Aims. The Precice intramedullary limb-lengthening system has demonstrated significant benefits over external fixation lengthening methods, leading to a paradigm shift in limb lengthening. This study compares outcomes following antegrade and retrograde femoral lengthening in both adolescent and adult patients. Patients and Methods. A retrospective review of prospectively collected data was undertaken of a consecutive series of 107 femoral lengthening operations in 92 patients. In total, 73 antegrade nails and 34 retrograde nails were inserted. Outcome was assessed by the regenerate healing index (HI), hip and knee range of movement (ROM), and the presence of any complications. Results. The mean lengthening was 4.65 cm (1.5 to 8) in the antegrade group and 4.64 cm (1.6 to 8) in the retrograde group. Of the 107 lengthenings, 100 had sufficient datapoints to calculate the mean HI. This was 31.6 days/cm (15 to 108). There was a trend toward a lower (better) HI with an antegrade nail and better outcomes in adolescent patients, but these were not statistically significant. Hip and knee ROM was maintained and/or improved following commencement of femoral lengthening in 44 patients (60%) of antegrade nails and 13 patients (38%) of retrograde nails. In female patients, loss of movement occurred both earlier and following less total length achieved. Minor implant complications included locking bolt migration and in one patient deformity of the nail, but no implant failed to lengthen and there were no deep infections. Three patients had delayed union, five patients required surgical intervention for joint contracture. Conclusion. This study confirms excellent results in femoral lengthening with antegrade and retrograde Precice nails. There is a trend for better healing and less restriction in hip and knee movement following antegrade nails. There are clinical scenarios, that mandate the use of a retrograde nail. However, when these are not present, we recommend the use of antegrade nailing. Cite this article: Bone Joint J 2019;101-B:1168–1176

Aims

The aim of this study was to gain a consensus for best practice of the assessment and management of children with idiopathic toe walking (ITW) in order to provide a benchmark for practitioners and guide the best consistent care.

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

Aims

The aim of this study was to determine the consensus best practice approach for the investigation and management of children (aged 0 to 15 years) in the UK with musculoskeletal infection (including septic arthritis, osteomyelitis, pyomyositis, tenosynovitis, fasciitis, and discitis). This consensus can then be used to ensure consistent, safe care for children in UK hospitals and those elsewhere with similar healthcare systems.

Aims

The aim of this retrospective cohort study was to assess and investigate the safety and efficacy of using a distal tibial osteotomy compared to proximal osteotomy for limb lengthening in children.

Between 1990 and 2001, 24 children aged between 15 months and 11 years presented with late orthopaedic sequelae after meningococcal septicaemia. The median time to presentation was 32 months (12 to 119) after the acute phase of the disease. The reasons for referral included angular deformity, limb-length discrepancy, joint contracture and problems with prosthetic fitting. Angular deformity with or without limb-length discrepancy was the most common presentation. Partial growth arrest was the cause of the angular deformity. Multiple growth-plate involvement occurred in 14 children. The lower limbs were affected much more often than the upper. Twenty-three children underwent operations for realignment of the mechanical axis and limb-length equalisation. In 15 patients with angular deformity around the knee the deformity recurred. As a result we recommend performing a realignment procedure with epiphysiodesis of the remaining growth plate when correcting angular deformities

Aims

With novel promising therapies potentially limiting progression of Dupuytren’s disease (DD), better patient stratification is needed. We aimed to quantify DD development and progression after seven years in a population-based cohort, and to identify factors predictive of disease development or progression.

We describe the treatment of three boys with cavernous lymphangioma of the legs. The suggested guidelines for treatment are extensive surgical resection, although complete resection is usually impossible, and approximately two weeks after the operation aspiration of serous fluid which has accumulated at the operation site, followed by injection of OK-432 (Picibanil). There was pyrexia and local inflammation for several days but the accumulation of serous fluid disappeared after the injection. There were no complications with no recurrence, joint contracture, or pain during a mean follow-up of 48 months (24 to 72). We conclude that an injection of OK-432 after surgical resection of cavernous lymphangioma of the legs in children is an effective treatment

In developed countries, children with cerebral palsy are treated from the time of diagnosis. This is usually not the case in developing countries where such patients often present at an age when it is traditionally believed that if walking has not already commenced, it is unlikely to. This study reports the outcome of the surgical treatment of 85 spastic diplegic patients at a mean of 8.5 years (5 to 12). All presented as untreated non-walkers and had achieved sitting balance by the age of five to six years. They underwent single-event multilevel surgery followed by physiotherapy and orthotic support. For outcome assessment, a modified functional walking scale was used at a mean of 3.5 years (2 to 5) post-operatively. At all levels, static joint contractures had resolved almost completely. All patients improved and became walkers, 18 (21.2%) as exercise, 39 (45.9%) as household and 28 (33%) as community walkers. This study shows that children with cerebral palsy who cannot walk and have not been treated can be helped by single-event multilevel surgery, provided that inclusion criteria are followed and a structural, supervised rehabilitation programme is in place

We treated 13 children with histologically confirmed cystic tuberculosis of bone. Ten had solitary cystic lesions and three had the multicystic form. Signs and symptoms were related mainly to the joint adjacent to the cyst. Most lesions were in the metaphyses of long bones. They were radiolucent, round or oval, and resembled pyogenic infections, aneurysmal and simple bone cysts, cartilaginous tumours or osteoid osteoma. Only two of the children had pulmonary tuberculosis. The Mantoux skin test was negative in four children and the ESR was normal in five. Curettage followed by anti-tuberculosis therapy for one year resulted in good healing, but two children had residual joint contractures. Biopsy should be taken from the cystic area rather than from the synovium when a joint is involved

Arthrogryposis multiplex congenita (AMC) is a rare disease with multiple joint contractures. It is widely believed that bilaterally dislocated hips should not be reduced since movement is satisfactory and open reduction has had poor results. Since 1977 we have performed a new method of open reduction using an extensive anterolateral approach on ten hips in five children with AMC. The mean age at surgery was 31.5 months (17 to 64) and the mean follow-up was 11.8 years (3.8 to 19.5). At the final follow-up all children walked without crutches or canes. Two managed independently, one required a long leg brace and two had short leg braces because of knee and/or foot problems. The clinical results were good in eight hips and fair in two and on the Severin classification seven hips were rated as good (group I or group II). We recommend the extensive anterolateral approach for unilateral or bilateral dislocation of the hip in children with arthrogryposis or developmental dislocation of the hip

Aims

To assess the characteristic clinical features, management, and outcome of patients who present to orthopaedic surgeons with functional dystonia affecting the foot and ankle.

Aims

In the UK, fasciectomy for Dupuytren’s contracture is generally performed under general or regional anaesthetic, with an arm tourniquet and in a hospital setting. We have changed our practice to use local anaesthetic with adrenaline, no arm tourniquet, and perform the surgery in a community setting. We present the outcome of a consecutive series of 30 patients.

Aims

Dupuytren’s contracture is a benign, myoproliferative condition affecting the palmar fascia that results in progressive contractures of the fingers. Despite increased knowledge of the cellular and connective tissue changes involved, neither a cure nor an optimum form of treatment exists. The aim of this systematic review was to summarize the best available evidence on the management of this condition.

1. A series of 705 fractures of the tibia is reviewed, 674 of which were treated conservatively. 2. The factors most conducive to delayed or non-union are initial displacement, comminution, associated soft-tissue wound and infection. The extent to which these are combined in any fracture determines its "personality" and its inherent propensity for union. 3. Eight fracture types are differentiated based on the above "personality rating." The incidence of delayed union or non-union varies from 9 per cent in the most favourable type to 39 per cent in the least favourable. Infection raises the incidence to 60 per cent. Comparative statistics which fail to recognise these differences can be entirely misleading. 4. Continuous traction does not retard union. 5. The results of conservative treatment are analysed with regard to union, deformity, stiff joints and contractures and the conclusion is reached that no case has yet been made out for internal fixation as the method of choice in the treatment of this fracture

Aims

Many authors have reported a shorter treatment time when using trifocal bone transport (TFT) rather than bifocal bone transport (BFT) in the management of long segmental tibial bone defects. However, the difference in the incidence of additional procedures, the true complications, and the final results have not been investigated.

The mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders with clinical manifestations relevant to the orthopaedic surgeon. Our aim was to review the recent advances in their management and the implications for surgical practice.

The current literature about MPSs is summarised, emphasising orthopaedic complications and their management.

Recent advances in the diagnosis and management of MPSs include the recognition of slowly progressive, late presenting subtypes, developments in life-prolonging systemic treatment and potentially new indications for surgical treatment. The outcomes of surgery in these patients are not yet validated and some procedures have a high rate of complications which differ from those in patients who do not have a MPS.

The diagnosis of a MPS should be considered in adolescents or young adults with a previously unrecognised dysplasia of the hip. Surgeons treating patients with a MPS should report their experience and studies should include the assessment of function and quality of life to guide treatment.

Cite this article: Bone Joint J 2017;99-B:1132–9

Aims

This study compared the long-term results following Salter osteotomy and Pemberton acetabuloplasty in children with developmental dysplasia of the hip (DDH). We assessed if there was a greater increase in pelvic height following the Salter osteotomy, and if this had a continued effect on pelvic tilt, lumbar curvature or functional outcomes.

The treatment of infected nonunions is difficult. Antibiotic cement-coated (ACC) rods provide stability as well as delivering antibiotics. We conducted a review of 110 infected nonunions treated with ACC rods. Patients were divided into two groups: group A (67 patients) with an infected arthrodesis, and group B (43 patients) with an infected nonunion in a long bone. In group A, infected arthrodesis, the success rate after the first procedure was 38/67 (57%), 29/67 (43%) required further surgery for either control of infection or non-union. At last follow-up, five patients required amputation, representing a limb salvage rate of 62/67 (93%) overall. In all, 29/67 (43%) presented with a bone defect with a mean size of 6.78 cm (2 to 25). Of those with a bone defect, 13/29 (45%) required further surgery and had a mean size of defect of 7.2 cm (3.5 to 25). The cultures were negative in 17/67 (26%) and the most common organism cultured was methicillin-resistant staphylococcus aureus (MRSA) (23/67, (35%)). In group B, long bones nonunion, the success rate after the first procedure was 26/43 (60%), 17/43 (40%) required further surgery for either control of infection or nonunion. The limb salvage rate at last follow-up was 43/43 (100%). A total of 22/43 (51%) had bone defect with a mean size of 4.7 cm (1.5 to 11.5). Of those patients with a bone defect, 93% required further surgery with a mean size of defect of 5.4 cm (3 to 8.5). The cultures were negative in 10/43 (24%) and the most common organism cultured was MRSA, 15/43 (35%). ACC rods are an effective form of treatment for an infected nonunion, with an acceptable rate of complications.

Cite this article: Bone Joint J 2014; 96-B:1349–54

The aims of this study were to report functional outcomes of salvage procedures for patients with cerebral palsy (CP) who have chronic dislocation of the hip using validated scoring systems, and to compare the results of three surgical techniques.

We reviewed 37 patients retrospectively. The mean age at the time of surgery was 12.2 years (8 to 22) and the mean follow-up was 56 months (24 to 114). Patients were divided into three groups: 14 who underwent proximal femoral resection arthroplasty (PFRA group 1), ten who underwent subtrochanteric valgus osteotomy (SVO group 2), and 13 who underwent subtrochanteric valgus osteotomy with resection of the femoral head (SVO with FHR group 3). All patients were evaluated using the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) and the Pediatric Quality of Life Inventory (PedsQL).

Significant improvements occurred in most CPCHILD and PedsQL subsection scores following surgery in all patients, without significant differences between the groups. There were 12 post-operative complications. Less severe complications were seen in group 1 than in groups 2 and 3.

Salvage surgery appears to provide pain relief in patients with CP who have painful chronic dislocation of the hip. The three salvage procedures produced similar results, however, we recommend the use of PFRA as the complications are less severe.

Take home message: Salvage surgery can be of benefit to patients with CP with chronic painful hip dislocation, but should be limited to selected patients considering complications.

Cite this article: Bone Joint J 2016;98-B:137–43.

Aims

Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution.

We describe 261 peripheral nerve injuries sustained in war by 100 consecutive service men and women injured in Iraq and Afghanistan. Their mean age was 26.5 years (18.1 to 42.6), the median interval between injury and first review was 4.2 months (mean 8.4 months (0.36 to 48.49)) and median follow-up was 28.4 months (mean 20.5 months (1.3 to 64.2)). The nerve lesions were predominantly focal prolonged conduction block/neurapraxia in 116 (45%), axonotmesis in 92 (35%) and neurotmesis in 53 (20%) and were evenly distributed between the upper and the lower limbs. Explosions accounted for 164 (63%): 213 (82%) nerve injuries were associated with open wounds. Two or more main nerves were injured in 70 patients. The ulnar, common peroneal and tibial nerves were most commonly injured. In 69 patients there was a vascular injury, fracture, or both at the level of the nerve lesion. Major tissue loss was present in 50 patients: amputation of at least one limb was needed in 18. A total of 36 patients continued in severe neuropathic pain.

This paper outlines the methods used in the assessment of these injuries and provides information about the depth and distribution of the nerve lesions, their associated injuries and neuropathic pain syndromes.

Degenerative problems of the hip in patients with childhood and adult onset neuromuscular disorders can be challenging to treat. Many orthopaedic surgeons are reluctant to recommend total hip replacement (THR) for patients with underlying neuromuscular disorders due to the perceived increased risks of dislocation, implant loosening, and lack of information about the functional outcomes and potential benefits of these procedures in these patients. Modular femoral components and alternative bearings which facilitate the use of large femoral heads, constrained acetabular components and perhaps more importantly, a better understanding about the complications and outcomes of THR in the patient with neuromuscular disorders, make this option viable. This paper will review the current literature and our experience with THR in the more frequently encountered neuromuscular disorders.

Cite this article: Bone Joint J 2014;96-B(11 Suppl A):27–31.

Two types of fracture, early and late, have been reported following limb lengthening in patients with achondroplasia (ACH) and hypochondroplasia (HCH).

We reviewed 25 patients with these conditions who underwent 72 segmental limb lengthening procedures involving the femur and/or tibia, between 2003 and 2011. Gender, age at surgery, lengthened segment, body mass index, the shape of the callus, the amount and percentage of lengthening and the healing index were evaluated to determine predictive factors for the occurrence of early (within three weeks after removal of the fixation pins) and late fracture (> three weeks after removal of the pins). The Mann‑Whitney U test and Pearson’s chi-squared test for univariate analysis and stepwise regression model for multivariate analysis were used to identify the predictive factor for each fracture. Only one patient (two tibiae) was excluded from the analysis due to excessively slow formation of the regenerate, which required supplementary measures. A total of 24 patients with 70 limbs were included in the study.

There were 11 early fractures in eight patients. The shape of the callus (lateral or central callus) was the only statistical variable related to the occurrence of early fracture in univariate and multivariate analyses. Late fracture was observed in six limbs and the mean time between removal of the fixation pins and fracture was 18.3 weeks (3.3 to 38.4). Lengthening of the tibia, larger healing index, and lateral or central callus were related to the occurrence of a late fracture in univariate analysis. A multivariate analysis demonstrated that the shape of the callus was the strongest predictor for late fracture (odds ratio: 19.3, 95% confidence interval: 2.91 to 128). Lateral or central callus had a significantly larger risk of fracture than fusiform, cylindrical, or concave callus.

Radiological monitoring of the shape of the callus during distraction is important to prevent early and late fracture of lengthened limbs in patients with ACH or HCH. In patients with thin callus formation, some measures to stimulate bone formation should be considered as early as possible.

Cite this article: Bone Joint J 2014;96-B:1269–73.

Coronal plane fractures of the posterior femoral condyle, also known as Hoffa fractures, are rare. Lateral fractures are three times more common than medial fractures, although the reason for this is not clear. The exact mechanism of injury is likely to be a vertical shear force on the posterior femoral condyle with varying degrees of knee flexion. These fractures are commonly associated with high-energy trauma and are a diagnostic and surgical challenge. Hoffa fractures are often associated with inter- or supracondylar distal femoral fractures and CT scans are useful in delineating the coronal shear component, which can easily be missed. There are few recommendations in the literature regarding the surgical approach and methods of fixation that may be used for this injury. Non-operative treatment has been associated with poor outcomes. The goals of treatment are anatomical reduction of the articular surface with rigid, stable fixation to allow early mobilisation in order to restore function. A surgical approach that allows access to the posterior aspect of the femoral condyle is described and the use of postero-anterior lag screws with or without an additional buttress plate for fixation of these difficult fractures.

Cite this article: Bone Joint J 2013;95-B:1165–71.

Ten patients, who were unsuitable for limb lengthening over an intramedullary nail, underwent lengthening with a submuscular locking plate. Their mean age at operation was 18.5 years (11 to 40). After fixing a locking plate submuscularly on the proximal segment, an external fixator was applied to lengthen the bone after corticotomy. Lengthening was at 1 mm/day and on reaching the target length, three or four screws were placed in the plate in the distal segment and the external fixator was removed. All patients achieved the pre-operative target length at a mean of 4.0 cm (3.2 to 5.5). The mean duration of external fixation was 61.6 days (45 to 113) and the mean external fixation index was 15.1 days/cm (13.2 to 20.5), which was less than one-third of the mean healing index (48 days/cm (41.3 to 55). There were only minor complications.

Lengthening with a submuscular locking plate can successfully permit early removal of the fixator with fewer complications and is a useful alternative in children or when nailing is difficult.

In Canada, Dupuytren's contracture is managed with partial fasciectomy or percutaneous needle aponeurotomy (PNA). Injectable collagenase will soon be available. The optimal management of Dupuytren’s contracture is controversial and trade-offs exist between the different methods. Using a cost-utility analysis approach, our aim was to identify the most cost-effective form of treatment for managing Dupuytren’s contracture it and the threshold at which collagenase is cost-effective. We developed an expected-value decision analysis model for Dupuytren’s contracture affecting a single finger, comparing the cost-effectiveness of fasciectomy, aponeurotomy and collagenase from a societal perspective. Cost-effectiveness, one-way sensitivity and variability analyses were performed using standard thresholds for cost effective treatment ($50 000 to $100 000/QALY gained). Percutaneous needle aponeurotomy was the preferred strategy for managing contractures affecting a single finger. The cost-effectiveness of primary aponeurotomy improved when repeated to treat recurrence. Fasciectomy was not cost-effective. Collagenase was cost-effective relative to and preferred over aponeurotomy at $875 and $470 per course of treatment, respectively.

In summary, our model supports the trend towards non-surgical interventions for managing Dupuytren’s contracture affecting a single finger. Injectable collagenase will only be feasible in our publicly funded healthcare system if it costs significantly less than current United States pricing.

Cite this article: Bone Joint J 2013;95-B:1094–1100.

Extensive limb lengthening may be indicated in achondroplastic patients who wish to achieve a height within the normal range for their population. However, increasing the magnitude of lengthening is associated with further complications particularly adjacent joint stiffness and fractures. We studied the relationship between the magnitude of femoral lengthening and callus pattern, adjacent joint stiffness and fracture of the regenerate bone in 40 femoral lengthenings in 20 achondroplastic patients. They were divided into two groups; group A had lengthening of less than 50% and group B of more than 50% of their initial femoral length. The patterns of radiological callus formation were classified according to shape, type and features. The incidence of callus features, knee stiffness and regenerate bone fracture were analysed in the two groups. Group B was associated with an increased incidence of concave, lateral and central callus shapes, adjacent joint and stiffness and fracture. Statistically, the incidence of stiffness in adjacent joints and regenerate bone fracture was significantly associated with the magnitude of lengthening.

We suggest that careful radiological assessment of the patterns of callus formation is a useful method for the evaluation and monitoring of regenerate bone.

We carried out a systematic review of the literature to evaluate the evidence regarding the clinical results of the Ilizarov method in the treatment of long bone defects of the lower limbs.

Only 37 reports (three non-randomised comparative studies, one prospective study and 33 case-series) met our inclusion criteria. Although several studies were unsatisfactory in terms of statistical heterogeneity, our analysis appears to show that the Ilizarov method of distraction osteogenesis significantly reduced the risk of deep infection in infected osseous lesions (risk ratio 0.14 (95% confidence interval (CI) 0.10 to 0.20), p < 0.001). However, there was a rate of re-fracture of 5% (95% CI 3 to 7), with a rate of neurovascular complications of 2.2% (95% CI 0.3 to 4) and an amputation rate of 2.9% (95% CI 1.4 to 4.4).The data was generally not statistically heterogeneous. Where tibial defects were > 8 cm, the risk of re-fracture increased (odds ratio 3.7 (95% CI 1.1 to 12.5), p = 0.036).

The technique is demanding for patients, illustrated by the voluntary amputation rate of 1.6% (95% CI 0 to 3.1), which underlines the need for careful patient selection.

Cite this article: Bone Joint J 2013;95-B:1673–80.

In this retrospective study we evaluated the method of acute shortening and distraction osteogenesis for the treatment of tibial nonunion with bone loss in 17 patients with a mean age of 36 years (10 to 58). The mean bone loss was 5.6 cm (3 to 10). In infected cases, we performed the treatment in two stages. The mean follow-up time was 43.5 months (24 to 96). The mean time in external fixation was 8.0 months (4 to 13) and the mean external fixator index was 1.4 months/cm (1.1 to 1.8). There was no recurrence of infection. The bone evaluation results were excellent in 16 patients and good in one, while functional results were excellent in 15 and good in two. The complication rate was 1.2 per patient.

We conclude that acute shortening and distraction osteogenesis is a safe, reliable and successful method for the treatment of tibial nonunion with bone loss, with a shorter period of treatment and lower rate of complication.

We have managed 21 patients with a fracture of the tibia complicated by bone and soft-tissue loss as a result of an open fracture in 10, or following debridement of an infected nonunion in 11, by resection of all the devitalised tissues, acute limb shortening to close the defect, application of an external fixator and metaphyseal osteotomy for re-lengthening. The mean bone loss was 4.7 cm (3 to 11). The mean age of the patients was 28.8 years (12 to 54) and the mean follow-up was 34.8 months (24 to 75).

All the fractures united with a well-aligned limb. The mean duration of treatment for the ten grade-III A+B open fractures (according to the Gustilo-Anderson classification) was 5.7 months (4.5 to 8) and for the nonunions, 7.6 months (5.5 to 12.5). Complications included one refracture, one transient palsy of the peroneal nerve and one equinus contracture of 10°.

This annotation considers the place of extra-articular reconstruction in the treatment of anterior cruciate ligament (ACL) deficiency. Extra-articular reconstruction has been employed over the last century to address ACL deficiency. However, the technique has not gained favour, primarily due to residual instability and the subsequent development of degenerative changes in the lateral compartment of the knee. Thus intra-articular reconstruction has become the technique of choice. However, intra-articular reconstruction does not restore normal knee kinematics. Some authors have recommended extra-articular reconstruction in conjunction with an intra-articular technique.

The anatomy and biomechanics of the anterolateral structures of the knee remain largely undetermined. Further studies to establish the structure and function of the anterolateral structures may lead to more anatomical extra-articular reconstruction techniques that supplement intra-articular reconstruction. This might reduce residual pivot shift after an intra-articular reconstruction and thus improve the post-operative kinematics of the knee.

Stüve-Wiedemann syndrome is an autosomal-recessive disorder characterised by bowing of the long bones, progressive scoliosis, episodic hyperthermia and respiratory distress, usually resulting in death in infancy. We reviewed five children with the condition who had been followed since birth and who survived into childhood with a mean age at operation of 7.8 years (5 to 14). There was marked functional impairment with dysplasia of the long bones and scoliosis. Treatment of the triplanar deformities of the femora involved the use of the Ilizarov technique with the Taylor Spatial Frame.

Walking was preserved and improved in three children along with considerable enhancement of the appearance. Early insertion of a growing rod to control the progressive juvenile scoliosis was beneficial. The use of the Taylor Spatial Frame is strongly recommended to address the major complex deformities of the lower limbs which are encountered in this condition and to prevent their progression.

The outcome of surgery in patients with medial epicondylitis of the elbow is less favourable in those with co-existent symptoms from the ulnar nerve. We wanted to know whether we could successfully treat such patients by using musculofascial lengthening of the flexor-pronator origin with simultaneous deep transposition of the ulnar nerve. We retrospectively reviewed 19 patients who were treated in this way. Seven had grade I and 12 had grade IIa ulnar neuropathy. At a mean follow-up of 38 months (24 to 48), the mean visual analogue scale pain scores improved from 3.7 to 0.3 at rest, from 6.6 to 2.1 with activities of daily living, and from 7.9 to 2.3 at work or sports, and the mean disabilities of the arm, shoulder and hand scores improved from 42.2 to 23.5.

These results suggest that this technique can be effective in treating patients with medial epicondylitis and coexistent ulnar nerve symptoms.

We have developed an illustrated questionnaire, the Hand20, comprising 20 short and easy-to-understand questions to assess disorders of the upper limb. We have examined the usefulness of this questionnaire by comparing reliability, validity, responsiveness and the level of missing data with those of the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire.

A series of 431 patients with disorders of the upper limb completed the Hand20 and the Japanese version of the DASH (DASH-JSSH) questionnaire. The norms for Hand20 scores were determined in another cross-sectional study.

Most patients had no difficulty in completing the Hand20 questionnaire, whereas the DASH-JSSH had a significantly higher rate of missing data. The standard score for the Hand20 was smaller than the reported norms for the DASH.

Our study showed that the Hand20 questionnaire provided validation comparable with that of the DASH-JSSH. Explanatory illustrations and short questions which were easy-to-understand led to better rates of response and fewer missing data, even in elderly individuals with cognitive deterioration.

Arthrolysis and dynamic splinting have been used in the treatment of elbow contractures, but there is no standardised protocol for treatment of severe contractures with an arc of flexion < 30°. We present our results of radical arthrolysis with twin incisions with the use of a monolateral hinged fixator to treat very severe extra-articular contracture of the elbow. This retrospective study included 26 patients (15 males and 11 females) with a mean age of 30 years (12 to 60). The mean duration of stiffness was 9.1 months (5.4 to 18) with mean follow-up of 5.2 years (3.5 to 9.4). The mean pre-operative arc of movement was 15.6° (0° to 30°), with mean pre-operative flexion of 64.1° (30° to 120°) and mean pre-operative extension of 52.1° (10° to 90°). Post-operatively the mean arc improved to 102.4° (60° to 135°), the mean flexion improved to 119.1° (90° to 140°) and mean extension improved to 16.8° (0° to 30°) (p < 0.001). The Mayo elbow score improved from a mean of 45 (30 to 65) to 89 (75 to 100) points, and 13 had excellent, nine had good, three had fair and one had a poor result. We had one case of severe instability and one wound dehiscence which responded well to treatment. One case had deep infection with poor results which responded well to treatment.

Our findings indicate that this method is very effective in the treatment of severe elbow contracture; however, a randomised controlled study is necessary for further evaluation.

Proximal osteotomies for forefoot deformity in patients with rheumatoid arthritis have hitherto not been described. We evaluated combination joint-preserving surgery involving three different proximal osteotomies for such deformities. A total of 30 patients (39 feet) with a mean age of 55.6 years (45 to 67) underwent combined first tarsometatarsal fusion and distal realignment, shortening oblique osteotomies of the bases of the second to fourth metatarsals and a fifth-ray osteotomy.

The mean follow-up was 36 months (24 to 68). The mean foot function index scores for pain, disability and activity subscales were 18, 23, and 16 respectively. The mean Japanese Society for Surgery of the Foot score improved significantly from 52.2 (41 to 68) to 89.6 (78 to 97). Post-operatively, 14 patients had forefoot stiffness, but had no disability. Most patients reported highly satisfactory walking ability. Residual deformity and callosities were absent. The mean hallux valgus and intermetatarsal angles decreased from 47.0° (20° to 67°) to 9.0° (2° to 23°) and from 14.1° (9° to 20°) to 4.6° (1° to 10°), respectively. Four patients had further surgery including removal of hardware in three and a fifth-ray osteotomy in one.

With good peri-operative medical management of rheumatoid arthritis, surgical repositioning of the metatarsophalangeal joint by metatarsal shortening and consequent relaxing of surrounding soft tissues can be successful. In early to intermediate stages of the disease, it can be performed in preference to joint-sacrificing procedures.

A total of 38 relapsed congenital clubfeet (16 stiff, 22 partially correctable) underwent revision of soft-tissue surgery, with or without a bony procedure, and transfer of the tendon of tibialis anterior at a mean age of 4.8 years (2.0 to 10.1). The tendon was transferred to the third cuneiform in five cases, to the base of the third metatarsal in ten and to the base of the fourth in 23. The patients were reviewed at a mean follow-up of 24.8 years (10.8 to 35.6). A total of 11 feet were regarded as failures (one a tendon failure, five with a subtalar fusion due to over-correction, and five with a triple arthrodesis due to under-correction or relapse).

In the remaining feet the clinical outcome was excellent or good in 20 and fair or poor in seven. The mean Laaveg-Ponseti score was 81.6 of 100 points (52 to 92). Stiffness was mild in four feet and moderate or severe in 23.

Comparison between the post-operative and follow-up radiographs showed statistically significant variations of the talo-first metatarsal angle towards abduction. Variations of the talocalcaneal angles and of the overlap ratio were not significant.

Extensive surgery for relapsed clubfoot has a high rate of poor long-term results. The addition of transfer of the tendon of tibialis anterior can restore balance and may provide some improvement of forefoot adduction. However, it has a considerable complication rate, including failure of transfer, over-correction, and weakening of dorsiflexion. The procedure should be reserved for those limited cases in which muscle imbalance is a causative or contributing factor.

Our study describes the clinical outcome of total ankle replacement (TAR) performed in patients with moderate to severe varus deformity. Between September 2004 and September 2007, 23 ankles with a varus deformity ≥ 10° and 22 with neutral alignment received a TAR. Following specific algorithms according to joint congruency, the varus ankles were managed by various additional procedures simultaneously with TAR.

After a mean follow-up of 27 months (12 to 47), the varus ankles improved significantly in all clinical measures (p < 0.0001 for visual analogue scale and American Orthopaedic Foot and Ankle Society score, p = 0.001 for range of movement). No significant differences were found between the varus and neutral groups regarding the clinical (p = 0.766 for visual analogue scale, p = 0.502 for American Orthopaedic Foot and Ankle Society score, p = 0.773 for range of movement) and radiological outcome (p = 0.339 for heterotopic ossification, p = 0.544 for medial cortical reaction, p = 0.128 for posterior focal osteolysis). Failure of the TAR with conversion to an arthrodesis occurred in one case in each group.

The clinical outcome of TAR performed in ankles with pre-operative varus alignment ≥ 10° is comparable with that of neutrally aligned ankles when appropriate additional procedures to correct the deformity are carried out simultaneously with TAR.

Complex regional pain syndrome is characterised by an exaggerated response to injury in a limb with intense prolonged pain, vasomotor disturbance, delayed functional recovery and trophic changes. This review describes the current knowledge of the condition and outlines the methods of treatment available with particular emphasis on the knee.

We developed a new porous scaffold made from a synthetic polymer, poly(DL-lactide-co-glycolide) (PLG), and evaluated its use in the repair of cartilage. Osteochondral defects made on the femoral trochlear of rabbits were treated by transplantation of the PLG scaffold, examined histologically and compared with an untreated control group.

Fibrous tissue was initially organised in an arcade array with poor cellularity at the articular surface of the scaffold. The tissue regenerated to cartilage at the articular surface. In the subchondral area, new bone formed and the scaffold was absorbed. The histological scores were significantly higher in the defects treated by the scaffold than in the control group (p < 0.05).

Our findings suggest that in an animal model the new porous PLG scaffold is effective for repairing full-thickness osteochondral defects without cultured cells and growth factors.

We report the outcome of 28 patients with spina bifida who between 1989 and 2006 underwent 43 lower extremity deformity corrections using the Ilizarov technique. The indications were a flexion deformity of the knee in 13 limbs, tibial rotational deformity in 11 and foot deformity in 19. The mean age at operation was 12.3 years (5.2 to 20.6). Patients had a mean of 1.6 previous operations (0 to 5) on the affected limb. The mean duration of treatment with a frame was 9.4 weeks (3 to 26) and the mean follow-up was 4.4 years (1 to 9). There were 12 problems (27.9%), five obstacles (11.6%) and 13 complications (30.2%) in the 43 procedures. Further operations were needed in seven patients. Three knees had significant recurrence of deformity. Two tibiae required further surgery for recurrence. All feet were plantigrade and braceable.

We conclude that the Ilizarov technique offers a refreshing approach to the complex lower-limb deformity in spina bifida.