Aims. The aim of this study is to determine the predictors of overall survival (OS) and predictive factors of poor prognosis of conventional high-grade osteosarcoma of the limbs in a single-centre in South Africa. Methods. We performed a retrospective cross-sectional analysis to identify the prognostic factors that predict the OS of patients with histologically confirmed high-grade conventional osteosarcoma of the limbs over ten years. We employed the Cox proportional regression model and the Kaplan-Meier method for statistical analysis. Results. This study comprised 77 patients at a three-year minimum follow-up. The predictors of poor OS were: the median age of ≤ 19 years (hazard ratio (HR) 0.96; 95% confidence interval (CI) 0.92 to 0.99; p = 0.021); median duration of symptoms ≥ five months (HR 0.91; 95% CI 0.83 to 0.99; p < 0.037); metastasis at diagnosis (i.e. Enneking stage III) (HR 3.33; 95% CI 1.81 to 6.00; p < 0.001); increased alkaline phosphatase (HR 3.28; 95% CI 1.33 to 8.11; p < 0.010); palliative treatment (HR 7.27; 95% CI 2.69 to 19.70); p < 0.001); and amputation (HR 3.71; 95% CI 1.12 to 12.25; p < 0.032). In contrast, definitive surgery (HR 0.11; 95% CI 0.03 to 0.38; p < 0.001) and curative treatment (HR 0.18; 95% CI 0.10 to 0.33; p < 0.001) were a protective factor. The Kaplan-Meier median survival time was 24 months, with OS of 57.1% at the three years. The projected five-year event-free survival was 10.3% and OS of 29.8% (HR 0.76; 95% CI 0.52 to 1.12; p = 0.128). Conclusion. In this series of high-grade conventional osteosarcoma of the appendicular skeleton from South Africa, 58.4% (n = 45) had detectable metastases at presentation; hence, an impoverished OS of five years was 29.8%. Large-scale future research is needed to validate our results. Cite this article: Bone Jt Open 2024;5(3):210–217

Bone metastases from renal cell carcinoma are aggressive, osteolytic lesions that often require operative intervention for fracture prophylaxis, fracture fixation or palliation. The lesions are hypervascular and intraoperative bleeding is a serious challenge for the orthopaedic surgeon. The purpose of this study was to determine the efficacy of preoperative tumour embolization in reducing blood loss during operative management of renal cell carcinoma metastases to bone. Patients were identified from a prospectively accumulated database (1996–2006). Inclusion criteria included operative management for renal cell metastasis to the pelvis or appendicular skeleton. Patients that were not embolised preoperatively due to renal insufficiency or obesity were excluded. Embolizations were performed the day of surgery by an interventional radiologist. Post-embolization runs were used to determine the percentage of blood flow reduction to the tumour. Variables analyzed included patient age, gender, location of tumour, surgical procedure, surgical time, number of units of packed red blood cells (PRBC) transfused, estimated intraoperative blood loss (EBL) and percentage embolised according to the post-embolization run. Student’s t-test was used to determine the effects of percentage embolization on EBL and number of units of transfused PRBCs. Thirty-five cases (twenty-eight patients) met the inclusion criteria. There were twenty males and eight females with an average age of sixty-five years (range, forty-three to eighty-nine years). The most common metastatic sites were the femur (nineteen cases), humerus (seven cases) and pelvis (six cases). There were ten cases of intramedullary nailing and twenty-five cases of tumor resection and reconstruction. Average surgical time was 4.5 hours (range, 0.75–10 hours) and average EBL was 1.5 litres (range, 0.25–12 litres). Embolization that successfully blocked at least 75% of the blood flow to the tumour significantly decreased surgical EBL (3.2 vs 0.6 litres, P< 0.05) and units of PRBCs transfused (5.6 vs 1.9, P=0.05) compared to those that did not. Two embolization-associated complications occurred including one case of toe gangrene and one case of muscle ischemia. Preoperative embolization significantly reduces blood loss and red blood cell transfusions resulting from surgical stabilization of renal cell metastases to bone. Close communication between the orthopaedic surgeon and interventional radiologist is imperative to maximise these benefits

Introduction. The pelvis has always been a difficult area for surgeons, with high complication rates from surgery and the perception of poor oncological outcomes. The aim of the study was to look at the surgical and oncological outcomes of pelvic tumours treated at our centre. Methods. From the 3100 primary bone tumours seen at the ROH. Information was retrieved on 539 patients seen with a primary bone tumour of the pelvis. The demographic details, oncological and surgical outcomes were reviewed. Results. The mean age at presentation (39 yrs old) and duration of symptoms prior to diagnosis (30 weeks) were both significantly greater than the appendicular skeleton. The volume at resection was 3.8 times greater than the appendicular skeleton. The rates of wide surgical margins and response to chemotherapy were significantly poorer (p<0.05) than the appendicular skeleton resulting in rates of local recurrence (27% vs 12%, p<0.05) and overall survival that were poorer that the appendicular skeleton (51% vs 31% 10 yr survival, p<0.05). The dilemma of treating pelvic ewings will be discussed together with the subanalysis of the EICESS 92/EUROEWING 99 trials in relation to pelvic ewings sarcoma. Conclusions. Despite poorer oncological outcomes surgical resection of pelvic tumours can deliver good oncological and surgical results but has high complication rates compared to tumours of the appendicular skeleton

The Cierny and Mader classification assists with decision-making in the management of osteomyelitis by strafying the host status and the pathoanatomy of disease. However the anatomical type IV represents a heterogenous group with regards to treatment requirements and outcomes. We propose that modification of the Cierny and Mader anatomical classification with an additional type V classifier (diffuse corticomedullary involvement with an associated critical bone defect) will allow more accurate stratification of patients and tailoring of treatment strategies. A retrospective review of 83 patients undergoing treatment for Cierny and Mader anatomical type IV osteomyelitis of the appendicular skeleton at a single centre was performed. Risk factors for the presence of a critical bone defect were female patients (OR 3.1 (95% CI 1.08– 8.92)) and requirement for soft tissue reconstruction (OR 3.35 (95% CI 1.35–8.31)); osteomyelitis of the femur was negatively associated with the presence of a critical bone defect (OR 0.13 (95% CI 0.03–0.66)). There was no statistical significant risk of adverse outcomes (failure to eradicate infection or achieve bone union) associated with the presence of a critical-sized bone defect. The median time to bone union was ten months (95% CI 7.9–12.1 months). There was a statistically significant difference in the median time to bone union between cases with a critical bone defect (12.0 months (95% 10.2–13.7 months)) and those without (6.0 months (95% CI 4.8–7.1 months)). This study provided evidence to support the introduction of a new subgroup of the Cierny and Mader anatomical classification (Type V). Using a standardised approach to management, comparable early outcomes can be achieved in patients with Cierny and Mader anatomical type V osteomyelitis. However, to achieve a successful outcome, there is a requirement for additional bone and soft tissue reconstruction procedures with an associated increase in treatment time

Introduction. The Cierny and Mader classification assists with decision-making by stratifying host status and the pathoanatomy of the disease. However, the anatomical type IV represents a heterogenous group with regards to treatment requirements and outcomes. We propose that modification of the Cierny and Mader anatomical classification with an additional type V classifier (diffuse corticomedullary involvement with an associated critical bone defect) will allow more accurate stratification of patients and tailoring of treatment strategies. Materials & Methods. A retrospective review of 83 patients undergoing treatment for Cierny and Mader anatomical type IV osteomyelitis of the appendicular skeleton at a single centre was performed. Results. Risk factors for the presence of a critical bone defect were female patients (OR 3.1 (95% CI 1.08–8.92)) and requirement for soft tissue reconstruction (OR 3.35 (95% CI 1.35–8.31)); osteomyelitis of the femur was negatively associated with the presence of a critical bone defect (OR 0.13 (95% CI 0.03–0.66)). There was no statistically significant risk of adverse outcomes (failure to eradicate infection or achieve bone union) associated with the presence of a critical-sized bone defect. The median time to bone union was ten months (95% CI 7.9–12.1 months). There was a statistically significant difference in the median time to bone union between cases with a critical bone defect (12.0 months (95% 10.2–13.7 months)) and those without (6.0 months (95% CI 4.8–7.1 months)). Conclusions. This study provided evidence to support the introduction of a new subgroup of the Cierny and Mader anatomical classification (Type V). Using a standardised approach to management, comparable early outcomes can be achieved in patients with Cierny and Mader anatomical type V osteomyelitis. However, to achieve a successful outcome, there is a requirement for additional bone and soft tissue reconstruction procedures with an associated increase in treatment time

The primary aim of this study was to compare the clinical outcomes of osteoid osteoma (OO) between the group of patients with the presence of nidus on biopsy samples from radiofrequency ablation (RFA) with those without nidus. Secondly, we aimed to examine other factors that may affect the outcomes of OO reflecting our experience as a tertiary orthopaedic oncology centre. We retrospectively reviewed 88 consecutive patients diagnosed with OO treated with RFA between November 2005 and March 2015, consisting of 63 males (72%) and 25 females (28%). Sixty-six patients (75%) had nidus present in their biopsy samples. Patients’ mean age was 17.6 years (4-53). Median duration of follow-up was 12.5 months (6-20.8). Lesions were located in the appendicular skeleton in seventy-nine patients (90%) while nine patients (10%) had an OO in the axial skeleton. Outcomes assessed were based on patients’ pain alleviation (partial, complete, or no pain improvement) and the need for further interventions. Pain improvement in the patient group with nidus in histology sample was significantly better than the group without nidus (OR 7.4, CI 1.35-41.4, p=0.021). The patient group with nidus on biopsy demonstrated less likelihood of having a repeat procedure compared to the group without nidus (OR 0.092, CI 0.016-0.542, p=0.008). Our study showed significantly better outcomes in pain improvement in appendicular lesions compared to the axially located lesions (p = 0.005). Patients with spinal lesions tend to have relatively poor pain relief than those with appendicular or pelvic lesions (p=0.007). Patients with nidus on histology had better pain alleviation compared to patients without nidus. The histological presence of nidus significantly reduces the chance of repeat interventions. The pain alleviation of OO following RFA is better in patients with appendicular lesions than spinal or axially located lesions

Introduction. The proximity of the superior tibiofibular articulation to neurovascular structures makes the management of extraosseous tumours of the proximal fibula challenging in bone tumour surgery. The aim of study is to establish whether the recurrence rates are higher in this anatomical area compared to data that already exists in publication for bone tumours throughout the appendicular skeleton. Method. A retrospective study of all patients treated for Ewing's tumours and osteosarcoma at a regional bone tumour unit between January 1995 and December 2009 was conducted. Results. Between January 1995 and December 2009, 17 patients with proximal fibular Ewing's tumours and osteosarcoma were managed surgically at a regional bone tumour unit. The overall recurrence rate of proximal fibular Ewings and osteosarcoma was found to be 29.4% and the overall 5 year survival was 70.5%. When separating the data according to tumour type, the results in this study demonstrated a recurrence rate of 50% for osteosarcoma and 11.1% for Ewing's Tumours. Five year survival was 50% for osteosarcoma and 88.9% for Ewing's tumours. Conclusion. This series demonstrated that aggressive bone tumours of the proximal fibula are a difficult entity to manage and recurrence rates and patient survival for osteosarcoma are poor when compared to published rates for the rest of the appendicular skeleton. While local surgical treatment and adjuvant therapy remain the standard treatment we recommend more radical tumour resection and post-operative radiotherapy for osteosarcoma of the proximal fibula

Solitary plasmacytomas in the appendicular skeleton are rare monoclonal expansions of plasmacytoid cells. They are two main hazards; local destruction of bone with resultant loss of function and possible fracture, and progression to Myeloma. Between February 1988 and July 2005 seven patients (4 male, 3 female) were treated for solitary plasmacytoma with surgical resection and endoprosthetic reconstruction. The median age was 46.7 (35-75). The site was: distal humerus (2), proximal humerus (2) proximal femur (2) proximal tibia (1). Three patients had sustained a pathological fracture. Five patients had received pre-operative radiotherapy and three received post-operative radiotherapy. Mean follow-up is 8.6 years. Two cases became infected at 2 and 5 years post-operatively and have had revisions of their endoprosthesis. Both remain functional at 18 and 15 years. No patient has suffered a local recurrence. Two patients have progressed to multiple Myeloma but no patients have died. Literature review shows that the progression of solitary bone plasmacytoma to Myeloma is around 53% despite radiotherapy, in an average period of 2-4 years. With resection and endoprosthetic reconstruction, the progression in this series has been 28% despite an average follow up of 8.6 years. Although the numbers are small, due to the rarity of the condition, surgical resection and endoprosthetic reconstruction reduces disease progression than radiotherapy alone. This produces far superior results compared to the intramedullary nailing of the long bones for this condition. Endoprosthetic reconstruction after resection should be given consideration in cases of solitary plasmacytoma of the appendicular skeleton when there is extensive bone destruction present. The optimal timing of local radiotherapy to be combined with surgery is still to be established

Objectives:. To determine the diagnostic performance of image-guided percutaneous core needle biopsy (CNB) in patients presenting with pathologic fractures of the appendicular skeleton. To determine factors associated with non-diagnostic biopsy and identify cases which should be considered for primary open biopsy. A retrospective audit identified 129 consecutive patients presenting with pathological fractures to a specialist orthopaedic oncology unit over a 9 year period. All patients underwent percutaneous CNB using CT (n=98), fluoroscopy (n=15) or US (n=16) guidance. In all cases MRI or CT was available prior to biopsy to assess the presence and degree of extra-osseous tumour mass. The resulting sample was classified as diagnostic (Group 1) or non-diagnostic (Group 2) on histopathological study. Diagnostic performance was evaluated on the basis of the diagnostic yield and accuracy; these were related to the site of the lesion and presence/absence of extra-osseous mass. Of 129 biopsies, 99 (77%) were classified as Group 1 and 30 (23%) as Group 2. The commonest sites of pathological fracture without associated soft tissue component and resulting in a non-diagnostic biopsy were the proximal femur and proximal humerus. The average cross-sectional diameter of lesions in Group 1 was 5.7 x 5.9cm. Of the 30 lesions comprising Group 2, no soft tissue component was identified on pre-biopsy cross-sectional imaging in 27 lesions (90%) whereas the remaining 3 (10%) showed a smaller extra-osseous soft tissue component compared to the lesions in Group 1. Image-guided percutaneous CNB is a reliable method for obtaining a tissue diagnosis in patients presenting with a pathologic fracture of the appendicular skeleton with high accuracy rate. However, those lesions which are purely intra-osseous or have only very small extra-osseous components are likely to be associated with a non-diagnostic biopsy, and should be considered for a primary open procedure

To assess the referral system and the clinical notes and radiographs of patients presenting with metastatic disease of long bones. All oncology consultants and registrars received a questionnaire regarding referral to the orthopaedic department for metastatic disease of the appendicular skeleton. Ninety three percent of oncologists did not use a reliable scoring system to assess risk of pathological fracture. The majority of oncologists referred with regards to degree of cortical erosion and increased pain on weight-bearing. Sixty percent felt an improvement in communication between the departments was required. The notes and radiographs were reviewed of twentyfive patients presenting with femoral metastatic lesions to the oncology department. Mirels scoring system was then applied to each patient to assess the risk of the possibility of a pathological fracture. Ten patients had a Mirels score of greater than eight. Three patients were referred for an Orthopaedic opinion regarding prophylactic fixation. Two patients had no fracture of the femur after three months. Five patients had a pathological fracture within three months, resulting in an emergency admission for surgery. Three patients had a Mirels score of 8. One patient suffered a pathological fracture. Twelve patients had a Mirels score of less than 8. None of these patients were referred for an Orthopaedic opinion. None of these patients had a pathological fracture within three months. In conclusion, we presently do not offer a multidisciplinary approach to metastatic disease affecting the appendicular skeleton. The majority of patients’ who score eight or above in the Mirels scoring system are at risk of fracture and do require prophylactic surgery. In keeping with the BOA guidelines, “Metastatic Bone Disease: A Guide to Good Practice”, we would recommend the introduction of a multidisciplinary approach and the use of a recognised scoring system to improve patient care

To determine the usefulness of isotope bone scintigraphy in investigating skeletal pain in children, we reviewed the bone scans, plain radiographs and clinical notes of consecutive children under 16 years of age presenting to children’s orthopaedic surgeons at two teaching hospitals in one city. There were 229 patients, of which 87 were boys and 142 girls. They had an average age of 11 years. 139 were investigated for back pain and 90 for skeletal pain in the appendicular skeleton. They were investigated for a variety of conditions including idiopathic back and skeletal pain, scoliosis, Scheuermann’s disease, spondylolysis, osteomyelitis and postoperative pain. There were positive scans in 4 out of 78 patients with idiopathic back pain, and 13 out of 64 with idiopathic skeletal pain. Overall the positive scan rate for all conditions was 10% for back conditions and 22% for pain in the appendicular skeleton. Of all patients with back pain the management was altered in only 3 children. Of all those investigated for appendicular skeletal pain, the management was altered in 6 children. Isotope bone scanning is a low yield and non-specific investigation that imparts a significant dose of radiation to the patient. It should not be used as a first line investigation for idiopathic back or skeletal pain in children. Other tools such as MRI should be considered initially. It still has a role in the investigation of children with obvious abnormality on radiographs, with spondylolysis and probably where there are worrying clinical features to the pain such as night pain and recent onset. The role of bone scanning in the investigation of skeletal pain should be re-evaluated in the investigation of skeletal pain

Ewing Sarcoma is the second most common primary bone sarcoma in young patients, however, there remains geographical variation in the treatment of these tumours. All patients receive neoadjuvant chemotherapy and, in most cases, the soft tissue mass diminishes significantly in volume. Controversy surrounds whether to then treat the pre- or post-chemotherapy tumour volume. Many centres advocate either (1) resection of the pre-chemotherapy volume or (2) treatment of the pre-chemotherapy volume with radiation followed by resection of the post-chemotherapy volume. These approaches increase both the short and long-term morbidity for this young patient population. In this study, we retrospectively reviewed our experience resecting only the post-chemotherapy volume without the use of (neo)adjuvant radiotherapy. A retrospective analysis of all patients with Ewing Sarcoma treated at a tertiary orthopaedic oncology centre was conducted. All patients were treated as per the consensus opinion of the multidisciplinary tumour board. Demographic and oncological variables were collected from our institutional database. Presentation and re-staging MRI scans were reviewed to evaluate pre- and post-chemotherapy tumour volumes. Operative and pathology reports were utilized to determine the extent of the surgical resection. Outcome variables included local recurrence free-, metastasis free- and overall survival. Sixty-five patients were identified in our institutional database of which 56 did not receive (neo)adjuvant radiotherapy. Median age at diagnosis was 24 years (range 13–64), 60% of patients were male and 67.6% of tumours were located in the appendicular skeleton. All 56 patients not treated with radiotherapy had resection of the post-chemotherapy tumour volume. There were 3 local recurrences in this group with a mean follow-up of 70.8 months (range 2 to 328). The median overall survival was 47 months and the mean of 70.8months. The rate of local recurrence is comparable to reports in the literature in which patients had their entire pre-chemotherapy tumour volume treated by radiation and/or surgery. Similarly, two-year overall survival for our patient cohort is not significantly different from previous studies in which more aggressive local control measures were employed. Resecting the post-chemotherapy tumour volume in Ewing Sarcoma without the use of (neo)adjuvant radiotherapy does not appear to increase the risk of local recurrence or negatively impact overall survival. This approach should be studied further as it reduces the risk of short and long-term complications for this patient population.”

The rate of fracture and subsequent nonunion after radiation therapy for soft-tissue sarcomas and bone tumors has been demonstrated to quite high. There is a paucity of data describing the optimal treatment for these nonunions. Free vascularized fibular grafts (FVFG) have been used successfully in the treatment of large segmental bone defects in the axial and appendicular skeleton, however, their efficacy with respect to treatment of radiated nonunions remains unclear. The purpose of the study was to assess the 1) union rate, 2) clinical outcomes, and 3) complications following FVFG for radiation-induced femoral fracture nonunions. We identified 24 patients who underwent FVFG for the treatment of radiation-induced femoral fracture nonunion between 1991 and 2015. Medical records were reviewed in order to determine oncologic diagnosis, total preoperative radiation dose, type of surgical treatment for the nonunion, clinical outcomes, and postoperative complications. There were 11 males and 13 females, with a mean age of 59 years (range, 29 – 78) and a mean follow-up duration of 61 months (range, 10 – 183 months). Three patients had a history of diabetes mellitus and three were current tobacco users at the time of FVFG. No patient was receiving chemotherapy during recovery from FVFG. Oncologic diagnoses included unspecified soft tissue sarcomas (n = 5), undifferentiated pleomorphic sarcoma (UPS) (n = 3), myxofibrosarcoma (n = 3), liposarcoma (n = 2), Ewing's sarcoma (n = 2), lymphoma (n = 2), hemangiopericytoma, leiomyosarcoma, multiple myeloma, myxoid chondrosarcoma, myxoid liposarcoma, neurofibrosarcoma, and renal cell carcinoma. Mean total radiation dose was 56.3 Gy (range, 39 – 72.5), given at a mean of 10.2 years prior to FVFG. The average FVFG length was 16.4 cm. In addition to FVFG, 13 patients underwent simultaneous autogenous iliac crest bone grafting, nine had other cancellous autografting, one received cancellous allograft, and three were treated with synthetic graft products. The FVFG was fixed as an onlay graft using lag screws in all cases, additional fixation was obtained with an intramedullary nail (n = 19), dynamic compression plate (n = 2), blade plate (n = 2), or lateral locking plate (n = 1). Nineteen (79%) fractures went on to union at a mean of 13.1 months (range, 4.8 – 28.1 months). Musculoskeletal Tumor Society scores improved from eight preoperatively to 22 at latest follow-up (p < 0.0001). Among the five fractures that failed to unite, two were converted to proximal femoral replacements (PFR), two remained stable pseudarthroses, and one was converted to a total hip arthroplasty. A 6th case did unite initially, however, subsequent failure lead to PFR. Seven patients (29%) required a second operative grafting. There were five additional complications including three infections, one wound dehiscence, and one screw fracture. No patient required amputation. Free vascularized fibular grafts are a reliable treatment option for radiation-induced pathologic femoral fracture nonunions, providing a union rate of 79%. Surgeons should remain cognizant, however, of the elevated rate of infectious complications and need for additional operative grafting procedures

Orthopaedic problems are common in patients with Ehlers-Danlos Syndrome (EDS). Articular hypermobility can be particularly disabling leading to instability in the appendicular skeleton. We present a case of an EDS patient presenting with knee pain and instability. It highlights important lessons to be learned when considering joint replacement in this patient group. A 51 year old lady with EDS underwent a posterior cruciate retaining total knee replacement for pain and instability. She dislocated her knee replacement three months post-operatively after a fall. Her knee was reduced at her local emergency department causing injury to the popliteal artery. She required urgent popliteal artery repair and fasciotomies. The common peroneal nerve was also irreversibly damaged by the dislocation. She has since had one further dislocation and is now awaiting revision surgery. When considering total knee replacement (TKR) in EDS, the patient must be warned of the inferior results compared to TKR for other causes. The increased risk of complications must be explained and a more constrained TKR design considered to address the inherent joint laxity. The potential consequences of a dislocated TKR can be disastrous and therefore relocation must be performed in a controlled environment in the operating theatre

Aims: To determine the usefulness of isotope bone scintography in investigating skeletal pain in children. Methods: We reviewed bone scans, notes and radiographs requested for children under 16 years presenting to two teaching hospitals in the city. Results: There were 229 patients with and average age of 11 years. 139 were investigated for back pain and 90 for skeletal pain in the appendicular skeleton. There were positive scans in 13 patients with back pain and 22 with pain elsewhere. The management was altered in only 3 children with back pain and 6 with other skeletal pain. Conclusions: Isotope bone scintigraphy is a low yield, and non-speciþc investigation that imparts a signiþcant dose of radiation. Its role in the investigation of skeletal pain should be re-evaluated

In idiopathic scoliosis the detection of extra-spinal left-right skeletal length asymmetries in the upper limbs, ribs, ilia and lower limbs [1–7] begs the question: are these asymmetries unconnected with the pathogenesis, or are they an indicator of what may also be happening in immature vertebrae of the spine? The vertebrate body plan has mirror-image bilateral symmetries (mirror symmetrical, homologous morphologies) that are highly conserved culminating in the adult form [8]. The normal human body can be viewed as containing paired skeletal structures in the axial and appendicular skeleton as a) separate left and right paired forms (e.g. long limb bones, ribs, ilia), and b) united in paired forms (e.g. vertebrae, skull, mandible). Each of these separate and united pairs are mirror-image forms – enantiomorphs. In idiopathic scoliosis, genetic and epigenetic (environmental) mechanisms [9–11] may disturb the symmetry control of enantiomorphic immature bones [12–13] and, by creating left-right endochondral growth asymmetries, cause the extra-spinal bone length asymmetries, and within one or more vertebrae create growth conflict with distortion as deformities (= unsynchronised bone growth concept) [14]. Conclusion: This enantiomorphic disorder concept applied to the axial skeleton during infancy, juvenility and adolescence – through reductionism into the molecular mechanisms of growth plate responses to different hormones at successive phases of development – provides a new theoretical insight to explain the whole body deformity of AIS. The concept suggests preventive surgery on spine and ribs

Aim: The literature suggests that the incidence of osteomyelitis in the paediatric population has changed. We undertook to examine changes in incidence, causative organisms and treatment regimes over a 13 year period. Methods: Patients admitted with a diagnosis of osteomyelitis between January 1991 and January 2004 were identified from hospital records and data collected from their medical and laboratory records. Results: A total of 362 patients were admitted over the study period with a mean age of 5.9 years. A significant decrease in the number of patients presenting over the study period with osteomyelitis was noted, from a peak of 77 cases in 1991 to 12 cases in 2003 (p< 0.05). There was no significant difference in patient age or length of hospital stay over the study period. The majority of cases involved the lower appendicular skeleton with Staphylcoccus Aureus being the commonest organism cultured (accounting for 60% of positive cultures). All cases were initially treated empirically with intravenous Flucloxicillin and oral Fusidic acid. Surgical debridement/decompression was required in 11% of cases. Conclusion: Osteomyelitis now appears to be a rare condition in children with a marked decrease in the incidence being noted over the study period. This correlates with the introduction of the Haemophilus Influenzae B vaccination in Ireland and may partly explain the decrease in incidence. The majority of cases settled on a course of non-operative management

To assess the referral system, clinical notes and radiographs of patients presenting with metastatic disease of long bones in a regional oncology unit. Thirty questionnaires were sent to oncologists asking about reasons for referral to orthopaedics and use of scoring system to assess risk of pathological fracture. Ninety three percent of oncologists did not use a reliable scoring system to assess risk of pathological fracture. The majority referred in respect to pain on mobilising and the presence of a lytic lesion. Sixty percent felt an improvement in communication between the departments was required. The notes and radiographs were reviewed of thirty-seven patients presenting with femoral metastatic lesions to the oncology department. Sixteen patients had a Mirels score of greater than eight. Four patients were referred for an Orthopaedic opinion. All patients underwent prophylactic fixation. Twelve patients with a score of greater than eight were not referred. Seven of theses patients suffered a pathological fracture within three months. Five patients had a Mirels score of 8. One patient had prophylactic fixation. No fractures occurred. Sixteen patients had a Mirels score of less than 8. None of these patients were referred for an orthopaedic opinion. None of these patients had a pathological fracture within three months. In conclusion, we presently do not offer a multidisciplinary approach to metastatic disease affecting the appendicular skeleton. The majority of patients’ who score eight or above in the Mirels scoring system are at risk of fracture and do require prophylactic surgery. In keeping with the BOA guidelines, “Metastatic Bone Disease: A Guide to Good Practice”, we would recommend that the introduction of a multidisciplinary approach and the use of a recognised scoring system is essential to improve patient care

Osteosarcoma is the most common second malignancy seen in retinoblastoma survivors. Risk of developing osteosarcoma in this group is estimated approximately 500 times higher than the general population. Prognosis in this setting has been reported significantly worse than conventional osteosarcoma despite multimodal management. Purpose of this study was to evaluate clinical features, molecular aspects and outcome of treatment in this subgroup of osteosarcoma patients. Between 1985 and 2004, from a total of about 1100 osteosarcomas, 7 survivors of retinoblastoma developing high-grade osteosarcoma as second malignancy presented at the authors’ Institution. Retrospective study was undertaken to analyze presentation, tissue expression of RB1, P53, PGP and DHFR, treatment and outcome of both retinoblastoma and osteosarcoma. Retinoblastoma was bilateral in 5 cases and unilateral in two. All the patients had been treated with a combination of surgery +/− chemotherapy +/− radiation. None of them had evidence of retinoblastoma at the time of second malignancy diagnosis. Average age at diagnosis of osteosarcoma was 14 years (9–17 years), mean interval between the two malignancies was 155 months. All the osteosarcomas were in the appendicular skeleton, all but one around the knee. Molecular analysis showed defective RB1 gene in all cases All the seven patients received contemporary multimodal management for osteosarcoma. All but one patient died of osteosarcoma within 30 months from diagnosis. The living patient had local recurrence 9 years after limb salvage and is currently disease free following amputation. Prognosis of osteosarcoma in retinoblastoma patients remains poor as compared to conventional high grade osteosarcoma despite multimodal management. No obvious correlation was found between poor prognosis and P53, PGP and DHFR expression