A Critical Appraisal of the Congress of Neurological Surgeons Evidence-Based Guidelines on the Evaluation and Treatment of Patients With Thoracolumbar Spine Trauma.

Background and objective Thoracolumbar spine trauma (TST) is frequently associated with spinal cord injury and other soft tissue and bony injuries. The management of such injuries requires an evidence-based approach. This study used the Appraisal of Guidelines for Research and Evaluation (AGREE) II instrument to assess the methodological quality of clinical guidelines for the management of TST published by the Congress of Neurological Surgeons (CNS). Methods All clinical guidelines on TST published by CNS until 2020 were assessed. Five appraisers from three international centers evaluated the quality of eligible clinical guidelines by using AGREE II. Mean AGREE II scores for each domain were determined. In higher-quality domains, the scores for individual items were analyzed. Results A total of 12 guidelines published by CNS on TST were assessed. Mean scores for all six domains were as follows: Scope and Purpose (75.2%), Stakeholder Involvement (45.4%), Rigor of Development (57.0%), Clarity of Presentation (58.7%), Applicability (16.9%), and Editorial Independence (64.1%). The mean score for the overall quality of all CNS guidelines was 52.9% [95% confidence interval (CI): 52.2-53.5%]. The overall agreement among appraisers was excellent [intra-class correlation coefficients (ICCs) for each guideline ranged from 0.903 to 0.963]. Conclusions CNS guidelines for the management of TST demonstrated acceptable quality across most domains; however, the domains of Applicability and Stakeholder Involvement could be further improved in future guideline updates. The assessors concluded that all guidelines could still be recommended for clinical practice with or without modifications.

Accelerated polyethylene wear and early failure of the uncemented Reflection acetabular component: a ten to fifteen-year follow-up study.

PURPOSE: The Reflection® second-generation uncemented acetabular component was designed to address increased rates of failure observed with the early acetabular designs. However, the reported survivorship of this acetabular component has been conflicting. The aim of this study was to describe the ten to 15-year survivorship and polyethylene wear rate for the uncemented Reflection® acetabular component performed as part of a primary total hip replacement. METHODS: One hundred and four consecutive Reflection® uncemented acetabular components in 97 patients were identified from a prospective arthroplasty database with a minimum of ten years of follow-up. No patient was lost to follow-up. Mean cohort age was 59.1 years. RESULTS: There were 24 revisions of the acetabular component. The all-cause survival rate at ten years was 77.2% [95% confidence interval (CI) 73.9-80.5]. Mean linear wear was 0.20 mm [standard deviation (SD) 0.08] per year and the volumetric wear was 106.2 mm(3) per year. At final follow-up, more than half the patients had osteolysis identified around the femoral component. The mean Oxford Hip Score was 35.6 (SD 9.8) at a mean follow-up of 11.9 years, with six (11.1%) excellent, 26 (48.1%) good, 17 (31.5%) fair and five (9.3%) poor outcomes. Despite the fact that more than a third had a fair or poor outcome, only four (7.4%) were not satisfied with their hip. CONCLUSIONS: Due to the high rate of relatively asymptomatic polyethylene wear and osteolysis associated with this acetabular component, in our department, we now review all surviving patients both clinically and radiographically on an annual basis.

A micro-architectural evaluation of osteoporotic human femoral heads to guide implant placement in proximal femoral fractures.

BACKGROUND AND PURPOSE: The micro-architecture of bone has been increasingly recognized as an important determinant of bone strength. Successful operative stabilization of fractures depends on bone strength. We evaluated the osseous micro-architecture and strength of the osteoporotic human femoral head. MATERIAL AND METHODS: 6 femoral heads, obtained during arthroplasty surgery for femoral neck fracture, underwent micro-computed tomography (microCT) scanning at 30 µm, and bone volume ratio (BV/TV), trabecular thickness, structural model index, connection density, and degree of anisotropy for volumes of interest throughout the head were derived. A further 15 femoral heads underwent mechanical testing of compressive failure stress of cubes of trabecular bone from different regions of the head. RESULTS: The greatest density and trabecular thickness was found in the central core that extended from the medial calcar to the physeal scar. This region also correlated with the greatest degree of anisotropy and proportion of plate-like trabeculae. In the epiphyseal region, the trabeculae were organized radially from the physeal scar. The weakest area was found at the apex and peripheral areas of the head. The strongest region was at the center of the head. INTERPRETATION: The center of the femoral head contained the strongest trabecular bone, with the thickest, most dense trabeculae. The apical region was weaker. From an anatomical and mechanical point of view, implants that achieve fixation in or below this central core may achieve the most stable fixation during fracture healing.

Shoulder exostoses in hereditary multiple exostoses: probability of surgery and malignant change.

HYPOTHESIS: Patients with hereditary multiple exostoses (HME) in association with palpable shoulder exostoses are more severely affected by their disease. MATERIALS AND METHODS: From a prospective database of 78 families with HME identified, 172 patients were identified. Demographic details, deformity, functional scores, standing height, number of exostoses, site, exostosin genotype (EXT1 and EXT2), surgical excision, and malignant change were recorded. Nonparametric tests were used to compare patients with and without shoulder exostoses (clavicle, scapula, and humerus). RESULTS: There were 5361 palpable exostoses, of which 14% were of the shoulder and were present in 145 patients (84.3%). There was a younger mean age (26.8 vs 37.9 years) and a male predominance in those individuals with shoulder exostoses (P = .0005). Patients with shoulder exostoses had significantly worse disease (P < .05). EXT1 mutations were more commonly observed in those with shoulder exostoses (odds ratio [OR], 20.6; 95% confidence interval [CI], 11.2-28.5; P = .001). The likelihood of surgical excision was greater in those with shoulder exostoses (OR, 2.8) and highest for scapular exostoses (OR, 3.7). Risk factors for surgical excision of shoulder exostoses were younger age (P = .03) and male gender (P < .008). Seven chondrosarcomas occurred, 2 scapular and a proximal humeral. The probability of malignant change of was highest for palpable scapular exostoses relative to any other anatomic site (OR, 12.3; P = .05). CONCLUSION: Shoulder exostoses have a male predominance, and patients are more likely to have an EXT1 mutation. The presence of shoulder exostoses could serve as a tool to identify those individuals at high probability of malignant change. DISCUSSION: The existence of shoulder exostoses identifies those individuals with a high probability of having an EXT1 genotype (OR 20.6, 94.4% sensitivity, 84.8% positive predictive value), which is associated with sarcomatous change.

Bizarre parosteal osteochondromatous proliferation of the metatarsal: a pediatric case report and archival review.

UNLABELLED: Bizarre parosteal osteochondromatous proliferations (BPOP), also known as Nora's lesions, are rare tumors occurring most commonly in the hands and feet. They are benign and rarely exhibit radiological evidence of cortical invasion. We report a case of BPOP showing atypical magnetic resonance imaging features that are inconsistent with BPOP and having a novel chromosomal aberration. We also review the BPOP cases in our regional benign bone tumor database. LEVEL OF CLINICAL EVIDENCE: 4.

Chondrosarcoma transformation in hereditary multiple exostoses: A systematic review and clinical and cost-effectiveness of a proposed screening model.

BACKGROUND: The most serious complication of hereditary multiple exostoses(HME) is chondrosarcoma transformation. Numerous authors have suggested that screening might allow early chondrosarcoma detection. However, literature-quoted incidences of malignant transformation are highly variable. METHODS: A systematic review of malignant transformation by sex, exostosin-1 mutation(EXT1), age and site was conducted searching Medline, Embase and CINHAL. Three HME screening strategies were then developed and compared using cost per life-year gained and incremental cost-effectiveness ratio (ICER). RESULTS: Systematic review: 18 papers with 852 chondrosarcomas were identified. The incidence of chondrosarcoma transformation averaged 4%, 75.2% occurring between ages 20-40 and 56.2% at the pelvis and proximal femur. Screening model: In the general HME population, plain radiographs provided cost per life-year gain of £19,013 compared to £53,392 in MRIs. ICER in MRIs compared to X-rays was £80,218. However, for every generation of HME patients screened over 20 years, X-ray radiation induced 0.65 cancers. Psychological effects of false-positives were marginal. Screening only higher-risk groups (males or EXT1) reduced cost but benefited fewer patients. CONCLUSIONS: Our results suggest that annual MRI screening for all HME patients between age 20-40 may be of value. However, the extent of anatomical imaging is subject to debate; it is possible that focused imaging protocols which scan from cervical spine to proximal femur may improve cost-effectiveness.

Determination of the mutation spectrum of the EXT1/EXT2 genes in British Caucasian patients with multiple osteochondromas, and exclusion of six candidate genes in EXT negative cases.

We describe here the spectrum and distribution of mutations in the EXT1 and EXT2 genes in the largest reported British Caucasian multiple osteochondromas (MO) population. Furthermore, we report for the first time the screening of the EXT1 and EXT2 promoters, 5'UTRs, and 3'UTRs, and exclude six potential MO candidate genes in individuals without a detectable mutation within the coding region of EXT1 and EXT2. The coding exons of EXT1 and EXT2 were screened in 72 unrelated probands affected with MO. Forty-six different mutations were identified in 56 probands, of which 29 were novel. Mutation in the EXT1 and EXT2 genes each accounted for 50% of the mutations identified. Of the 72 probands, 42 were of British Caucasian descent, which when added to the 41 British Caucasian families previously reported from our total cohort, gave a total of 83 families. This cohort's proportional frequency for EXT1/EXT2 mutation was 53%/47%. We also validated the technique of high-resolution melting analysis in a blind study using 27 unique EXT1 or EXT2 mutations. This technique was found to be sensitive with a detection rate of 100% regarding heterozygote detection for EXT mutation scanning. Furthermore, this technique has a very high throughput and is very cost-effective.

Sarcoma excision and pattern of complicating sensory neuropathy.

A potential complication of sarcoma excision surgery is a sensory neurological dysfunction around the surgical scar. This study utilised both objective and subjective sensation assessment modalities, to evaluate 22 patients after sarcoma surgery, for a sensory deficit. 93% had an objective sensory deficit. Light touch is less likely to be damaged than pinprick sensation, and two-point discrimination is significantly reduced around the scar. Results also show that an increased scar size leads to an increased light touch and pinprick deficit and that two-point discriminatory ability around the scar improves as time after surgery elapses. 91% had a subjective deficit, most likely tingling or pain, and numbness was most probable with lower limb sarcomas. Results also demonstrated that there were no significant relationships between any specific subjective and objective deficits. In conclusion, sensory disturbance after sarcoma surgery is common and debilitating. Efforts to minimize scar length are paramount in the prevention of sensory deficit. Sensation may also recover to an extent; thus, sensory reeducation techniques must become an integral aspect of management plans. Finally to obtain a comprehensive assessment of sensory function, both objective and subjective assessment techniques must be utilised.

Subacromial impingement syndrome in a patient with hereditary multiple exostosis: a case report.

BACKGROUND: Hereditary multiple exostosis (HME) is characterised by multiple osteochondromas that are distributed throughout the skeleton, invariably involving the shoulder girdle. Tumours within the subacromial space can cause secondary irritation of the rotator cuff and result in subacromial impingement syndrome. CASE PRESENTATION: We describe a 19 year old female patient with HME who presented with subacromial impingement syndrome secondary to a benign exostosis originating from the spine of the scapular and projecting into the subacromial space. CONCLUSION: The unique aspects of this report was that the origin of the exostosis, which was not observed on early standard radiographs of the shoulder, and the use of arthroscopic excision of the exostosis. Hence we believe a low threshold for additional imaging, such as a magnetic resonance imaging, should be considered for patients with HME with subacromial impingement syndrome to ensure a symptomatic exostosis is not neglected. Arthroscopic excision of a benign subacromial exostosis is effective, offering a minimally invasive approach with relief of the patient's symptoms.

Skeletal growth patterns in hereditary multiple exostoses: a natural history.

Hereditary multiple exostoses (HME) is a commonly inherited musculoskeletal condition and is associated with a diminished stature. We demonstrated that adults with HME were significantly shorter when compared with a control group (P<0.001); preadolescents, however, were significantly taller than predicted (P=0.01). This was reflected by their height centile; 58% of the adults were under the 25th centile, whereas 53% of the preadolescence group were above the 75th centile. Stature was more severely affected in patients with an EXT1 mutation (P=0.008). This study illustrates a novel age-related growth pattern associated with HME, which is also affected by genotype.

Malignant lesions are rare in medial third of the clavicle in children: the European Juvenile Medial End of Clavicle Tumour study.

Condensing osteitis is a condition presenting to all paediatric orthopaedic services, but the prevalence of the condition and optimal management is difficult to determine from the literature. Many case reports in the orthopaedic literature describe biopsy to exclude malignancy as mandatory, whereas expert radiological opinion has suggested that lesions can be classified as typical of sclerosing osteitis on imaging alone. The aim of this study was to calculate an accurate incidence of malignancy at the medial end of the clavicle in children based on data held by national and regional cancer registries in Europe. In addition, this study determined the published success of biopsy in identifying a causative organism. The investigators wrote to 173 European national or regional cancer registries requesting the number of malignant lesions at the medial end of the clavicle in those less than 19 years of age, how long the registry had been in existence and the size of the population served. A literature review was conducted of Medline and Pubmed using the terms, 'condensing osteitis,' 'chronic recurrent multiostotic osteomyelitis,' 'acute osteomyelitis,' 'chronic osteomyelitis clavicle,' 'sclerosing osteitis' and 'sclerosing osteomyelitis' and refined to those regarding the clavicle. The incidence of malignancy at the medial end of the clavicle was found to be extremely low (one case every 275 child-years at risk). In addition, biopsy rarely identified a causative organism with only two of 89 biopsies being positive. We suggest that for a chronic nonmalignant process in which clinical features are typical, serial imaging with follow-up is sufficient although timely biopsy would be recommended when doubt exists.

Sarcoidosis Presenting as a Proximal Phalangeal Bony Swelling-a Case and Review of the Literature.

We report a case of a 27-year-old man who presented with a progressive painful swelling at the base of his left index finger, with radiographs and a computed tomography scan revealing a lytic lesion of the proximal phalanx. Following further investigation, the patient underwent a bone biopsy that revealed a florid noncaseating granulomatous chronic inflammatory infiltrate, compatible with sarcoidosis. Osseous sarcoidosis of the hand is uncommon and, in the absence of significant systemic disease, is rarely the primary presenting feature. Early diagnosis and treatment of such undetermined bone pathology, via referral to a regional musculoskeletal tumor service, can prevent significant future complications.

An age- and sex-matched comparative study on both-bone diaphyseal paediatric forearm fracture.

PURPOSE: Intramedullary (IM) nailing and plating are recognised fixation methods for both-bone midshaft forearm fractures. Although both methods are effective, IM nailing has recently been the accepted operative treatment for the paediatric population. The aim of the study was to compare the differences in the radiographic and functional outcomes of an age- and sex-matched cohort of children following treatment by IM fixation or plate fixation with screws for an unstable both-bone diaphyseal fracture. METHODS: A retrospective study was conducted and 17 age- and sex-matched pairs of patients returned for a research review clinic. The average age of our patients was 11.6 years at follow up, with 11 boys and six girls in each group. The mean follow up was similar in both groups (IM 31.5 months, plating 31.8 months). RESULTS: Plating and IM nailing result in good or excellent functional and radiological outcomes. Radiographs at the review clinic showed complete healing in the plating group, with reconstitution of the radial bow. Three patients in the IM group did not regain the natural radial bow radiographically. There were no significant differences between both groups for maximum radial bow and its location (P > 0.05). However, the maximum radial bow was significantly different from normative values in both groups (P = 0.003 plate, P = 0.005 IM). No non-union or malunion was observed. There were no significant differences in the loss of forearm motion and grip strength between both groups. There was no difference in the Pediatric Orthopaedic Society of North America (POSNA) scores between both groups. The plating group had a significantly worse Manchester scar score than the IM group (P = 0.012). One major complication was observed in each group: osteomyelitis for IM fixation and ulnar never palsy for plating. CONCLUSION: Our study suggests that functional outcome is likely to be equivalent, regardless of which method of internal fixation is used.

Pretibial subcutaneous soft tissue lesions presenting to a paediatric orthopaedic clinic.

Pretibial swellings in children usually represent erythema nodosum which exhibits characteristic skin changes. Three cases of pretibial subcutaneous lesions are presented which had no skin involvement or bony abnormalities on plain radiographs. At initial presentation, concerns of malignancy were raised but these lesions were ultimately diagnosed as granuloma annulare, fat necrosis and subcutaneous infection. In combination with clinical assessment and plain radiographs, MRI proved invaluable in reaching diagnosis and excluding neoplasia. Biopsy was only required in one case in this series after MRI. A tissue diagnosis, however, remains mandatory if there is any doubt over the nature of such lesions.

The association between ulnar length and forearm movement in patients with multiple osteochondromas.

PURPOSE: To determine the relationship between the length of the ulna as a proportion of height (proportional ulnar length [PUL]), forearm and wrist ranges of motion, and degree of observable deformity in people with hereditary multiple exostoses. METHODS: One hundred forty-two people with hereditary multiple exostoses were examined; 35 were under the age of 15 years and therefore were presumed to be skeletally immature. Elbow, forearm, and wrist motion were measured, and the radius and ulna were palpated for osteochondromas. Ulnar length was estimated as a proportion of height (PUL) in skeletally immature subjects. The relationships between total active motion, number of palpable osteochondromas, and proportional length were examined for one randomly selected limb from each subject. RESULTS: A negative correlation was found between the number of palpable osteochondromas and range of forearm rotation. The degree of forearm motion in those under the age of 15 years was directly related to PUL and indirectly related to the number of palpable osteochondromas. Children whose PUL is within the normal range have a normal range of motion. CONCLUSIONS: In a child with hereditary multiple exostoses affecting the forearm, the PUL is associated with the range of movement and deformity, and it can be a useful adjunct in deciding the appropriate management.

The rate of skeletal maturation in the Scottish population: a comparison across 25 years (1980-2005).

BACKGROUND: A retrospective, cross-sectional study was performed to compare the rates of skeletal maturation in Scottish populations 25 years apart (1980-2005). METHODS: Two cohorts of sequentially radiographs were identified from patients at a Scottish pediatric hospital in 1980 and 2005. All radiographs were performed after trauma. The bone age was measured according to the Carpal scoring system of the Tanner-Whitehouse 2 method. RESULTS: One hundred four and 103 radiographs were included from 1980 and 2005, respectively. The radiographs from 2005 showed the children to have achieved older bone ages for given chronological ages than in 1980 (P < 0.0001). CONCLUSIONS: The findings have an important implication for the management of children with limb-length discrepancy. Children may not have the growth potential anticipated by chronological age; this will affect the timing of surgery and medical treatment of abnormalities of puberty and stature.