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

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

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

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. The femoral neck in children is a common site for bone lesions. The majority are benign. However these lesions can cause diagnostic problems. Aim. To present a spectrum of chronic lesions of the femoral neck in children and emphasize the importance of tissue diagnosis. Materials and methods. Thirty two children with isolated chronic bone lesions in the femoral neck treated between 1994 and 2013were retrospectively reviewed. The ages ranged between 1–13 years. Clinical features were pain and limp. Routine blood tests, x-rays and CT scans were done in all and MRI scans in 5 cases. All diagnoses were confirmed histologically. Results. Three radiological patterns were seen: lucent or cystic in 22, infiltrative (permeative)in 2, and localized densities with nidus in 8 cases. Histologically the lesions were subacute osteomyelitis in 4, tuberculosis in 9, simple bone cyst in 7, osteoid osteoma in 7, chondroblastoma in 1, monostotic fibrous dysplasia in 2 and eosinophilic granuloma in 2 cases. Two tuberculous lesions were associated with subluxation of the hip and involvement of the head occurred in 2 others. Treatment and outcome. All lesions were curetted. Bone grafting was done in 10. Immobilisation was by internal fixation in 1, traction in 2 and spica cast in 29 cases. Follow up was 9 months to 11 years. Healing occurred in the majority. Recurrence occurred in 2 cases. Coxa vara developed in 6, and growth disturbance with shortening in 9 patients. Discussion. Femoral neck lesions are mainly benign, present diagnostic difficulty and treatment is challenging. There are problems with immobilization and of purchase with fixation devices due to poor bone stock on the neck of femur. The spica cast is a reliable method of immobilization in children under 10years. Growth disturbance and coxa vara can result after healing. CT scan is useful in assessing the architecture of the bone. NO DISCLOSURES

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

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

1 . Six patients have been presented in whom an established diagnosis of non-osteogenic fibroma of bone was made. Metaplastic bone was identified within the tumour tissues. 2. Three other patients are reported in whom the diagnosis appeared to be, on radiological and histological grounds, either fibrous dysplasia or non-osteogenic fibroma. 3. This evidence has convinced the author that the two lesions are frequently not distinctive and that they are, in fact, closely related. Because the natural history of the two conditions, especially in their simple or monostotic form, is also the same, there is good reason to consider them as varying histological manifestations of the same pathogenetic process

Objective: To report a rare case of lymphomatous transformation in a Pagetic bone. Methods: A 61yr old lady with an 8yr history of monostotic Paget’s disease affecting her right proximal humerus presented with increasing right arm pain. Initial investigations including plain films, a radioisotope bone scan and MRI scan showed evidence of malignant transformation. Results: The patient was admitted for an incision biopsy. Initial pathological examination suggested a high grade Paget’s sarcoma. However, further stains and immunohistochemical markers showed bone involvement by a malignant B-cell lymphoma. Conclusion: Although sarcoma is by far the commonest malignant transformation of Pagetic bone, rare cases of lymphoma must also be considered, especially since the management and prognosis are radically different

Background: Paget’s disease of the sacrum is rare. A monostotic lesion in the sacrum is reported. A case with an unusual presentation is discussed. Introduction: A 53 years old man was referred to our unit with a 5–6 months history of abdomen discomfort and tenesmus. He had a history of low back pain and was noted to have an area of increasing numbness over the left buttock. A prominence of the left sacroiliac region was noticed and on rectal examination a bony hard mass was palpable posteriorly. A plain x-ray of the pelvis showed a gross expansion and enlargement of the sacrum with lucent area and widespread new bone formation. Biochemical test revealed a raised alkaline phosphatase level. A MRI scan reported a large tumour arising from the sacrum. with a differential of chordoma, paget’s sarcoma or an osteochondroma. In addition a bone scan reported raised uptake in the pelvis. An open incision biopsy was performed and the histology report was consistent with appearance of paget’s disease with no evidence of sarcoma. Interestingly the patient symptoms improved after the biopsy. He was commenced on biphosphonates. A surveillance scan is to be performed in due course. Conclusion: This case was unusual in terms of clinical presentation and location. Furthermore even the most sophisticated imaging modalities may fail to establish the diagnosis and biopsy is then necessary. This should always be performed in specialized centers, in order to minimise complications

Purpose: Aim of this study was to analyse our Institution experience with osteosarcoma in Paget’s disease (PD). Methods: Twenty-six patients treated between 1961 and 2006 were retrospectively analysed. Information focusing on treatment, imaging and oncologic outcome were obtained from clinical charts. Results Fifteen patients had previous diagnosis (mean time 9 yrs) of monostotic (80%) or polyostotic (20%) PD; in 11 sarcoma and PD were contemporarily diagnosed. The most frequents osteosarcoma histotypes observed were osteoblastic in 18 cases (69%), fibroblastic in 5 cases (19%), teleangectasic in 2 patients and chondroblastic in one case. In 6 patients surgery only was performed: 3 amputations and 3 resections. In 3 pts surgery (amputation), adjuvant chemotherapy and radio-therapy were given. In 1 pt surgery (amputation) and radiotherapy. In 12 patients surgery and chemotherapy: adjuvant in 10 cases (8 amputations and 2 resections) and neoadjuvant in 2 (both amputations). Two patients had only radiotherapy and 2 had only chemotherapy. Oncologic outcome showed 4 pts with no evidence sisease at a mean follow up of 139 months (min.42.6, max.257.4) and 22 died with disease at a mean time of 20.15 months (min.1, max. 84). One patient only of 6 (11%) treated with surgery only is NED at 10 years, while the other 5 died from disease at a mean time of 30 mos. Three of 12 patients (25%) treated with surgery and chemotherapy are NED at a mean follow up of 12 years, while 9 died of disease at a mean time of 24 months. All patients treated without surgery died at of 7.5 mos on average (min.1 – max.13.7). Conclusions: Sarcomas in PD have a poor prognosis in pts treated without surgery or with surgery only. Surgery with chemotherapy – when feasible – improves prognosis

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

Introduction Starting in 1994 a series of four studies of Osteolymphoma (Primary Bone Lymphoma) have been undertaken. These studies were intended to characterise the disease and provide reliable information about treatment outcomes. Methods Following a pilot study of 17 patients at West-mead hospital, an Australia-wide survey was conducted through the Australasian Radiation Oncology Lymphoma Group (AROLG) and data were published relating to 70 cases from nine institutions. Thereafter a prospective clinical trial was started under the auspices of the Trans-Tasman Radiation Oncology Group (TROG) and is currently accruing satisfactorily. To foster accrual to this trial the International Extranodal Study Group (IELSG) was invited to participate and they indicated they would only do so after a retrospective study of their own databases was undertaken. This retrospective study is also underway. Results Results so far indicate a highly heterogeneous disease but with some common features, including some previously unnoticed behaviours, particularly the tendency to occur and recur mulitfocally both monostotically and polyostotically. The results are worse than other lymphomas but the use of new combinations of chemotherapy and radiotherapy is being tested and is likely to improve upon the outcomes. After eradicating the disease, there is a significant risk of subsequent fracture related to treatment factors. Orthopaedic surgery still has an important role to play in obtaining sufficient biopsy material without compromising stability, in providing stability where needed prior to radiotherapy and chemotherapy and in the treatment of subsequent fractures. Conclusions Specialists involved in the treatment of bone tumours are encouraged to become familiar with this world-wide, Australian based line of investigation so that maximum accrual can be achieved. In relation to the conduct of this study, one or more of the authors is in receipt of a research grant from a non-commercial source

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

Purpose: The cause of Paget’s disease, osteitis deformans, defined by increased bone resorption followed by excessive attempts at repair, remains unclear. The current hypothesis is that bone is slowly infected by paramyxovirus in genetically predisposed patients. We report the case of a patient with monostotic Paget’s disease of the pelvis which was transferred to the homolateral distal femur after total hip arthroplasty. Case report: A 66-year-old man with Paget’s disease of the hemi-pelvis developed pagetic hip deformation requiring a total hip arthroplasty performed in 1993. A Charnley-Kerboull cemented prosthesis was implanted. The medullary canal was plugged with fragments of cancellous bone harvested from the acetabulum (drill holes required to achieve rotatory stability of the acetabulum). One year later, an asymptomatic osteolytic lesion of the distal femur was identified at the level of the medullary plug. Hip function remained excellent despite extension to the distal and proximal femur. All inflammatory parameters were normal and the femoral component was not loosened. 99m-technetium scintigraphy revealed strong uptake in the pelvis but also in the distal femur under the femoral stem. Isolated femoral stem revision was performed in 1996 as a preventive measure against stress fracture. Pathology analysis of surgical specimens identified Paget’s disease (osteoclast hyperactivity, mosaic aspect of the osteoblasts. After femoral revision with a long stem and medical treatment with biphosphonates, the osteolytic lesion disappeared progressively, taking on the classical sclerous sequelar aspect of osteitis deformans. The disease remained quiescent six years after revision. Hip function remained excellent at last follow-up. Discussion and conclusion: This is the first report of this type of complication after total hip arthroplasty. This observation that Paget’s disease can be transferred via a autologous cancellous bone graft is in favour of the viral hypothesis, suggesting osteoclastic infection by contiguity. This complication has never been observed with frozen or radiated allograft material although such material may have been harvested from pathological bone, notably with Paget’s disease

Pathological fractures in children can occur as a result of a variety of conditions, ranging from metabolic diseases and infection to tumours. Fractures through benign and malignant bone tumours should be recognised and managed appropriately by the treating orthopaedic surgeon. The most common benign bone tumours that cause pathological fractures in children are unicameral bone cysts, aneurysmal bone cysts, non-ossifying fibromas and fibrous dysplasia. Although pathological fractures through a primary bone malignancy are rare, these should be recognised quickly in order to achieve better outcomes. A thorough history, physical examination and review of plain radiographs are crucial to determine the cause and guide treatment. In most benign cases the fracture will heal and the lesion can be addressed at the time of the fracture, or after the fracture is healed. A step-wise and multidisciplinary approach is necessary in caring for paediatric patients with malignancies. Pathological fractures do not have to be treated by amputation; these fractures can heal and limb salvage can be performed when indicated.

We present a retrospective review of 167 patients aged 18 years and under who were treated for chronic haematogenous osteomyelitis at our elective orthopaedic hospital in Malawi over a period of four years. The median age at presentation was eight years (1 to 18). There were 239 hospital admissions for treatment during the period of the study. In 117 patients one admission was necessary, in 35 two, and in 15 more than two.

A surgical strategy of infection control followed by reconstruction and stabilisation was employed, based on the Beit CURE radiological classification of chronic haematogenous osteomyelitis as a guide to treatment. At a minimum follow-up of one year after the end of the study none of the patients had returned to our hospital with recurrent infection.

A total of 350 operations were performed on the 167 patients. This represented 6.7% of all children’s operations performed in our hospital during this period. One operation only was required in 110 patients and none required more than three. Below-knee amputation was performed in two patients with chronic calcaneal osteomyelitis as the best surgical option for function. The most common organism cultured from operative specimens was Staphylococcus aureus, and the tibia was the bone most commonly affected. Polyostotic osteomyelitis occurred in four patients. We believe this is the largest reported series of patients treated for chronic haematogenous osteomyelitis.

We present a retrospective study of patients suffering from a variety of benign tumours in whom external fixators were used to treat deformity and limb-length discrepancy, and for the reconstruction of bone defects. A total of 43 limbs in 31 patients (12 male and 19 female) with a mean age of 14 years (2 to 54) were treated.

The diagnosis was Ollier’s disease in 12 limbs, fibrous dysplasia in 11, osteochondroma in eight, giant cell tumour in five, osteofibrous dysplasia in five and non-ossifying fibroma in two. The lesions were treated in the tibia in 19 limbs, in the femur in 16, and in the forearm in eight. The Ilizarov frame was used in 25 limbs, the Taylor Spatial Frame in seven, the Orthofix fixator in six, the Monotube in four and the Heidelberg fixator in one. The mean follow-up was 72 months (22 to 221).

The mean external fixation period was 168 days (71 to 352). The mean external fixation index was 42 days/cm (22.2 to 102.0) in the 22 patients who required limb lengthening. The mean correction angle for those with angular deformity was 23° (7° to 45°).

At final follow-up all patients had returned to normal activities. Four patients required a second operation for recurrent deformity of further limb lengthening. Local recurrence occurred in one patient, requiring further surgery.