We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities.

There are many types of treatment used to manage the frozen shoulder, but there is no consensus on how best to manage patients with this painful and debilitating condition. We conducted a review of the evidence of the effectiveness of interventions used to manage primary frozen shoulder using the Cochrane Database of Systematic Reviews, the Database of Abstracts of Reviews of Effects, the Physiotherapy Evidence Database, MEDLINE and EMBASE without language or date restrictions up to April 2009. Two authors independently applied selection criteria and assessed the quality of systematic reviews using the Assessment of Multiple Systematic Reviews (AMSTAR) tool. Data were synthesised narratively, with emphasis placed on assessing the quality of evidence.

In total, 758 titles and abstracts were identified and screened, which resulted in the inclusion of 11 systematic reviews. Although these met most of the AMSTAR quality criteria, there was insufficient evidence to draw firm conclusions about the effectiveness of treatments commonly used to manage a frozen shoulder. This was mostly due to poor methodological quality and small sample size in primary studies included in the reviews. We found no reviews evaluating surgical interventions.

More rigorous randomised trials are needed to evaluate the treatments used for frozen shoulder.

We aimed to identify the incidence, outcome and prognostic factors associated with spindle cell sarcomas of bone (SCSB). We studied 196 patients with a primary non-metastatic tumour treated with the intent to cure. The results were compared with those of osteosarcoma patients treated at our hospital during the same period. The overall incidence of SCSB was 7.8% of all patients with a primary bone sarcoma. The five- and ten-year survival rates were 67.0% and 60.0%, respectively, which were better than those of patients with osteosarcoma treated over the same period. All histological subtypes had similar outcomes. On univariate analysis, factors that were significantly associated with decreased survival were age > 40 years, size > 8 cm, the presence of a pathological fracture, amputation, involved margins and a poor response to pre-operative chemotherapy. Multivariate analyses showed that age > 65 years, amputation and involved margins were all statistically significant prognostic factors. Involved margins and poor response to pre-operative chemotherapy were associated with an increased risk of local recurrence. SCSB has a better prognosis than osteosarcoma when matched for age. Most prognostic factors for osteosarcoma also seem to apply to SCSB. Patients with SCSB should be treated in the same way as patients of the same age with osteosarcoma.

We performed a systematic review of the literature to evaluate the use and interpretation of generic and disease-specific functional outcome instruments in the reporting of outcome after the surgical treatment of disruptions of the pelvic ring. A total of 28 papers met our inclusion criteria, with eight reporting only generic outcome instruments, 13 reporting only pelvis-specific outcome instruments, and six reporting both. The Short-Form 36 (SF-36) was by far the most commonly used generic outcome instrument, used in 12 papers, with widely variable reporting of scores. The pelvis-specific outcome instruments were used in 19 studies; the Majeed score in ten, Iowa pelvic score in six, Hannover pelvic score in two and the Orlando pelvic score in one. Four sets of authors, all testing construct validity based on correlation with the SF-36, performed psychometric testing of three pelvis-specific instruments (Majeed, IPS and Orlando scores). No testing of responsiveness, content validity, criterion validity, internal consistency or reproducibility was performed.

The existing literature in this area is inadequate to inform surgeons or patients in a meaningful way about the functional outcomes of these fractures after fixation.

Patients with infected arthroplasties are normally treated with a two-stage exchange procedure using polymethylmethacrylate bone cement spacers impregnated with antibiotics. However, spacers may act as a foreign body to which micro-organisms may adhere and grow. In this study it was hypothesised that subclinical infection may be diagnosed with sonication of the surface biofilm of the spacer. The aims were to assess the presence of subclinical infection through sonication of the spacer at the time of a second-stage procedure, and to determine the relationship between subclinical infection and the clinical outcome. Of 55 patients studied, 11 (20%) were diagnosed with subclinical infection. At a mean follow-up of 12 months (interquartile range 6 to 18), clinical failure was found in 18 (32.7%) patients. Of the patients previously diagnosed with subclinical infection, 63% (7 of 11) had failed compared with 25% (11 of 44) of those without subclinical infection (odds ratio 5.25, 95% confidence interval 1.29 to 21.4, p = 0.021). Sonication of the biofilm of the surface of the spacer is useful in order to exclude subclinical infection and therefore contributes to improving the outcome after two-stage procedures.

The Canadian Orthopaedic Trauma Society was started in an endeavour to answer the difficult problem of obtaining enough patients to perform top-quality research into fractures. By maintaining a high standard, including randomised study design, inclusivity, open discussion among surgeons and excellent long-term follow-up, this group has become a leader in the orthopaedic research community. This annotation describes the short history, important components and spirit necessary to build a research community or team which will function well despite the difficult research environment facing individual surgeons.

We reviewed the clinical and radiological results of six patients who had sustained traumatic separation of the distal epiphysis of the humerus at birth.

The correct diagnosis was made from plain radiographs and often supplemented with ultrasonography, MRI and arthrography. An orthopaedic surgeon saw two patients within two days of birth, and the other four were seen at between nine and 30 days. The two neonates underwent unsuccessful attempts at closed reduction. In the remaining patients, seen after the age of eight days, no attempt at reduction was made. All six displaced fractures were immobilised in a cast with the elbow at 90° of flexion and the forearm pronated.

When seen at a mean of 58 months (16 to 120) after injury, the clinical and radiological results were excellent in five patients, with complete realignment of the injury. In one patient the forearm lay in slightly reduced valgus with the elbow in full extension.

Traumatic separation of the distal epiphysis of the humerus may be missed on the maternity wards and not diagnosed until after discharge from hospital. However, even when no attempt is made to reduce the displaced epiphysis, a good clinical result can be expected.

Our aim was to evaluate the effect of adding inhibitory casting to the treatment of young children with cerebral palsy who received injections of botulinum neurotoxin A (BoNT-A) to gastrocnemius for equinus gait. Of the 20 patients in the series, 11 in group A had inhibitory casts applied on the day of the first set of BoNT-A injections and nine in group B did not have casting. Both groups received another BoNT-A injection four months later. The patients were followed for eight months and examined at five intervals.

Both groups showed significant improvement in gait parameters and function (p < 0.0001) and selective motor control (p = 0.041, − 0.036) throughout the study. Group A had significantly better passive dorsiflexion of the ankle (p = 0.029), observational gait score (p = 0.006) and selective motor control (p = 0.036). We conclude that the addition of inhibitory casting enhances and prolongs the results of treatment and mainly influences the passive range of movement, while BoNT-A mostly influences the dynamic motion. The second injection further improved the results of most parameters. The gross motor function measure, the selective motor control test and the modified Tardieu scale correlated well with the results of treatment.

We recommend the use of inhibitory casting whenever augmentation of the effect of treatment with BoNT-A is needed.

The optimal method for the management of neglected traumatic bifacetal dislocation of the subaxial cervical spine has not been established. We treated four patients in whom the mean delay between injury and presentation was four months (1 to 5). There were two dislocations at the C5-6 level and one each at C4-5 and C3-4. The mean age of the patients was 48.2 years (27 to 60). Each patient presented with neck pain and restricted movement of the cervical spine. Three of the four had a myelopathy.

We carried out a two-stage procedure under the same anaesthetic. First, a posterior soft-tissue release and partial facetectomy were undertaken. This allowed partial reduction of the dislocation which was then supplemented by interspinous wiring and corticocancellous graft. Next, through an anterior approach, discectomy, tricortical bone grafting and anterior cervical plating were carried out.

All the patients achieved a nearly anatomical reduction and sagittal alignment. The mean follow-up was 2.6 years (1 to 4). The myelopathy settled completely in the three patients who had a pre-operative neurological deficit. There was no graft dislodgement or graft-related problems. Bony fusion occurred in all patients and a satisfactory reduction was maintained.

The posteroanterior procedure for neglected traumatic bifacetal dislocation of the subaxial cervical spine is a good method of achieving sagittal alignment with less risk of iatrogenic neurological injury, a reduced operating time, decreased blood loss, and a shorter hospital stay compared with other procedures.

It is unclear whether there is a limit to the amount of distal bone required to support fixation of supracondylar periprosthetic femoral fractures. This retrospective multicentre study evaluated lateral locked plating of periprosthetic supracondylar femoral fractures and compared the results according to extension of the fracture distal with the proximal border of the femoral prosthetic component.

Between 1999 and 2008, 89 patients underwent lateral locked plating of a supracondylar periprosthetic femoral fracture, of whom 61 patients with a mean age of 72 years (42 to 96) comprising 53 women, were available after a minimum follow-up of six months or until fracture healing. Patients were grouped into those with fractures located proximally (28) and those with fractures that extended distal to the proximal border of the femoral component (33).

Delayed healing and nonunion occurred respectively in five (18%) and three (11%) of more proximal fractures, and in two (6%) and five (15%) of the fractures with distal extension (p = 0.23 for delayed healing; p = 0.72 for nonunion, Fisher’s exact test). Four construct failures (14%) occurred in more proximal fractures, and three (9%) in fractures with distal extension (p = 0.51). Of the two deep infections that occurred in each group, one resolved after surgical debridement and antibiotics, and one progressed to a nonunion.

Extreme distal periprosthetic supracondylar fractures of the femur are not a contra-indication to lateral locked plating. These fractures can be managed with internal fixation, with predictable results, similar to those seen in more proximal fractures.

A series of 103 acute fractures of the coronoid process of the ulna in 101 patients was reviewed to determine their frequency. The Regan-Morrey classification, treatment, associated injuries, course and outcomes were evaluated. Of the 103 fractures, 34 were type IA, 17 type IB, ten type IIA, 19 type IIB, ten type IIIA and 13 type IIIB. A total of 44 type-I fractures (86%) were treated conservatively, while 22 type-II (76%) and all type-III fractures were managed by operation.

At follow-up at a mean of 3.4 years (1 to 8.9) the range of movement differed significantly between the types of fracture (p = 0.002). Patients with associated injuries had a lower Mayo elbow performance score (p = 0.03), less extension (p = 0.03), more pain (p = 0.007) and less pronosupination (p = 0.004), than those without associated injuries. The presence of a fracture of the radial head had the greatest effect on outcome. An improvement in outcome relative to that of a previous series was noted, perhaps because of more aggressive management and early mobilisation. While not providing complete information about the true details of a fracture and its nature, the Regan-Morrey classification is useful as a broad index of severity and prognosis.

Fractures of the odontoid in children with an open basilar synchondrosis differ from those which occur in older children and adults. We have reviewed the morphology of these fractures and present a classification system for them.

There were four distinct patterns of fracture (types IA to IC and type II) which were distinguished by the site of the fracture, the degree of displacement and the presence or absence of atlantoaxial dislocation. Children with a closed synchondrosis were classified using the system devised by Anderson and D’Alonzo. Those with an open synchondrosis had a comparatively lower incidence of traumatic brain injury, a higher rate of missed diagnosis and a shorter mean stay in hospital. Certain subtypes (type IA and type II) are likely to be missed on plain radiographs and therefore more advanced imaging is recommended. We suggest staged treatment with initial stabilisation in a Halo body jacket and early fusion for those with unstable injuries, severe displacement or neurological involvement.

The first 325 Exeter Universal stems (309 patients) implanted at the originating centre were inserted between March 1988 and February 1990 by a group of surgeons with differing experience. In this report we describe the clinical and radiological results at a mean of 15.7 years (14.7 to 17.3) after operation with no loss to follow-up. There were 97 patients (108 hips) with replacements still in situ and 31 (31 hips) who had undergone a further procedure. With an endpoint of revision for aseptic loosening, the survivorship at 17 years was 100% and 90.4% for the femoral and acetabular component, respectively. The mean Merle D’Aubigné and Postel scores at review were 5.4 (sd 0.97) for pain and 4.5 (sd 1.72) for function. The mean Oxford score was 38.4 (sd 9.8) (0 to 48 worst-to-best scale) and the mean combined Harris pain and function score was 73.2 (sd 16.9). Radiological review showed excellent preservation of bone stock in the proximal femur and no failures of the femoral component.

A consecutive series of 372 patients who underwent surgery for disorders of the rotator cuff involving arthroscopic subacromial decompression and open or arthroscopic repairs of the cuff were prospectively investigated as to the comparability of subjective and objective assessment scores of shoulder function. Assessments were made before operation and at 3, 6, 12, 18 and 24 months after surgery using the Disabilities of the Arm, Shoulder, and Hand score, the Oxford shoulder score and the Constant-Murley score, which was used as a reference. All scores were standardised to a scale of 0 to 100 for comparison. Statistical analysis compared the post-operative course and the mean score for the subjective Disabilities to the Arm, Shoulder and Hand score and Oxford shoulder score, with the objective Constant score at each interval. A strong correlation was evident between both subjective scores and the Constant score. We concluded that both the subjective scores would be useful substitutes for the Constant score, obviating the need for a trained investigator and the specialist equipment required to perform the Constant score.

Endoprosthetic replacement of the proximal femur may be required to treat primary bone tumours or destructive metastases either with impending or established pathological fracture. Modular prostheses are available off the shelf and can be adapted to most reconstructive situations for this purpose. We have assessed the clinical and functional outcome of using the METS (Stanmore Implants Worldwide) modular tumour prosthesis to reconstruct the proximal femur in 100 consecutive patients between 2001 and 2006. We compared the results with the published series for patients managed with modular and custom-made endoprosthetic replacements for the same conditions.

There were 52 males and 48 females with a mean age of 56.3 years (16 to 84) and a mean follow-up of 24.6 months (0 to 60). In 65 patients the procedure was undertaken for metastases, in 25 for a primary bone tumour, and in ten for other malignant conditions. A total of 46 patients presented with a pathological fracture, and 19 presented with failed fixation of a previous pathological fracture. The overall patient survival was 63.6% at one year and 23.1% at five years, and was significantly better for patients with a primary bone tumour than for those with metastatic tumour (82.3% vs 53.3%, respectively at one year (p = 0.003)). There were six early dislocations of which five could be treated by closed reduction. No patient needed revision surgery for dislocation. Revision surgery was required by six (6%) patients, five for pain caused by acetabular wear and one for tumour progression. Amputation was needed in four patients for local recurrence or infection.

The estimated five-year implant survival with revision as the endpoint was 90.7%. The mean Toronto Extremity Salvage score was 61% (51% to 95%). The implant survival and complications resulting from the use of the modular system were comparable to the published series of both custom-made and other modular proximal femoral implants.

We conclude that at intermediate follow-up the modular tumour prosthesis for proximal femur replacement provides versatility, a low incidence of implant-related complications and acceptable function for patients with metastatic tumours, pathological fractures and failed fixation of the proximal femur. It also functions as well as a custom-made endoprosthetic replacement.

Fracture of the atlas is rare in children. We report a case of fracture of the atlas through a synchondrosis of the anterior arch complicated by atlantoaxial rotatory fixation in a four-year-old girl.

We have analysed the pattern of symptoms in patients presenting with synovial sarcoma to identify factors which led to long delays in diagnosis. In 35 children, the early symptoms and the results of clinical and radiological investigation were reviewed, along with the presumed diagnoses. The duration of symptoms was separated into patient delay and doctor delay.

Only half of the patients had one or more of the four clinical findings suggestive of sarcoma according to the guidance of the National Institute for Clinical Excellence at the onset of symptoms. Of the 33 children for whom data were available, 16 (48.5%) presented with a painless mass and in ten (30.3%) no mass was identified. Seven (21.2%) had an unexplained joint contracture. Many had been extensively investigated unsuccessfully. The mean duration of symptoms was 98 weeks (2 to 364), the mean patient delay was 43 weeks (0 to 156) and the mean doctor delay was 50 weeks (0 to 362). The mean number of doctors seen before referral was three (1 to 6) and for 15 patients the diagnosis was obtained after unplanned excision. Tumours around the knee and elbow were associated with a longer duration of symptoms and longer doctor delay compared with those at other sites. Delays did not improve significantly over the period of our study of 21 years, and we were unable to show that delay in diagnosis led to a worse prognosis.

Our findings highlight the variety of symptoms associated with synovial sarcoma and encourage greater awareness of this tumour as a potential diagnosis in childhood.

We report the outcome at a mean of 93 months (73 to 110) of 71 patients with an acute fracture of the scaphoid who were randomised to Herbert screw fixation (35) or below-elbow plaster cast immobilisation (36). These 71 patients represent the majority of a randomised series of 88 patients whose short-term outcome has previously been reported. Those patients available for later review were similar in age, gender and hand dominance.

There was no statistical difference in symptoms and disability as assessed by the mean Patient Evaluation Measure (p = 0.4), or mean Patient-Rated Wrist Evaluation (p = 0.9), the mean range of movement of the wrist (p = 0.4), mean grip strength (p = 0.8), or mean pinch strength (p = 0.4).

Radiographs were available from the final review for 59 patients. Osteoarthritic changes were seen in the scaphotrapezial and radioscaphoid joints in eight (13.5%) and six patients (10.2%), respectively. Three patients had asymptomatic lucency surrounding the screw. One non-operatively treated patient developed nonunion with avascular necrosis. In five patients who were treated non-operatively (16%) there was an abnormal scapholunate angle ( > 60°), but in four of these patients this finding was asymptomatic.

No medium-term difference in function or radiological outcome was identified between the two treatment groups.

Giant-cell tumour of the synovium is known to affect the fingers or toes of adults. It has seldom been described in the spine and rarely in the thoracic vertebrae or in a child. The lesions of giant-cell tumour of the synovium have a classical radiological appearance, but require a high index of suspicion for correct recognition. Unlike giant-cell tumour of the synovium at other well-known sites, spinal lesions lack the characteristic papillary architecture, thereby raising other diagnostic possibilities. We describe a giant-cell tumour of the synovium of the left facet joint of a thoracic vertebra in a nine-year-old girl. The tumour was treated successfully by surgical excision.