Background. Fibrous dysplasia is a developmental anomaly of bone formation that may exist in a monostotic or polystotic form. Surgical treatment is considered advisable only with presence of significant or progressive deformity or persistent pain. Early surgery is indicated before the tumor expands or fracture occurs. Methods. We reviewed a series of 21 patients, 14 had monostotic whereas 7 had polystotic fibrous dysplasia. There was no case of Mc Cune Albright. We treated all of these patients with curettage and corticocancellous bone graft and also fixation with reconstruction nails. Follow up ranged between 1 and 5 years. Functional and radiographic outcomes were scored. Results. Russel Taylor IM nail and Gamma nail were used in 11 and 10 patients, respectively. Their mean age at the time of diagnosis was 28 years for monostotic for of the disease and 20 years for polystotic ones. Postoperatively, All patients had good bone healing and complete incorporation of the implanted graft, although it last longer in the case of corrective osteotomy for severe varus. Using of Gamma nail was easier for us in addition to shorter operation time. Up to now, no case of recurrency or pathologic fracture has been seen in our patients. Chronic hip pain was the most common problem in these patients but they reported no restriction of activity of daily living. Conclusion. Clinical results of reconstruction nails were safe and satisfactory in patients with fibrous dysplasia of proximal femour

Introduction. Fibrous dysplasia is a pathological condition, where normal medullary bone is replaced by fibrous tissue and small, woven specules of bone. Fibrous dysplasia can occur in epiphysis, metaphysis or diaphysis. Occationally, biopsy is necessary to establish the diagnosis. We present a review of operative treatment using the Ilizarov technique. The management of tibial fibrous dysplasia in children are curettage or subperiosteal resection to extra periosteal wide resection followed by bone transport. Materials and Methods. A total of 18 patients were treated between 2010 – 2020; 12 patients came with pain and 6 with pain and deformity. All patients were treated by Ilizarov technique. Age ranges from 4–14 years. 12 patients by enbloc excision and bone transportation and 6 patients were treated by osteotomy at the true apex of the deformity by introducing the k/wires in the medullary cavity with stable fixation by Ilizarov device. The longest duration for bone transport was 16 weeks (14–20 weeks) for application, after deformity correction was 20 weeks. We have never used any kind of bone grafts. Results. All the 18 patients were treated successfully by Ilizarov compression distraction device. The patients with localized tibial pathology with deformity had the shortest period on the Ilizarov apparatus, 14 weeks. Conclusions. Preservation and bone regeneration by distraction histogenesis constitutes a highly conservative limb saving surgery. Patients with bone defects of <10 cm, a great deal of preserved healthy tissue and good prognosis are good candidates for these methods

1 . Twenty patients with fibrous dysplasia, confirmed histologically, are reported and discussed in regard to classification, etiology, pathogenesis and treatment. The various fibrous or fibrocystic lesions of bone are characterised briefly for purpose of contrast, and the position of fibrous dysplasia in this heterogeneous collection is suggested. 2. The classification of fibrous dysplasia based on the degree of skeletal involvement is used, and the diagnostic, therapeutic and prognostic implications of this classification emphasised. The authors endorse the opinion that fibrous dysplasia is a developmental defect. Clinical, histological and radiographic evidence is presented to point to the distinct evolution of the monostotic lesions, to which a positive and aggressive approach is recommended

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

Purpose: Fibrous dysplasia is a rare condition accounting for about 1% of benign bone tumours. Both mono- and polyostotic dysplasia is described. Treatment of small tumours is not particularly difficult, but extensive or complicated tumours are another problem. The purpose of this work was to present the results of surgical treatment of a consecutive series of 64 patients. Particular focus was placed on complications. Material and methods: This retrospective series included 64 patients with fibrous dysplasia. Mean age was 32 years. There were 37 women and 27 men. Monostotic dysplasia was noted in 58 cases. The femur was the most frequent localisation (n=19). Seven patients had a fracture. Histological proof was obtained in 61 patients. In three patients, fibrous dysplasia was associated with another tumour (aneurysmal cyst or adamantinoma). Several therapeutic strategies were proposed: abstention for three patients, biopsy alone for 13, resection for six, curettage for 34. The curettage was filled in 23 cases with osteosynthesis in 11. Eight patients were given other surgical treatments (prosthesis, osteosynthesis, amputation). Results: We had five postoperative complications. At mean follow-up of 45 months, 14% of the patients still suffered from generally mild to moderate pain. Radiologically, there were four recurrences, 12 unchanged, 18 regressions, and 19 remissions (generally after resection or curettage with filling). Long-term follow-up revealed malignant transformation in two patients. Discussion: Lesions treated by biopsy alone cured or regressed in 30% of the cases while curettage with filling led to cure or regression in 70% of the cases. We did not include patients treated by bisphosphonates in this series because of insufficient follow-up. Nevertheless, this treatment appears to have a promising effect on pain. Conclusion: Results of treatment for fibrous dysplasia are not as good as might be suspected for this benign disease. The role of surgery appears to be on the decline, to be replaced by bisphosphonates whose long-term efficacy must be evaluated

A brief review of the literature on malignant change occurring in fibrous dysplasia is given and a further case of a sarcoma arising in a patient with polyostotic fibrous dysplasia is reported

Fibrous dysplasia is a genetic not hereditary disease of the skeleton, most frequently located at the femoral bone. The extension and the radiographic morphology of the femoral lesion are variable, and the prognosis is unpredictable. The purpose of this study is to propose a radiographic classification of the femoral lesion, related to the prognosis and the natural history of the disease. The authors reviewed the radiographic examinations of 25 patients affected by fibrous dysplasia of bone with femoral involvement, who were followed-up at least two years after diagnosis. Eleven patients had bilateral localization of the disease, for a total of 36 affected femurs. A radiographic classification of the femoral lesion was proposed based on the localization, the extension and the type of the lesion. The authors classify the femoral lesions as lytic, lytic with surrounding sclerosis, lytic and sclerotic, sclerotic and complex. They observed a prevalence of the lytic lesions and involvement of the proximal part of the femur. Many lesions (40%) expanded with age, and the sclerotic component also increased. The lytic lesions worsened with age, causing severe deformity of the femur. However the mostly sclerotic lesions turned out to be stable at follow-up. The worsening of the lesion was not related to the sex of the subjects; 77% of the worsened lesions were observed in patients younger than 20 years of age, while 66% of the stable lesions were found in patients older than 20 years. In conclusion, this study demonstrated that the prognosis of the femoral lesion in fibrous dysplasia is related to the type of lesion, as per the radiographic classification here in described. Prognosis is also related to the age of the patient and the presence of a sclerotic component, which suggested a greater stability of the lesion

Although fibrous dysplasia is a benign bone disease, in few cases patient are suffering from severe pain of the skeletal system. The aim of this study was to evaluate the current state regarding pain of patients with fibrous dysplasia treated at our hospital. We searched our digital database since 1990 for patients with fibrous dysplasia. Subsequent we verified the histological diagnosis by reviewing the final pathologic report. Additional we called the identified patients by phone to make an enquiry about their pain course and associated treatment. For rating pain intensity we used a numeric rating scale with a range within zero to ten. We identified 43 patients (21 male, 22 female) with an average age at initial diagnosis of 40 years (range 10 to 72years). The mean follow up was 6 years (range 1 to 23 years). Among these 43 patients we were able to contact 33 by phone. Initial diagnosis was made due to pain in 23 cases, nearly coequal by coincidental examination in 20 cases, for fracture in two cases and for local swelling and bone deformity each time in two cases. Thirty-six patients revealed monostotic and seven patients polyostotic involvement. The following locations were found: three times craniofacial, four times within the spine, eight times at the upper extremity, ten times in the pelvis and 31 times at the lower limb. Two patients were suffering additionally from Mazabraud Syndrome. Actual values at the numeric rating scale regarding pain ranged from 0 to 9 with a mean value of 1. Specific in the polyostotic group we found an average value of 3 and three of seven patients stated a value greater than 5 for persistent pain. Five patients with polyostotic involvement were treated with bisphosphonat for pain control with good response. It is remarkable that patients with polyostotic involvement have marked higher values for pain intensity at the numeric rating scale. So therefore we should have a closer look for potential reasons explaining that fact. In accordance with previous published studies we found that pain decreased by intermittent intravenous application of bisphosphonates

The Pamidronate inhibits osteoclastic bone resorption and have been successfully used as an intravenous infusion in the treatment of fibrous dysplasia (FD) of the bone. We describe the preliminary results of this approach in a 14 years old male patient with a monostotic fibrous dysplasia of the femur. A biopsy was performed before given the biphosphonate. He received 2 cycles of 180 mg intravenous infusion of pamidronate every months (60 mg/day for 3 days): Clinical symptoms, serum levels of calcium and electrolytes were valued during each treatment. X-rays and BMD of total skeleton studies were performed at baseline and every 3 months. We observed a significant clinical improvement of the pain associated with the radiographic evidence of the thickening of bone cortex surrounding the lesion. Therefore, pamidronate seems to be a valid therapeutic option for patients with FD of the bone

We report the case of a 12-year-old boy with polyostotic fibrous dysplasia of the upper femur in whom a massive customised polyethylene prosthesis functioned successfully for more than fifty years

Malignant transformation of fibrous dysplasia to chondrosarcoma is rare. We report a case in which malignancy developed in an area of fibrous dysplasia in the ilium. We believe this to be the second reported case at this site. Treatment was by excision of the hemipelvis including the ala of the sacrum. A review of the literature is presented

1. Monostotic fibrous dysplasia in the long bones occurs most frequently in adolescence. In the jaws it is found mainly in early adult life. It presents later in the ribs, probably because it is often asymptomatic in this site. 2. The disease is equally distributed in both sexes. 3. Reactivation may occasionally occur in later life and in pregnancy. 4. Successful surgical treatment is by no means always easily achieved, and requires, in addition to the problems of fracture fixation and the correction of deformity, careful consideration of the age of the patient, the activity of the lesion and the extent to which it involves the cortical bone

A patient with polyostotic fibrous dysplasia had several fractures of the right lower limb. An above-knee amputation was eventually performed, followed by arthrodesis of the hip. Five years later the stump became painful and swelled with dramatic rapidity. Biopsy showed that this was not due to malignant change, but that an aneurysmal bone cyst had developed in association with the fibrous dysplasia

1. A condition of fibrous dysplasia of the jaws occurring in four brothers and a sister has been under observation since 1931. 2. Three of the five patients have been operated upon for correction of grotesque deformity, with satisfactory results. 3. The pathological nature of the disorder is discussed

We describe our experience with vascularised bone grafting for the treatment of fibrous dysplasia of the upper limb in eight patients, five men and three women, aged between 17 and 36 years. The site was in the humerus in six and the radius in two. Persistent pain, progression of the lesion and pathological fracture with delayed union were the indications for surgical intervention. We used a vascularised fibular graft after curettage of the lesion. Function and radiological progress were serially monitored. Early radiological union of the graft occurred at periods ranging from 8 to 14 weeks. The mean period for reconstitution of the diameter of the bone was 14 months (12 to 18) predominantly through inductive formation of bone around the vascularised graft, which was a prominent feature in all patients. There were no recurrences and none of the grafts sustained a fracture or failed to unite. After operation function was excellent in three patients and good in five. Vascularised bone grafts provide a safe and reliable means of ensuring good continuity of bone with little risk of recurrence and failure

An area of fibrous dysplasia of bone may undergo rapid enlargement which may be due to either cystic degeneration or malignant transformation. These complications may be clinically and radiologically indistinguishable and, unless both are borne in mind, incorrect management may follow. Magnetic resonance imaging was used in one of our cases and was the only imaging modality to demonstrate the true nature of the condition

1. A case of fibrous dysplasia in a woman aged fifty, with unilateral skeletal lesions, cutaneous pigmentation and possibly accelerated skeletal growth, is described. 2. At necropsy, the bony changes were found to be complicated by many of the changes of lipoid granulomatosis. Disseminated plaques of demyelinisation were found in the central nervous system. 3. The implications of the case are discussed

Deformity of the proximal femur in fibrous dysplasia leads to deviation of the mechanical axis of the hip, which may lead to the development of secondary osteoarthritis (OA). This study investigated the prevalence and predisposing factors for the development of OA in patients with fibrous dysplasia of the proximal femur. We reviewed the records of 209 patients from our institutional database with fibrous dysplasia of the proximal femur, investigating possible predisposing factors including patient demographics, the extent of the coxa vara deformity, the presence of peri-articular disease, and the overall burden of skeletal disease. Of the 209 patients, 24 (12%) had radiological evidence of OA in the ipsilateral hip. The prevalence was significantly higher in patients with polyostotic fibrous dysplasia compared with those with monostotic disease (p < 0.001). In a subgroup analysis of patients with polyostotic disease, the extent of deformity (quantified using the neck–shaft angle), and the presence of peri-articular disease (whether in the head of the femur or the acetabulum) were significant predictors of osteoarthritis (neck–shaft angle likelihood ratio (LR) = 0.847 per 1° increase, p = 0.004; presence of lesion in the head of the femur LR = 9.947, p = 0.027; presence of lesion in the acetabulum LR = 11.231, p = 0.014). . Our data suggest that patients with polyostotic fibrous dysplasia have a high risk of developing secondary OA of the hips. This risk is higher in patients with peri-articular disease, and those with a more severe deformity of proximal femur. Cite this article: Bone Joint J 2015;97-B:1007–11

Genetic skeletal disorders constitute a rare and heterogeneous bone diseases often leading to poor quality of life. Several surgical options are available. The surgeon must deal with specific features (bone deformity, previous procedures, abnormal bone quality, stiffness or instability, muscle weakness). The questions concern the feasibility of the procedures and the surgical strategy. 55 patients (26 W, 29 M) were reviewed between 2016 and 2022. The mean age of the patients was 35 years (17–71). The diagnosis included 9 hereditary multiple exostoses, 8 osteogenesis imperfecta, 6 multiple epiphyseal dysplasia congenita, 6 achondroplasia, 4 osteopetrosis, 3 pycnodysostosis, 3 hypophosphatemic rickets, 3 fibrous dysplasia, 2 mucopolysaccharidosis, and 10 miscellaneous. 25 patients were referred for hip problems (40 hips). 4 patients (7 hips) requiring a THA have not been operated (4 planned). 4 patients (6 hips) had a proximal femoral fixation (2 osteotomies, 4 fracture fixations). 17 patients (27 hips) sustained a THA (25 primary, 2 revisions). All of them were operated by one operator, using a posterolateral approach and standard implants (including 7 dysplastic and 2 short stems). No customized implant has been used. As regard the 27 THAs, the mean follow up was 4.2 years (1–12). The early complications included 2 femoral cracks and 1 femur fracture. There were 2 revisions (1 cup loosening at 2 years, 1 stem loosening at 4 years). No infection nor dislocation occurred. All the patients were satisfied with their treatment and regain some autonomy. 3 THA were considered as unfeasible. Constitutional bone diseases need a multidisciplinary program of care. The indication for surgery is based on a mutual trust patient/surgeon, a careful evaluation of benefits/risks, and an accurate imaging to anticipate the difficulties. The expected results are a better function and quality of life, and a stability over time

Aim. Patient quality of life (QoL) in untreated bone infection was compared to other chronic conditions and stratified by disease severity. Method. Patients referred for treatment of osteomyelitis (including fracture related infection) were identified prospectively between 2019 and 2023. Patients with confirmed infection completed the EuroQol EQ-5D-5L questionnaire. Clinicians blinded to EQ-index score, grouped patients according to JS-BACH Classification into ‘Uncomplicated’, ‘Complex’ or ‘Limited treatment options’. A systematic review of the literature was performed of other conditions that have been stratified using EQ-index score. Results. 257 patients were referred, and 219 had suspected osteomyelitis. 196 patients had long bone infection and reported an average EQ-index score of 0.455 (SD 0.343). 23 patients with pelvic osteomyelitis had an average EQ-index score of 0.098 (SD 0.308). Compared to other chronic conditions, patients with long-bone osteomyelitis had worse QoL when compared to different types of malignancy (including bladder, oropharyngeal, colorectal, thyroid and myeloma), cardiorespiratory disease (including asthma, COPD and ischaemic heart disease), psychiatric conditions (including depression, pain and anxiety), endocrine disorders (including diabetes mellitus), neurological conditions (including Parkinson's disease, chronic pain and radiculopathy) and musculoskeletal conditions (including osteogenesis imperfecta, fibrous dysplasia and x-linked hypophosphataemic rickets). QoL in long-bone infection was similar to conditions such as Prada-Willi syndrome, Crohn's disease and juvenile idiopathic arthritis. Patients who had a history of stroke or multiple sclerosis reported worse QoL scores compared to long-bone infection. Patients who had pelvic osteomyelitis gave significantly lower QoL scores when compared to all other conditions that were available for comparison in the literature. In long bone infection, 41 cases (21.0%) were classified as ‘Uncomplicated’, 136 (69.4%) as ‘Complex’ and 19 (9.7%) as ‘Limited treatment options available’. Within classification stratification, patients with ‘Uncomplicated’ long bone infections reported a mean EQ-index score of 0.618 (SD 0.227) which was significantly higher compared to ‘Complex’ (EQ-index: 0.410 SD 0.359, p=0.004) and ‘Limited treatment options available’ (EQ-index: 0.400 SD 0.346, p=0.007). Conclusions. Bone and joint infections have a significant impact on patient quality of life. It is much worse when compared to other common chronic conditions, including malignancy, cardiovascular and neurological diseases. This has not been previously reported but may focus attention on the need for more investment in this patient group