The Morquio syndrome is a rare disorder which presents with a number of musculoskeletal problems. The literature describing total knee replacement in these patients is sparse. We describe the management of a patient with bilateral instability and pain in the knees using bilateral constrained knee replacements, and followed up for five years with pre- and postoperative knee scores. We highlight the difficulties encountered and discuss the end results.

The Ponseti and French taping methods have reduced the incidence of major surgery in congenital idiopathic clubfoot but incur a significant burden of care, including heel-cord tenotomy. We developed a non-operative regime to reduce treatment intensity without affecting outcome. We treated 402 primary idiopathic clubfeet in patients aged < three months who presented between September 1991 and August 2008. Their Harrold and Walker grades were 6.0% mild, 25.6% moderate and 68.4% severe. All underwent a dynamic outpatient taping regime over five weeks based on Ponseti manipulation, modified Jones strapping and home exercises. Feet with residual equinus (six feet, 1.5%) or relapse within six months (83 feet, 20.9%) underwent one to three additional tapings. Correction was maintained with below-knee splints, exercises and shoes. The clinical outcome at three years of age (385 feet, 95.8% follow-up) showed that taping alone corrected 357 feet (92.7%, ‘good’). Late relapses or failure of taping required limited posterior release in 20 feet (5.2%, ‘fair’) or posteromedial release in eight feet (2.1%, ‘poor’). The long-term (> 10 years) outcomes in 44 feet (23.8% follow-up) were assessed by the Laaveg–Ponseti method as excellent (23 feet, 52.3%), good (17 feet, 38.6%), fair (three feet, 6.8%) or poor (one foot, 2.3%). These compare favourably with published long-term results of the Ponseti or French methods. This dynamic taping regime is a simple non-operative method that delivers improved medium-term and promising long-term results.

Cite this article: Bone Joint J 2013;95-B:271–8.

The credibility and creativity of an author may be gauged by the number of scientific papers he or she has published, as well as the frequency of citations of a particular paper reflecting the impact of the data on the area of practice. The object of this study was to identify and analyse the qualities of the top 100 cited papers in orthopaedic surgery. The database of the Science Citation Index of the Institute for Scientific Information (1945 to 2008) was used. A total of 1490 papers were cited more than 100 times, with the top 100 being subjected to further analysis. The majority originated in the United States, followed by the United Kingdom. The top 100 papers were published in seven specific orthopaedic journals.

Analysis of the most-cited orthopaedic papers allows us a unique insight into the qualitites, characteristics and clinical innovations required for a paper to attain ‘classic’ status.

We present a retrospective review of 167 patients aged 18 years and under who were treated for chronic haematogenous osteomyelitis at our elective orthopaedic hospital in Malawi over a period of four years. The median age at presentation was eight years (1 to 18). There were 239 hospital admissions for treatment during the period of the study. In 117 patients one admission was necessary, in 35 two, and in 15 more than two.

A surgical strategy of infection control followed by reconstruction and stabilisation was employed, based on the Beit CURE radiological classification of chronic haematogenous osteomyelitis as a guide to treatment. At a minimum follow-up of one year after the end of the study none of the patients had returned to our hospital with recurrent infection.

A total of 350 operations were performed on the 167 patients. This represented 6.7% of all children’s operations performed in our hospital during this period. One operation only was required in 110 patients and none required more than three. Below-knee amputation was performed in two patients with chronic calcaneal osteomyelitis as the best surgical option for function. The most common organism cultured from operative specimens was Staphylococcus aureus, and the tibia was the bone most commonly affected. Polyostotic osteomyelitis occurred in four patients. We believe this is the largest reported series of patients treated for chronic haematogenous osteomyelitis.

We compared the clinical, radiological and quality-of-life outcomes between hybrid and total pedicle screw instrumentation in patients undergoing surgery for neuromuscular scoliosis. A matched comparison using prospectively collected data was undertaken. A total of 66 patients underwent posterior or anteroposterior correction and fusion with hybrid (n = 33, mean age at surgery 15.8 years (9.10 to 19.6)) or total pedicle screw instrumentation (n = 33, mean age 14.7 years (7.0 to 20.7)) with a minimum follow-up of two years. The major curve pre-operatively was a mean of 87° (sd 29, 25° to 141°) and 81° (sd 18, 47° to 116°) in the hybrid and total pedicle screw groups, respectively (p = 0.29) and at a minimum of two years it was 33° (sd 20; 2° to 87°) and 20° (sd 12; 1° to 55°), respectively (p = 0.0016). The mean correction of the major curve was 59% (41% to 88%) in the hybrid and 75% (43% to 99%) in the total pedicle screw groups at two-year follow-up (p = 0.0011). The mean operating time was 7.45 hours (sd 2.18) and 6.04 hours (sd 1.71) in the hybrid and total pedicle screw groups, respectively (p = 0.001), and the mean intra-operative blood loss was 3760 ml (sd 2790) and 1785 ml (sd 1110), respectively (p = 0.001).

Total pedicle screw instrumentation provided shorter operating times, less blood loss and better correction of the major curve compared with hybrid constructs in patients undergoing surgery for neuromuscular scoliosis.

The outcome of 56 children (61 shoulders) treated surgically at the Rizzoli Institute between April 1975 and June 2010 for congenital elevation of the scapula is reported. There were 31 girls and 25 boys with a mean age at surgery of 6.4 years (2 to 15). The deformity involved the right shoulder in 20 cases, the left in 31 and was bilateral in five. The degree of the deformity was graded clinically and radiologically according to the classifications of Cavendish and Rigault, respectively. All patients underwent a modified Green procedure combined, in selected cases, with resection of the superomedial portion of the scapula and excision of any omovertebral connection. After a mean follow-up of 10.9 years (1 to 29.3), there was cosmetic improvement by at least one Cavendish grade in 54 shoulders (88.5%). The mean abduction of the shoulder improved from 92° (50° to 155°) to 112° (90° to 170°) and the mean flexion improved from 121° (80° to 160°) to 155° (120° to 175°). The unsatisfactory cosmetic result in seven shoulders was due to coexistent scoliosis in two cases and insufficient reduction of the scapular elevation in the other five. An incomplete upper brachial plexus palsy occurred post-operatively in three patients but resolved within seven months.

We suggest that a modified Green procedure combined with resection of the superomedial portion of the scapula provides good cosmetic and functional results in patients with Sprengel’s shoulder.

We present our early experience of arthroscopic reduction of the dislocated hip in very young infants with developmental dysplasia of the hip (DDH).

Eight dislocated hips, which had failed attempts at closed reduction, were treated by arthroscopy of the hip in five children with a mean age of 5.8 months (4 to 7). A two-portal technique was used, with a medial sub-adductor portal for a 2.7 mm cannulated system with a 70° arthroscope and an anterolateral portal for the instruments. Following evaluation of the key intra-articular structures, the hypertrophic ligamentum teres and acetabular pulvinar were resected, and a limited release of the capsule was performed prior to reduction of the hip. All hips were reduced by a single arthroscopic procedure, the reduction being confirmed on MRI scan. None of the hips had an inverted labrum. The greatest obstacle to reduction was a constriction of the capsule. At a mean follow-up of 13.2 months (9 to 24), all eight hips remained stable. Three developed avascular necrosis. The mean acetabular index decreased from 35.5° (30° to 40°) pre-operatively to 23.3° (17° to 28°).

This study demonstrates that arthroscopic reduction is feasible using two standardised portals. Longer follow-up studies are necessary to evaluate the functional results.

Between 2005 and 2010 ten consecutive children with high-energy open diaphyseal tibial fractures were treated by early reduction and application of a programmable circular external fixator. They were all male with a mean age of 11.5 years (5.2 to 15.4), and they were followed for a mean of 34.5 months (6 to 77). Full weight-bearing was allowed immediately post-operatively. The mean time from application to removal of the frame was 16 weeks (12 to 21). The mean deformity following removal of the frame was 0.15° (0° to 1.5°) of coronal angulation, 0.2° (0° to 2°) sagittal angulation, 1.1 mm (0 to 10) coronal translation, and 0.5 mm (0 to 2) sagittal translation. All patients achieved consolidated bony union and satisfactory wound healing. There were no cases of delayed or nonunion, compartment syndrome or neurovascular injury. Four patients had a mild superficial pin site infection; all settled with a single course of oral antibiotics. No patient had a deep infection or re-fracture following removal of the frame. The time to union was comparable with, or better than, other published methods of stabilisation for these injuries. The stable fixator configuration not only facilitates management of the accompanying soft-tissue injury but enables anatomical post-injury alignment, which is important in view of the limited remodelling potential of the tibia in children aged > ten years. Where appropriate expertise exists, we recommend this technique for the management of high-energy open tibial fractures in children.

This nationwide prospective study was designed to determine prognostic factors and evaluate the outcome of different treatments of Perthes’ disease.

A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’ disease over a period of five years and 425 patients were reported and followed for five years. Of these, 368 with unilateral disease were included in the present study. The hips were classified radiologically according to a modified two-group Catterall classification and the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up, when a modified three-group Stulberg classification was used as a radiological outcome measure. For patients over six years of age at diagnosis and with more than 50% necrosis of the femoral head (152 patients), the surgeons at the different hospitals had chosen one of three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were available for the five-year follow-up.

The strongest predictor of outcome was femoral head involvement of more or less than 50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95% CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteotomy gave a significantly better outcome than orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in outcome after any of the treatments in children under six years (p = 0.73).

We recommend proximal femoral varus osteotomy in children aged six years and over at the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction orthosis should be abandoned in Perthes’ disease.

Elastic stable intramedullary nailing (ESIN) is generally acknowledged to be the treatment of choice for displaced diaphyseal femoral fractures in children over the age of three years, although complication rates of up to 50% are described. Pre-bending the nails is recommended, but there are no published data to support this. Using synthetic bones and a standardised simulated fracture, we performed biomechanical testing to determine the influence on the stability of the fracture of pre-bending the nails before implantation. Standard ESIN was performed on 24 synthetic femoral models with a spiral fracture. In eight cases the nails were inserted without any pre-bending, in a further eight cases they were pre-bent to 30° and in the last group of eight cases they were pre-bent to 60°. Mechanical testing revealed that pre-bending to 60° produced a significant increase in the stiffness or stability of the fracture. Pre-bending to 60° showed a significant positive influence on the stiffness compared with unbent nails. Pre-bending to 30° improved stiffness only slightly.

These findings validate the recommendations for pre-bending, but the degree of pre-bend should exceed 30°. Adopting higher degrees of pre-bending should improve stability in spiral fractures and reduce the complications of varus deformity and shortening.

We undertook a prospective study to evaluate the prognostic significance of the serum levels of vascular endothelial growth factor (VEGF) in predicting the survival of patients with osteosarcoma. The levels were measured by an enzyme-linked immunosorbent assay in 15 patients with osteosarcoma before commencing treatment. The patients were divided into two groups, with a high or a low serum VEGF level, and the incidence of metastases and overall survival rate were compared.

No significant relationship was observed between the serum VEGF levels and gender, age, the size of the tumour or the response to pre-operative chemotherapy. Patients with a serum VEGF > 1000 pg/ml had significantly worse survival than those with a level < 1000 pg/ml (p = 0.002). The serum VEGF level may be useful in predicting the prognosis for survival in patients with osteosarcoma.

Fractures of the proximal humerus with concomitant vascular injury are rare in children. We describe the presentation, diagnosis, and treatment of a fracture of the proximal humerus in association with an axillary artery injury in a child.

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.

Iliacus haematoma is a relatively rare condition, which may cause a local compressive neuropathy. It is usually diagnosed in adults with haemophilia or those on anticoagulation treatment and may occur after trauma. We present the case of a healthy 15-year-old boy with a femoral neuropathy due to an iliacus haematoma which resolved following conservative treatment.

Between July 1994 and June 2004, 60 patients with 76 slipped upper femoral epiphyses were managed within the adult trauma service of three hospitals. Treatment was by a single cannulated screw.

Of these cases, 53 were unilateral, in 17 of which uncomplicated prophylactic fixation of the contralateral hip was performed. Of the other 36 cases, nine presented with a subsequent slip despite ongoing out-patient care. The subsequent slip was unpredictable in timing and unrelated to the age at the initial slip. It was more often unstable and in one case avascular necrosis developed. The overall rate of avascular necrosis, although in accordance with the literature, was 60% in acute unstable slips with a slip angle greater than 40°.

In our experience, prophylactic fixation was safer than continued observation of the contralateral hip.

This brief annotation summarises the particular contributions made by the annual Edinburgh International Trauma Symposium in various areas of research into aspects of orthopaedic trauma and the management of acutely injured patients, during the 25 years since its establishment.

Progressive angular deformity of an extremity due to differential physeal arrest is the most common late orthopaedic sequela following meningococcal septicaemia in childhood. A total of ten patients (14 ankles) with distal tibial physeal arrest as a consequence of meningococcal septicaemia have been reviewed. Radiological analysis of their ankles has demonstrated a distinct pattern of deformity. In 13 of 14 cases the distal fibular physis was unaffected and continued distal fibular growth contributed to a varus deformity. We recommend that surgical management should take account of this consistent finding during the correction of these deformities.