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The Bone & Joint Journal
Vol. 96-B, Issue 5 | Pages 673 - 676
1 May 2014
Han I Choi ES Kim H

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


The Journal of Bone & Joint Surgery British Volume
Vol. 51-B, Issue 2 | Pages 300 - 306
1 May 1969
Henry A

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


The Bone & Joint Journal
Vol. 97-B, Issue 7 | Pages 1007 - 1011
1 Jul 2015
Kim H Im SB Han I

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


The Journal of Bone & Joint Surgery British Volume
Vol. 44-B, Issue 2 | Pages 302 - 318
1 May 1962
Stewart MJ Gilmer WS Edmonson AS

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 Journal of Bone & Joint Surgery British Volume
Vol. 46-B, Issue 2 | Pages 233 - 243
1 May 1964
Morton KS

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


Bone & Joint Research
Vol. 1, Issue 10 | Pages 272 - 280
1 Oct 2012
De Mattos CBR Binitie O Dormans JP

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.


The Journal of Bone & Joint Surgery British Volume
Vol. 92-B, Issue 8 | Pages 1138 - 1143
1 Aug 2010
Beckles VLL Jones HW Harrison WJ

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.


The Journal of Bone & Joint Surgery British Volume
Vol. 89-B, Issue 8 | Pages 1077 - 1083
1 Aug 2007
Tsuchiya H Morsy AF Matsubara H Watanabe K Abdel-Wanis ME Tomita K

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.