South, Northfield, Birmingham B312AP, UK
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Sakurakichi K, Tsuchiya H, Yamashiro T, Watanabe K, Matsubara H, Tomita K. Ilizarov technique for correction of the Shepherd's crook deformity: a report of two cases. J Orthop Surg (Hong Kong) 2008; 16:254-6. [PMID: 18725684 DOI: 10.1177/230949900801600226] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023] Open
Abstract
We report 2 patients with fibrous dysplasia who underwent correction, using the Ilizarov technique, of Shepherd's crook deformities and pathological fractures of the left femurs. A 12-year-old boy underwent an opening wedge osteotomy and a 48-degree gradual correction, whereas a 43-year-old woman underwent a 34-degree acute correction without osteotomy at the fracture site. Both patients could initiate early weight bearing. Final leg function was excellent and alignment was maintained. No complications were encountered. Both patients had no difficulty sleeping and no major complaints about the Ilizarov technique. It is more important to achieve accurate alignment than resection of the lesion. The Ilizarov technique is effective for treating the Shepherd's crook deformity in patients with fibrous dysplasia.
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Affiliation(s)
- K Sakurakichi
- Department of Orthopaedic Surgery, School of Medicine, Kanazawa University, Kanazawa, Japan
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Buecker PJ, Gebhardt MC. Are fibula strut allografts a reliable alternative for periarticular reconstruction after curettage for bone tumors? Clin Orthop Relat Res 2007; 461:170-4. [PMID: 17806150 DOI: 10.1097/blo.0b013e31804e8470] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Bone tumors occurring in periarticular locations may cause a substantial risk of subchondral fracture and collapse. Poly-methylmethacrylate has been used for reconstruction in these instances because of its immediate structural stability. Allogeneic graft allows for biologic incorporation and remodeling, but it takes longer. Adding a fibula strut graft to the construct seems to provide structural stability during incorporation of the particulate graft. Seventeen of 22 (77.3%) patients returned to their full preoperative level of function after this procedure. We believe adding fibula strut to particulate allograft when using the buttress technique provides structural integrity and biologically active reconstruction, while maintaining a recurrence rate similar to those described for other reconstruction techniques.
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Affiliation(s)
- Peter J Buecker
- Harvard Medical School, Beth-Israel Deaconess Medical Center, Children's Hospital Boston, Boston, MA, USA.
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Watanabe K, Tsuchiya H, Sakurakichi K, Matsubara H, Tomita K. Double-level correction with the Taylor Spatial Frame for shepherd's crook deformity in fibrous dysplasia. J Orthop Sci 2007; 12:390-4. [PMID: 17657561 DOI: 10.1007/s00776-007-1132-3] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/27/2006] [Accepted: 01/29/2007] [Indexed: 12/28/2022]
Affiliation(s)
- Koji Watanabe
- Department of Orthopaedic Surgery, Graduate School of Medical Science, Kanazawa University, 13-1 Takara-machi, Kanazawa, Japan
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Leet AI, Collins MT. Current approach to fibrous dysplasia of bone and McCune-Albright syndrome. J Child Orthop 2007; 1:3-17. [PMID: 19308500 PMCID: PMC2656698 DOI: 10.1007/s11832-007-0006-8] [Citation(s) in RCA: 55] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/15/2006] [Accepted: 12/22/2006] [Indexed: 02/07/2023] Open
Abstract
Fibrous dysplasia (FD) of bone is an uncommon disease caused by sporadic, congenital mutations in the cAMP regulating protein, G(s)alpha. It is an example of somatic mosaicism in which a wide spectrum of disease is possible. Widespread skeletal involvement is often associated with varying combinations of café-au-lait skin spots, and/or endocrine dysfunction (precocious puberty, renal phosphate wasting, hyperthyroidism, and/or growth hormone excess). Unrecognized and untreated endocrine dysfunction can exacerbate the skeletal disease. The diagnosis is usually established on clinical grounds on the basis of physical examination and typical radiographic appearance. Occasionally, gene testing of affected tissue may be helpful. The skeletal sites involved with disease are established at an early age, and the complications of fracture deformity are most pronounced in childhood. Bone pain in the absence of a fracture is more common in adults, but can also be present in children. Treatment with bisphosphonates is usually effective at relieving pain, but probably has no effect on the natural history of the disease. Scoliosis, which was previously thought to be an uncommon occurrence, has been shown to be common and progressive, and as such, warrants investigation and, when necessary, surgical treatment. The surgical management of FD remains challenging. Timing and technique remain controversial, but some consensus exists in that grafting materials (of any type) usually fail and should not be a central aspect of the surgical approach. Intramedullary devices are in general superior to side plates and screws. In extremely widespread disease with very early fracture and deformity, no surgical approach will affect final functional outcome. Efforts should be made for the initiation of international collaborative studies to better define optimal surgical approaches to the treatment of this challenging disease.
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Affiliation(s)
- Arabella I. Leet
- />Division of Pediatric Orthopedics, Johns Hopkins University, Baltimore, MD USA
| | - Michael T. Collins
- />Skeletal Clinical Studies Unit, Craniofacial and Skeletal Diseases Branch, National Institute of Dental and Craniofacial Research, National Institutes of Health, Department of Health and Human Services, Bethesda, MD USA
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Affiliation(s)
- Robert P Stanton
- Department of Orthopaedics, Nemours Children's Clinic, Pensacola, Florida 32504, USA.
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Marshman L, David KM. Fibrous dysplasia. J Neurosurg Spine 2006; 4:516-7; author reply 517. [PMID: 16776368 DOI: 10.3171/spi.2006.4.6.516] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
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Matsuya S, Hatori M, Hosaka M, Ito K, Dohi O, Endo M, Kokubun S. Operative treatment by external fixation for polyostotic fibrous dysplasia in the elbow joint. A case report. Ups J Med Sci 2006; 111:269-74. [PMID: 16961184 DOI: 10.3109/2000-1967-056] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/13/2022] Open
Abstract
Fibrous dysplasia is a condition in which normal skeletal bone changes into fibroblastic stroma and immature bone. We report our experience of a unilateral external fixator applied for the treatment of polyostotic fibrous dysplasia arising in the elbow. The patient was a 38 year-old man. His main complaint was left elbow pain and a mass, which increased in size. The histological diagnosis from the needle biopsy was fibrous dysplasia. An articulated unilateral external fixator was used for immobilization. The lesions in the left distal humerus and proximal ulna were curetted and cancellous bone was packed into the cavity. An external fixator (Elbow Fixator, Orthofix Inc., McKinney, USA) was applied from the proximal humeral shaft to the distal ulnar shaft for postoperative tentative immobilization and preservation of the affected elbow joint movement. At two years after the operation, the range of motion of the affected elbow was 90 degrees in flexion and -35 degrees in extension. The range of motion in pronation and supination of the left forearm was normal. No evidence of recurrence was noted at two years after surgery. The merits of using an external fixator were: an earlier start of range of motion exercises, and a decrease in the risk of tumor dissemination.
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Affiliation(s)
- Shigetsune Matsuya
- Department of Orthopaedic Surgery, Tohoku University School of Medicine, 1-1 Seiryomachi, Aobaku, Sendai 980-8574, Japan
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Abstract
UNLABELLED Treatment of pathologic femoral neck fractures secondary to extensive lesions of fibrous dysplasia and simple bone cysts has been controversial. We think that modified Pauwels' intertrochanteric osteotomy and osteosynthesis can result in sound healing of the lesion and of the fracture, with no recurrence, low complication rates, and good functional results. Seven patients were treated with a uniform approach consisting of biopsy (Stage I) and osteotomy with osteosynthesis (Stage II). Curettage of the lesion and bone grafting were not done. The average followup was 79.28 months (range, 32-142 months). All of the fractures and osteotomy sites healed in means of 14 weeks (range, 12-16 weeks) and 9.1 weeks (range, 8-10 weeks), respectively. All fibrous dysplasia lesions healed radiologically. Grade IV radiographic healing was seen in both patients with simple bone cysts after 35 and 92 months. Using the Musculoskeletal Tumor Society functional evaluation and Toronto Extremity Salvage Score, all the patients had good to excellent results. Complications such as infection, local recurrence, refracture, femoral neck deformity, osteonecrosis of the femoral head, and growth arrest of capital femoral physis were not seen. In addition to biomechanical advantages, the procedure seems to have had a biologic role in healing of the fracture and of the lesion by initiating osteogenesis to replace the defect with new bone. LEVEL OF EVIDENCE Therapeutic study, Level IV (case series--no, or historical control group). See the Guidelines for Authors for a complete description of levels of evidence.
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Affiliation(s)
- N K Magu
- Departments of Orthopaedics, Pt. B.D. Sharma PGIMS, Rohtak (Haryana) India.
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Chen WJ, Chen WM, Chiang CC, Huang CK, Chen TH, Lo WH. Shepherd's crook deformity of polyostotic fibrous dysplasia treated with corrective osteotomy and dynamic hip screw. J Chin Med Assoc 2005; 68:343-6. [PMID: 16038377 DOI: 10.1016/s1726-4901(09)70173-x] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/23/2022] Open
Abstract
Fibrous dysplasia, a condition in which the skeleton fails to develop normally, is characterized by fibroblastic stroma and immature bone. Bowing of the long bones occurs frequently in the polyostotic form, and stress fractures often result. Shepherd's crook deformity is a characteristic feature of fibrous dysplasia. The goal of its treatment is to obtain normal walking ability and relieve pain due to pathologic fracture secondary to the deformity; however, correction of the deformity is a surgical challenge. We present 2 cases of shepherd's crook deformity treated with corrective osteotomy and a dynamic hip screw. Both cases showed good bone healing and no recurrent deformity. The gross deformities were corrected, and both patients were pain-free after operation.
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Affiliation(s)
- Wei-Jen Chen
- Department of Orthopedics and Traumatology, Taipei Veterans General Hospital, Taipei, Taiwan, R.O.C
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Abstract
Fractures through bone tumors are often difficult to treat. We reviewed our combined experience with this problem in children, as well as the existing literature, to formulate management guidelines. For this study, prospective databases (1987 to 2002) from three referral centers were screened for pathologic fractures occurring under the age of 14 years. One hundred five patients presented with fracture through unicameral bone cyst, nonossifying fibroma, fibrous dysplasia, aneurysmal bone cyst and osteosarcoma. Seventeen patients were excluded. The most common primary locations were the proximal humerus and proximal femur. Pathologic fracture through nonossifying fibroma had the best outcome; union occurred with nonsurgical treatment in all cases. Unicameral bone cyst required surgical treatment to avoid persistence of the cyst and refracture. However fracture healing was predictable without surgical treatment. Proximal femoral lesions tended to heal in malunion if not fixed surgically. Aneurysmal bone cyst required surgical treatment for the lesion to heal and to allow the fracture to heal as well. Percutaneous sclerotherapy may be the treatment of choice for many of these lesions. Fibrous dysplasia allows fracture healing with nonoperative therapy. Progressive deformity requires followup and surgical correction. Malignant lesions presenting a pathologic fracture are best managed by initial nonoperative therapy during investigation and neoadjuvant therapy when possible, followed by definitive treatment.
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Affiliation(s)
- Eduardo J Ortiz
- Department of Orthopaedic Surgery, Musculoskeletal Oncology Unit, Fundación Hospital Alcorcon, Madrid, Spain.
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