The management of joint replacement in lysosomal storage diseases has not been well reported. We present three patients with progressive degenerative changes of the hips who required bilateral total hip replacement in early childhood. The stature of the patients make it essential to have access to appropriately scaled prostheses. Consideration has to be given to associated disorders of the skeleton which must be carefully screened to ensure safety in providing appropriate anaesthesia as well as ensuring that there is no cardiac abnormality. In one patient, a periprosthetic fracture was sustained in one hip in the early post-operative course requiring internal fixation.

The patient made a full recovery and all six hips were clinically and radiologically satisfactory at mid-term review.

We compared 55 consecutive total hip replacements performed on 53 morbidly obese patients with osteoarthritis with a matched group of 55 total hip replacements in 53 non-obese patients. The groups were matched for age, gender, prosthesis type, laterality and preoperative Harris Hip Score. They were followed prospectively for five years and the outcomes were assessed using the Harris Hip Score, the Short-form 36 score and radiological findings.

Survival at five years using revision surgery as an endpoint, was 90.9% (95% confidence interval 82.9 to 98.9) for the morbidly obese and 100% for the non-obese patients. The Harris Hip and the Short-form 36 scores were significantly better in the non-obese group (p < 0.001). The morbidly obese patients had a higher rate of complications (22% vs 5%, p = 0.012), which included dislocation and both superficial and deep infection.

In light of these inferior results, morbidly obese patients should be advised to lose weight before undergoing a total hip replacement, and counselled regarding the complications. Despite these poorer results, however, the patients have improved function and quality of life.

We report the outcome of 161 of 257 surgically fixed acetabular fractures. The operations were undertaken between 1989 and 1998 and the patients were followed for a minimum of ten years. Anthropometric data, fracture pattern, time to surgery, associated injuries, surgical approach, complications and outcome were recorded. Modified Merle D’Aubigné score and Matta radiological scoring systems were used as outcome measures. We observed simple fractures in 108 patients (42%) and associated fractures in 149 (58%).

The result was excellent in 75 patients (47%), good in 41 (25%), fair in 12 (7%) and poor in 33 (20%). Poor prognostic factors included increasing age, delay to surgery, quality of reduction and some fracture patterns. Complications were common in the medium- to long-term and functional outcome was variable. The gold-standard treatment for displaced acetabular fractures remains open reduction and internal fixation performed in dedicated units by specialist surgeons as soon as possible.

We present a prospective review of the two-year functional outcome of 37 Avon patellofemoral joint replacements carried out in 29 patients with a mean age of 66 years (30 to 82) between October 2002 and March 2007. No patients were lost to follow-up. This is the first independent assessment of this prosthesis using both subjective and objective analysis of outcome. At two years the median Oxford knee score was 39 (interquartile range 32 to 44), the median American Knee Society objective score was 95 (interquartile range 90 to 100), the median American Knee Society functional score was 85 (interquartile range 60 to 100), and the median Melbourne Knee score was 28 (interquartile range 21 to 30). Two patients underwent further surgery. Only one patient reported an unsatisfactory outcome.

We conclude that the promising early results observed by the designing centre are reproducible and provide further support for the role of patellofemoral joint replacement.

We reviewed the rate of revision of unicompartmental knee replacements (UKR) from the New Zealand Joint Registry between 1999 and 2008. There were 4284 UKRs, of which 236 required revision, 205 to a total knee replacement (U2T) and 31 to a further unicompartmental knee replacement (U2U). We used these data to establish whether the survival and functional outcome for revised UKRs were comparable with those of primary total knee replacement (TKR). The rate of revision for the U2T cohort was four times higher than that for a primary TKR (1.97 vs 0.48; p < 0.05). The mean Oxford Knee Score was also significantly worse in the U2T group than that of the primary TKR group (30.02 vs 37.16; p < 0.01). The rate of revision for conversion of a failed UKR to a further UKR (U2U cohort) was 13 times higher than that for a primary TKR.

The poor outcome of a UKR converted to a primary TKR compared with a primary TKR should contra-indicate the use of a UKR as a more conservative procedure in the younger patient.

Osteogenesis imperfecta is a rare inherited disorder of connective tissue which may present with recurrent fractures which are prone to nonunion and malunion resulting in deformity. Some patients develop osteoarthritis of the hip. Formation of hyperplastic callus after recurrent fractures may deform the shape of the femur and preclude the use of standard implants at joint replacement. Replacement can thus be technically demanding.

We present a case of bilateral hip replacement in a patient with osteogenesis imperfecta and hyperplastic callus which was treated by the use of long femoral allografts and cemented femoral stems.

We present the outcomes in 38 consecutive patients who had total ankle replacement using the Ankle Evolution System with a minimum follow-up of four years. Pain and function were assessed using the American Orthopaedic Foot and Ankle Society (AOFAS) score and regular standardised anteroposterior and lateral weight-bearing radiographs were obtained. Patient satisfaction and complications were recorded and the survival of the implants was demonstrated by the Kaplan-Meier method.

The mean follow-up was for 57.8 months (48 to 80). The cumulative survival rate at six years was 94.7% (95% confidence interval 80.3 to 98.7). The mean total AOFAS score was 88.1 (53 to 100). The mean score for pain was 35.8 (20 to 40). Ten patients presented with edge-loading of whom nine had corrective surgery. Two ankles were revised, one to an arthrodesis and the other to replace the tibial component. Nine patients showed radiological evidence of osteolysis. They had minimal non-progressive symptoms and further surgery was not undertaken. Nevertheless, the concerns about osteolysis led to the implant being withdrawn by the manufacturer.

The medium-term results of the ankle evolution system ankle replacement are satisfactory with high patient satisfaction, but the rate of osteolysis is of some concern. The long-term benefit of this procedure has yet to be determined.

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.

This is a retrospective study of six children with ununited scaphoid fractures treated conservatively. Their mean age was 12.8 years (9.7 to 16.3). Five had no early treatment. Radiological signs of nonunion were found at a mean of 4.6 months (3 to 7) after injury. Treatment consisted of cast immobilisation until clinical and radiological union. The mean clinical and radiological follow-up was for 67 months (17 to 90). We assessed the symptoms, the range of movement of the wrist and the grip strength to calculate the Modified Mayo Wrist score.

The fracture united in all patients after a mean period of immobilisation of 5.3 months (3 to 7). Five patients were pain free; one had mild pain. All returned to regular activities, and had a range of movement and grip strength within 25% of normal, resulting in an excellent Modified Mayo Wrist score.

Prolonged treatment with cast immobilisation resulted in union of the fracture and an excellent Modified Wrist Score in all patients.

Between May 1998 and May 2007 we carried out 50 Avon patellofemoral joint replacements in 32 patients with isolated patellofemoral osteoarthritis.

There were no revisions in the first five years, giving a cumulative survival of 100% for those with a minimum follow-up of five years. The mean follow-up was 5.3 years (2.1 to 10.2). The median Oxford knee score was 30.5 (interquartile range 22.25 to 42.25). In patients with bilateral replacements the median Euroqol General health score was 50 which was significantly lower than that of 75 in those with a unilateral replacement (p = 0.047). The main complication was progression of disease, which was identified radiologically in 11 knees (22%). This highlights the need for accurate selection of patients. Our findings suggest that the Avon prosthesis survives well and gives a satisfactory functional outcome in the medium term.

We report five children who presented at the mean age of 1.5 years (1.1 to 1.9) with a progressive thoracolumbar kyphosis associated with segmental instability and subluxation of the spine at the level above an anteriorly-wedged hypoplastic vertebra at L1 or L2. The spinal deformity appeared to be developmental and not congenital in origin. The anterior wedging of the vertebra may have been secondary to localised segmental instability and subsequent kyphotic deformity.

We suggest the term ‘infantile developmental thoracolumbar kyphosis with segmental subluxation of the spine’ to differentiate this type of deformity from congenital displacement of the spine in which the congenital vertebral anomaly does not resolve. Infantile developmental kyphosis with segmental subluxation of the spine, if progressive, may carry the risk of neurological compromise. In all of our patients the kyphotic deformity progressed over a period of three months and all were treated by localised posterior spinal fusion. At a mean follow-up of 6.6 years (5.0 to 9.0), gradual correction of the kyphosis was seen on serial radiographs as well as reconstitution of the hypoplastic wedged vertebra to normality. Exploration of the arthrodesis was necessary at nine months in one patient who developed a pseudarthrosis.

We compared the incidence and severity of complications during and after closing- and opening-wedge high tibial osteotomy used for the treatment of varus arthritis of the knee, and identified the risk factors associated with the development of complications. In total, 104 patients underwent laterally based closing-wedge and 90 medial opening-wedge high tibial osteotomy between January 1993 and December 2006. The characteristics of each group were similar. All the patients were followed up for more than 12 months. We assessed the outcome using the Hospital for Special Surgery knee score, and recorded the complications. Age, gender, obesity (body mass index > 27.5 kg/m²), the type of osteotomy (closing versus opening) and the pre-operative mechanical axis were subjected to risk-factor analysis.

The mean Hospital for Special Surgery score in the closing and opening groups improved from 73.4 (54 to 86) to 91.8 (81 to 100) and from 73.8 (56 to 88) to 93 (84 to 100), respectively. The incidence of complications overall and of major complications in both groups was not significantly different (p = 0.20 overall complication, p = 0.29 major complication). Logistic regression analysis adjusting for obesity and the pre-operative mechanical axis showed that obesity remained a significant independent risk factor (odds ratio = 3.23) of a major complication after high tibial osteotomy.

Our results suggest that the opening-wedge high tibial osteotomy can be an alternative treatment option for young patients with medial compartment osteoarthritis and varus deformity.

This study used CT analysis to determine the rotational alignment of 39 painful and 26 painless fixed-bearing total knee replacements (TKRs) from a cohort of 740 NexGen Legacy posterior-stabilised and cruciate-retaining prostheses implanted between May 1996 and August 2003.

The mean rotation of the tibial component was 4.3° of internal rotation (25.4° internal to 13.9° external rotation) in the painful group and 2.2° of external rotation (8.5° internal to 18.2° external rotation) in the painfree group (p = 0.024). In the painful group 17 tibial components were internally rotated more than 9° compared with none in the painfree group (p < 0.001). Additionally, six femoral components in the painful group were internally rotated more than 6° compared with none in the painfree group (p = 0.017). External rotational errors were not found to be associated with pain.

Overall, 22 (56.4%) of the painful TKRs had internal rotational errors involving the femoral, the tibial or both components. It is estimated that at least 4.6% of all our TKRs have been implanted with significant internal rotational errors.

The management of osteoarthritis of the knee associated with patellar instability secondary to external tibial torsion > 45° is challenging. Patellofemoral biomechanics in these patients cannot be achieved by intra-articular correction using standard techniques of total knee replacement.

We reviewed seven patients (eight knees) with recurrent patellar dislocation and one with bilateral irreducible lateral dislocation who had undergone simultaneous total knee replacement and internal tibial derotational osteotomy. All had osteoarthritis and severe external tibial torsion. The mean follow-up was for 47.2 months (24 to 120).

The mean objective and functional Knee Society scores improved significantly (p = 0.0001) from 29.7 and 41.5 pre-operatively to 71.4 and 73.5 post-operatively, respectively. In all patients the osteotomies healed and patellar stability was restored.

Excessive external tibial torsion should be identified and corrected in patients with osteoarthritis and patellar instability. Simultaneous internal rotation osteotomy of the tibia and total knee replacement is a technically demanding but effective treatment for such patients.

We have reviewed our experience of the removal of deep extremity orthopaedic implants in children to establish the nature, rate and risk of complications associated with this procedure. A retrospective review was performed of 801 children who had 1223 implants inserted and subsequently removed over a period of 17 years. Bivariate analysis of possible predictors including clinical factors, complications associated with implant insertion and indications for removal and the complications encountered at removal was performed. A logistical regression model was then constructed using those predictors which were significantly associated with surgical complications from the bivariate analyses. Odds ratios estimated in the logistical regression models were converted to risk ratios.

The overall rate of complications after removal of the implant was 12.5% (100 complications in 801 patients), with 48 (6.0%) major and 52 (6.5%) minor. Children with a complication after insertion of the initial implant or with a non-elective indication for removal, a neuromuscular disease associated with a seizure disorder or a neuromuscular disease in those unable to walk, had a significantly greater chance of having a major complication after removal of the implant. Children with all four of these predictors were 14.6 times more likely to have a major complication.

This paper describes the preliminary results of a proximal tibial endoprosthesis which spares the knee joint and enables retention of the natural articulation by replacing part of the tibial metaphysis and diaphysis. In eight patients who had a primary malignant bone tumour of the proximal tibia, the distal stem, which had a hydroxyapatite-coated collar to improve fixation, was cemented into the medullary canal. The proximal end had hydroxyapatite-coated extracortical plates which were secured to the remaining proximal tibial metaphysis using cortical screws. The mean age of the patients at operation was 28.9 years (8 to 43) and the mean follow-up was for 35 months (4 to 48). The mean Musculoskeletal Tumour Society score was 79% (57% to 90%), the mean Oxford Knee score was 40 points of 48 (36 to 46) and the mean knee flexion was 112° (100° to 120°). In one patient, revision to a below-knee amputation through the prosthesis was required because of recurrence of the tumour. Another patient sustained a periprosthetic fracture which healed with a painful malunion. This was revised to a further endoprosthesis which replaced the knee.

In the remaining six patients the prosthesis allowed preservation of the knee joint with good function and no early evidence of loosening. Further follow-up is required to assess the longevity of these prostheses.

The survivorship of contemporary resurfacing arthroplasty of the hip using metal-on-metal bearings is better than that of first generation designs, but short-term failures still occur. The most common reasons for failure are fracture of the femoral neck, loosening of the component, osteonecrosis of the femoral head, reaction to metal debris and malpositioning of the component. In 2008 the Australian National Joint Registry reported an inverse relationship between the size of the head component and the risk of revision in resurfacing hip arthroplasty. Hips with a femoral component size of ≤ 44 mm have a fivefold increased risk of revision than those with femoral components of ≥ 55 mm irrespective of gender. We have reviewed the literature to explore this observation and to identify possible reasons including the design of the implant, loading of the femoral neck, the orientation of the component, the production of wear debris and the effects of metal ions, penetration of cement and vascularity of the femoral head. Our conclusion is that although multifactorial, the most important contributors to failure in resurfacing arthroplasty of the hip are likely to be the design and geometry of the component and the orientation of the acetabular component.

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.

Between 1995 and 2006, five intra-articular osteotomies of the head of the radius were performed in patients with symptomatic healed displaced articular fractures. Pre-operatively, all patients complained of persistent painful clicking on movement. Only patients with mild or no degenerative changes of the radial head and capitellum were considered for osteotomy. The operations were performed at a mean of 8.2 months (4 to 13) after injury and the patients were reviewed at a mean of 5.5 years (15 months to 12 years) after the osteotomy.

The average Mayo Elbow Performance Index Score improved significantly from 74 before to 88 after operation, with four patients rated as good or excellent (p < 0.05). The subjective patient satisfaction score was 8.4 on a ten-point scale. All osteotomies healed and there were no complications.

In this small series intra-articular osteotomy of the head of the radius was a safe and effective treatment for symptomatic intra-articular malunion without advanced degenerative changes.

A review of the current literature shows that there is a lack of consensus regarding the treatment of spondylolysis and spondylolisthesis in children and adolescents. Most of the views and recommendations provided in various reports are weakly supported by evidence. There is a limited amount of information about the natural history of the condition, making it difficult to compare the effectiveness of various conservative and operative treatments. This systematic review summarises the current knowledge on spondylolysis and spondylolisthesis and attempts to present a rational approach to the evaluation and management of this condition in children and adolescents.