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

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

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

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

Introduction. Metabolic bone disease encompasses disorders of bone mineralization, abnormal matrix formation or deposition and alteration in osteoblastic and osteoclastic activity. In the paediatric cohort, patients with metabolic bone disease present with pain, fractures and deformities. The aim was to evaluate the use of lateral entry rigid intramedullary nailing in lower limbs in children and adolescents. Materials and Methods. Retrospective review was performed for an 11-year period. Lower limb rigid intramedullary nailing was performed in 27 patients with a total of 63 segments (57 femora, 6 tibiae). Majority of patients had underlying diagnoses of osteogenesis imperfecta or fibrous dysplasia (including McCune Albright disease). Mean age at surgery was 14 years. Indications for surgery included acute fractures, prophylactic stabilisation, previous nonunion and malunion, deformity correction and lengthening via distraction osteogenesis. Results. All fractures healed. Correction of deformity was successfully achieved in all segments. Delayed union occurred in 4 segments in 1 patient and was successfully treated with nail dynamization. Other complications included prominence, cortical penetrance and loosening of locking screws. One patient who had lengthening performed had nonunion and was managed with exchange nailing and adjunctive measures. Conclusions. Rigid intramedullary nailing is very effective in stabilisation and deformity correction of long bones in adolescent patients with pathological bone disease. The technique has low complication rates. We recommend the use of this technique in paediatric units with experience in managing metabolic bone conditions

This case series highlights the use of the Ganz approach (trochanteric slide approach) and surgical dislocation for excision of fibrous dysplasia of the femoral neck, pigmented villonodular synovitis and synovial chondromatosis of the hip. The first patient was a 16-year-old girl, who presented with pain in her hip, having fallen whilst playing football. Investigations revealed a fibrous dysplasia, which was successfully excised returning her to an active lifestyle. The second patient was a 27-year-old lady, who presented having suffered left hip pain for four years. She was diagnosed with a pigmented villonodular synovitis, which was excised and the patient was able to return to the gym. The third patient was a 41-year-old lady, who presented after experiencing right hip pain both at night and at rest for a year, without any trauma. She was diagnosed with synovial chondromatosis and returned to all activities of daily living. The Ganz approach allows safe dislocation of the hip joint without the risk of osteonecrosis of the femoral head. We demonstrate that it is possible to obtain excellent exposure of the femoral neck, head and acetabulum to surgically treat these three tumours of the hip. The surgeon can thus be reassured that complete excision of the tumour has occurred. This series can recommend the Ganz approach with trochanteric slide and full surgical dislocation of the hip to excise pigmented villonodular synovitis, synovial chondromatosis and fibrous dysplasia of the hip

A rare case of malignant transformation of fibrous dysplasia to chondrosarcoma involving the pelvis, treated by hemipelvectomy, was described by our team in a published case report. Twenty-four years later, the patient remains recurrence-free, with a good functional outcome that allows him to be independent in everyday activities and work in full time employment. Functional outcome following hemipelvectomy for pelvic malignancy is an evolving topic, as improved imaging and surgical techniques result in earlier diagnosis and a better overall prognosis. Sarcomas involving the pelvis still represent a challenging topic for surgeons. During the last twenty-four years, there have been some advances in the limb- salvage treatment of pelvic tumours. An internal hemipelvectomy is currently considered to be a reasonable treatment option, with good functional outcomes and achievement of satisfactory tumour clearance margins, in well- selected cases. In all cases however, the main focus should be in the adequate resection of the lesion, followed by restoration of maintenance of stability. We report a fascinating case of a patient who underwent internal hemipelvectomy without reconstruction for chondrosarcomatous transformation of pelvic fibrous dysplasia, with emphasis on the clear surgical resection margins and disease-free status of the patient and satisfactory functional outcome. We support that internal hemipelvectomy is an acceptable treatment option in well- selected cases and can achieve clear tumour resection margins, resulting in long term disease-free results, and a good limb- salvage functional outcome. We believe that joint stability in this patient is mainly a result of preservation of the adductor muscle group, which prevents the hip joint from upward migration and allows the patient to use his left leg for walking

Introduction: Mazabraud syndrome is a rare disorder characterised by fibrous dysplasia and intramuscular myxomas. We present six new cases. Method: A reterospective review was performed on six patients from our institution. This is a consecutive series over 48 months. Results: The patient group consisted of three females and three males. The six patients were referred to our bone tumour unit from other hospitals (four by orthopaedic teams and two by general surgeons). The referring hospitals had been unable to exclude a malignant process. Imaging was tailored to each individual case. Three of our six patients demonstrated polyostotic fibrous dysplasia. The commonest site of the osseous leision was the proximal femur (n=4). The majority of the patients had solitary myxomas (n=4) with only two being multiple myxomas. The average size of the myxomas was 3.7 cm. All soft tissue leisions were ipsilateral to the osseous leisions. Five of the six patients were treated with surgical excision. Discussion: In contrast to the existing literature the majority(n=4) of the six patients had solitary myxomas and the male to female distribution was equal. One of the cases was also unique to the current literature with the syndrome present in the right elbow. The number of cases that we have seen in a short time may also be an indication that this syndrome is not as rare as scarcity of the published cases would imply

Isolated femoral neck lesions in children are rare and the differential diagnosis is wide. This study examines the spectrum of chronic non-traumatic bone lesions seen in the femoral neck region in children and emphasises the importance of tissue diagnosis. From 1990 to 2003, 23 children aged 1 to 3 years with chronic bone lesions of the femoral neck region were seen. Their case records and radiographs were reviewed retrospectively. Pain and a limp for 4 to 12 weeks were the main symptoms. Most children (18) had right-sided lesions. All had routine blood investigations, radiographs and bone scans. Histological and microbiological studies were done when pus and granulation tissue were found. We found infections, benign tumours and haematopoetic lesions. There were seven cases of tuberculosis and three of subacute pyogenic osteomyelitis. Six children had simple bone cysts, one a chondroblastoma, one a fibrous dysplasia and three osteoid osteoma. Two had eosinophilic granulomata. All 23 lesions were treated by curettage and biopsied. Bone graft was used in all the simple bone cysts, fibrous dysplasia and chondroblastoma. Internal fixation was used in one displaced fracture and the rest were immobilised in a spica. Follow-up ranged from 9 months to 8 years. All lesions showed healing. Coxa vara occurred in four patients and growth disturbances in five. Tuberculous lesions caused the greatest diagnostic dilemma as they resembled several benign bony conditions. Thorough examination is essential and biopsy is mandatory

Mast cells (MC), the tissue-based effector cells in allergic diseases, have many functions. Within bone tissue, they have been linked with new blood vessel formation and marrow fibrosis and it has been proposed that they are capable of promoting osteoclastic bone resorption. MC numbers are known to increase in a number of osteolytic conditions e.g. osteoporosis, hyperparathyroidism and periodontitis. In fracture callus, too, large numbers of MC are present, especially during the onset of remodelling where it is believed they may be responsible for osteoclast recruitment and/or differentiation. The aim of this study was to look for further evidence of mast cell (MC) involvement in pathological bone resorption. MC activity was assessed in tissue sections of osteolytic conditions including Paget’s disease of bone, rheumatoid arthritis and fibrous dysplasia together with several benign and malignant bone tumours. MCs were identified by toluidine blue staining and by immunostaining with a commercial antibody against MC tryptase. Extensive infiltration of mast cells was observed in fibrous dysplasia, rheumatoid arthritis and Paget’s disease of bone and mast cell accumulation was seen at the bone resorbing margin of a number of enlarging bone tumours including osteosarcoma, giant cell tumour of bone, osteoma and osteoid osteoma. MCs, along with other inflammatory cells, are known to accumulate at the margins of soft tissue tumours where they are thought to promote tumour growth. The current findings are consistent with a similar role for mast cells in the primary bone tumours examined. In each of the conditions studied, an additional role for MC may be that of promoting bone lysis. MC are known to contain numerous factors including TNF-alpha and IL-1, which are potent stimulators of osteoclast formation and activity. It is concluded that MCs may contribute to the fibrosis, angiogenesis and increased bone resorption seen in certain metabolic bone diseases. MC activity may also be an important factor contributing to the lysis that occurs in numerous other pathological situations including at the margins of aggressive primary bone tumours and skeletal metastases, leading to the expansion of these lesions

This case series highlights the use of the Ganz approach and surgical dislocation for excision of fibrous dysplasia of the femoral neck, pigmented villonodular synovitis and synovial chrondromatosis of the hip, which has never been described for use with all three tumours together. These are rare benign tumours, which were found incidentally and required excision. We demonstrate that it is possible to obtain excellent exposure of the femoral neck, head and acetabulum allowing easy inspection, exploration and debridement of these three tumours of the hip

The technique involves impaction of cancellous bone into a cavitary femur. If segmental defects are present, the defects can be closed with stainless steel mesh. The technique requires retrograde fill of the femoral cavity with cancellous chips of appropriate size to create a new endomedullary canal. By using a set of trial impactors that are slightly larger than the real implants the cancellous bone is impacted into the tube. Subsequent proximal impaction of bone is performed with square tip or half moon impactors. A key part of the technique is to impact the bone tightly into the tube especially around the calcar to provide optimal stability. Finally a polished tapered stem is cemented using almost liquid cement in order to achieve interdigitation of the implant to the cancellous bone. The technique as described is rarely performed today in many centers around the world. In the US, the technique lost its interest because of the lengthy operative times, unacceptable rate of peri-operative and post-operative fractures and most importantly, owing to the success of tapered fluted modular stems. In centers such as Exeter where the technique was popularised, it is rarely performed today as well, as the primary cemented stems used there, rarely require revision. There is ample experience from around the globe, however, with the technique. Much has been learned about the best size and choice of cancellous graft, force of impaction, surface finish of the cemented stem, importance of stem length, and the limitations and complications of the technique. There are also good histology data that demonstrate successful vascularization and incorporation of the impacted cancellous bone chips and host bone. Our experience at the clinic was excellent with the technique as reported in CORR in 2003 by M Cabanela. The results at mid-term demonstrated minimal subsidence and good graft incorporation. Six of 54 hips, however, had a post-operative distal femoral fracture requiring ORIF. The use of longer cemented stems may decrease the risk of distal fracture and was subsequently reported by the author after reviewing a case series from Exeter. Today, I perform this technique once or twice per year. It is an option in the younger patient, where bone restoration is desired. Usually in a Paprosky Type IV femur, where a closed tube can be recreated and the proximal bone is reasonable. If the proximal bone is of poor quality, then I prefer to perform a transfemoral osteotomy, and perform an allograft prosthetic composite instead of impaction grafting, and wrap the proximal bone around the structural allograft. I prefer this technique as I can maintain the soft tissues over the bone and avoid the stripping that would be required to reinforce the bone with struts or mesh. Another indication for its use in the primary setting is in the patient with fibrous dysplasia

Introduction. Version abnormalities of the femur, either retroversion or excessive anteversion, cause pain and hip joint damage due to impingement or instability respectively. A retrospective clinical review was conducted on patients undergoing a subtrochanteric derotation osteotomy for either excessive anteversion or retroversion of the femur. Methods. A total of 49 derotation osteotomies were performed in 39 patients. There were 32 females and 7 males. Average age was 29 years (range 14 to 59 years). Osteotomies were performed closed with an intramedullary saw (Figure 1). Fixation was performed with a variety of intramedullary nails. Patients requiring a varus or valgus intertrochanteric osteotomy were excluded. Pure rotational corrections only were performed. Twenty-four percent of patients had a retroversion deformity (average −8° retroversion, range +1 to −23°), 76% had excessive anteversion of the femur (average +36° anteversion, range +22° to +53°). Etiology was post-traumatic in 5 (10%), diplegic cerebral palsy in 4 (8%), fibrous dysplasia in 2 (4%), Prader-Willi Syndrome in 1 (2%) and idiopathic in 37 (76%). Previous surgery had been performed in 51% of hips. Fifty-seven percent underwent concomitant surgery with the index femoral derotation osteotomy, including hip arthroscopy in 39% (labral debridement alone or with femoral neck osteochondroplasty), a tibial derotation osteotomy in 12% and periacetabular osteotomy in 6%. Concomitant tibial osteotomies were performed to correct a compensatory excessive external tibial torsion that would be exacerbated in the correction of excessive femoral anteversion. The modified Harris Hip Score was used to assess the results in patients with a minimum of 24 months follow-up. Results. There were no non-unions. Average time to union was 3.3 months. One late infection occurred 10 months after surgery, treated successfully with hardware removal and antibiotics. Two patients, one with Prader-Willi syndrome and one with Ehlers-Danlos syndrome, were converted to total hip replacement. At an average follow-up of 6.1 years (range 2 to 19.1 years), the modified Harris Hip Score improved by 26 points (p< 0.001, Wilcoxon signed-ranks test). The results were rated as excellent in 71%, good in 22%, fair in 5% and poor in 3%. Subsequent surgery was required in 73%, 93% of which were hardware removals. Discussion and Conclusion. A closed, subtrochanteric derotation osteotomy of the femur is a safe and effective procedure to treat either femoral retroversion or excessive anteversion. Excellent or good results were obtained in 93%, despite the need for subsequent hardware removal in more than two-thirds of the patients. For figures/tables, please contact authors directly.

Introduction:. Sinus histiocytosis with massive lymphadenopathy (SHML) also known as Rosai – Dorfman disease is a disease of bone marrow stem cell origin. It affects lymph nodes primarily. Solitary bone lesions are very rare and can cause diagnostic difficulty. Aim:. To increase the awareness of SHML as a cause of cystic bone lesions. Materials and methods:. A 2 year old presented with 4 months history of pain and swelling of the distal forearm. There was no history of tuberculosis or HIV disease. The swelling was 4 × 3 cm firm, non-fluctuant and slightly tender. There were no lymph nodes. Radiographs showed an oval cystic lesion expanding with a well-defined margin. The ulnar cortex was deficient. CT scan confirmed a cystic lesion with contents of granulation tissue. The Hb and WCC were normal, ESR 20 was, CRP<5 and mantoux was negative. At surgery the lesion was curretted. The contents resembled tuberculous granulation but there was no caseation. The borders were well formed, the ulnar cortex was deficient. Results:. The histology revealed granulation tissue with numerous large histiocytes and immuno chemistry confirmed Rosai Dorfman disease. Healing with sclerosis was seen at 6 months. Discussion:. Rosai Dorfman disease is a systemic disease of bone marrow stem cells and lymphadenopathy is the prominent manifestation. Only ±8% of cases have been reported with bone involvement and 4% of these had no lymphadenopathy. The lesions are cystic and medullary but cortical involvement can occur. Solitary ossseous lesions characterized by a background of histocytes without eosinophils can mimic Langerhans histocytosis, localized osteomyelitis, fibrous dysplasia, tuberculosis, simple or aneurysmal bone cysts and metastatic deposits. Conclusion:. Lesions of haematopoetic origin should be considered in the diagnosis of lucent bone lesions in children

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

Aim. To estimate the risk of bone malignancy arising in premalignant conditions. Methods. There are quite a number of possible premalignant conditions with considerable uncertainty about the actual risk of a bone sarcoma developing. The incidence of these malignant conditions was identified from a prospective database containing 3000 primary bone sarcomas. Results. 178 of the 3000 patients with newly diagnosed bone sarcomas had a pre-exiting condition which in all probability led to the sarcoma. These included 50 with previous radiotherapy treatment and 47 with Paget's disease. 31 patients developed malignancy in HME, 8 with neurofibromatosis and 7 each with Ollier's disease and retinoblastoma. There were 4 malignancies in patients with Mafucci's syndrome, 3 in patients with fibrous dysplasia, 3 in patients with synovial chondromatosis and 2 in patients with Rothmund-Thomson syndrome. Given that the incidence of bone sarcomas is 9/million population per year, our 3000 patients represent 333 million population years. When the incidence of a condition is known in the population this allows an estimation of the risk of malignancy compared with the normal population. Retinoblastoma for instance is known to arise in 1 in 16000 births. The 7 malignancies we saw thus represents a risk to individuals with retinoblastoma of 336/million/yr - a figure 37 times the risk of the normal population. Approximate figures of risk have been calculated for other entities. Conclusion. Data from a supra-regional register allows an approximate estimate of the increased risk of bone tumours in premalignant conditions