Objectives

To determine the pattern of mutations of the WISP3 gene in clinically identified progressive pseudorheumatoid dysplasia (PPD) in an Indian population.

Aims. We investigated the long-term performance of the Tripolar Trident acetabular component used for recurrent dislocation in revision total hip arthroplasty. We assessed: 1) rate of re-dislocation; 2) incidence of complications requiring re-operation; and 3) Western Ontario and McMaster Universities osteoarthritis index (WOMAC) pain and functional scores. Patients and Methods. We retrospectively identified 111 patients who had 113 revision tripolar constrained liners between 1994 and 2008. All patients had undergone revision hip arthroplasty before the constrained liner was used: 13 after the first revision, 17 after the second, 38 after the third, and 45 after more than three revisions. A total of 75 hips (73 patients) were treated with Tripolar liners due to recurrent instability with abductor deficiency, In addition, six patients had associated cerebral palsy, four had poliomyelitis, two had multiple sclerosis, two had spina bifida, two had spondyloepiphyseal dysplasia, one had previous reversal of an arthrodesis, and 21 had proximal femoral replacements. The mean age of patients at time of Tripolar insertions was 72 years (53 to 89); there were 69 female patients (two bilateral) and 42 male patients. All patients were followed up for a mean of 15 years (10 to 24). Overall, 55 patients (57 hips) died between April 2011 and February 2018, at a mean of 167 months (122 to 217) following their tripolar liner implantation. We extracted demographics, implant data, rate of dislocations, and incidence of other complications. Results. At ten years, the Kaplan–Meier survivorship for dislocation was 95.6% (95% confidence interval (CI) 90 to 98), with 101 patients at risk. At 20 years, the survivorship for dislocation was 90.6% (95% CI 81.0 to 95.5), with one patient at risk. Eight patients (7.2%) had a dislocation of their constrained liners. At ten years, the survival to any event was 89.4% (95% CI 82 to 93.8), with 96 patients at risk. At 20 years, the survival to any event was 82.5% (95% CI 71.9 to 89.3), with one patient at risk. Five hips (4.4%) had deep infection. Two patients (1.8%) developed dissociated constraining rings with pain but without dislocation, which required re-operation. Two patients (1.8%) had periprosthetic femoral fractures, without dislocation, that were treated by revision stems along with exchange of the well-functioning constrained liners. Conclusion. Constrained tripolar liners used at revision hip arthroplasty provided favourable results in the long term for treatment of recurrent dislocation and for patients at high risk of dislocation. Cite this article: Bone Joint J 2019;101-B(6 Supple B):123–126

Aims

The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes.

Aims

This study aimed to evaluate the relationship between hip shape and mid-term function in Perthes’ disease. It also explored whether the modified three-group Stulberg classification can offer similar prognostic information to the five-group system.

Aims

The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management.

Aims

The aim of this study was to evaluate the epidemiology and treatment of Perthes’ disease of the hip.

Aims

The Patient-Reported Outcomes Measurement Information System (PROMIS) has demonstrated faster administration, lower burden of data capture and reduced floor and ceiling effects compared to traditional Patient Reported Outcomes Measurements (PROMs). We investigated the suitability of PROMIS Mobility score in assessing physical function in the sequelae of childhood hip disease.

Aims

The aim of this study was to present the first retrieval analysis findings of PRECICE STRYDE intermedullary nails removed from patients, providing useful information in the post-market surveillance of these recently introduced devices.

Aims

Computer hexapod assisted orthopaedic surgery (CHAOS), is a method to achieve the intra-operative correction of long bone deformities using a hexapod external fixator before definitive internal fixation with minimally invasive stabilisation techniques.

The aims of this study were to determine the reliability of this method in a consecutive case series of patients undergoing femoral deformity correction, with a minimum six-month follow-up, to assess the complications and to define the ideal group of patients for whom this treatment is appropriate.

We compared the complications and outcome of tibial lengthening using the Ilizarov method with and without the use of a supplementary intramedullary nail. In a retrospective case-matched series assembled from 176 patients with tibial lengthening, we matched 52 patients (26 pairs, group A with nail and group B without) according to the following criteria in order of importance: 1) difference in amount of lengthening (± 2 cm); 2) percentage difference in lengthening (± 5%); 3) difference in patient’s age (± seven years); 4) aetiology of the shortening, and 5) level of difficulty in obtaining the correction. The outcome was evaluated using the external fixator index, the healing index and an outcome score according to the criteria of Paley. It was found that some complications were specific to group A or B respectively, but others were common to both groups.

The outcome was generally better in lengthenings with a nail, although there was a higher incidence of rectifiable equinus deformity in these patients.

The purpose of this study was to determine the annual incidence of Perthes’ disease in Korea and compare this with other populations. A survey identified all newly diagnosed children with Perthes’ disease aged 14 years or younger in South Honam, Korea, between January 1999 and December 2001. A total of 84 children were included: 29 in 1999, 28 in 2000 and 27 in 2001. The mean annual incidence was 3.8 per 100 000. This is similar to that reported in other Asian countries, but higher than in black populations and lower than in Caucasians.

Acute angulation at the thoracolumbar junction with segmental subluxation of the spine occurring at the level above an anteriorly hypoplastic vertebra in otherwise normal children is a rare condition described as infantile developmental thoracolumbar kyphosis. Three patient series with total of 18 children have been reported in the literature. We report five children who presented with thoracolumbar kyphosis and discuss the treatment algorithm. We reviewed the medical records and spinal imaging at initial clinical presentation and at minimum two-year follow-up. The mean age at presentation was eight months (two to 12). All five children had L2 anterior vertebral body hypoplasia. The kyphosis improved spontaneously in three children kept under monitoring. In contrast, the deformity was progressive in two patients who were treated with bracing. The kyphosis and segmental subluxation corrected at latest follow-up (mean age 52 months; 48 to 60) in all patients with near complete reconstitution of the anomalous vertebra. The deformity and radiological imaging on a young child can cause anxiety to both parents and treating physicians. Diagnostic workup and treatment algorithm in the management of infantile developmental thoracolumbar kyphosis is proposed. Observation is indicated for non-progressive kyphosis and bracing if there is evidence of kyphosis and segmental subluxation deterioration beyond walking age. Surgical stabilisation of the spine can be reserved for severe progressive deformities unresponsive to conservative treatment.

Cite this article: Bone Joint J 2015;97-B:982–7.

The December 2013 Children’s orthopaedics Roundup³⁶⁰ looks at: Long term-changes in hip morphology following osteotomy; Arthrogrypotic wrist contractures are surgically amenable; Paediatric femoral lengthening over a nail; Current management of paediatric supracondylar fractures; MRI perfusion index predictive of Perthes’ progression; Abduction bracing effective in residual acetabular deformity; Hurler syndrome in the spotlight; and the Pavlik works for femoral fractures too!

Patients with skeletal dysplasia are prone to developing advanced osteoarthritis of the knee requiring total knee replacement (TKR) at a younger age than the general population. TKR in this unique group of patients is a technically demanding procedure owing to the deformity, flexion contracture, generalised hypotonia and ligamentous laxity. We retrospectively reviewed the outcome of 11 TKRs performed in eight patients with skeletal dysplasia at our institution using the Stanmore Modular Individualised Lower Extremity System (SMILES) custom-made rotating-hinge TKR. There were three men and five women with mean age of 57 years (41 to 79). Patients were followed clinically and radiologically for a mean of seven years (3 to 11.5). The mean Knee Society clinical and function scores improved from 24 (14 to 36) and 20 (5 to 40) pre-operatively, respectively, to 68 (28 to 80) and 50 (22 to 74), respectively, at final follow-up. Four complications were recorded, including a patellar fracture following a fall, a tibial peri-prosthetic fracture, persistent anterior knee pain, and aseptic loosening of a femoral component requiring revision. Our results demonstrate that custom primary rotating-hinge TKR in patients with skeletal dysplasia is effective at relieving pain, with a satisfactory range of movement and improved function. It compensates for bony deformity and ligament deficiency and reduces the likelihood of corrective osteotomy. Patellofemoral joint complications are frequent and functional outcome is worse than with primary TKR in the general population.

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 studied the effects of hyperbaric oxygen (HBO) and zoledronic acid (ZA) on posterior lumbar fusion using a validated animal model. A total of 40 New Zealand white rabbits underwent posterior lumbar fusion at L5–6 with autogenous iliac bone grafting. They were divided randomly into four groups as follows: group 1, control; group 2, HBO (2.4 atm for two hours daily); group 3, local ZA (20 μg of ZA mixed with bone graft); and group 4, combined HBO and local ZA. All the animals were killed six weeks after surgery and the fusion segments were subjected to radiological analysis, manual palpation, biomechanical testing and histological examination.

Five rabbits died within two weeks of operation. Thus, 35 rabbits (eight in group 1 and nine in groups 2, 3 and 4) completed the study. The rates of fusion in groups 3 and 4 (p = 0.015) were higher than in group 1 (p < 0.001) in terms of radiological analysis and in group 4 was higher than in group 1 with regard to manual palpation (p = 0.015). We found a statistically significant difference in the biomechanical analysis between groups 1 and 4 (p = 0.024). Histological examination also showed a statistically significant difference between groups 1 and 4 (p = 0.036).

Our results suggest that local ZA combined with HBO may improve the success rate in posterior lumbar spinal fusion.

We have reviewed 70 Harris-Galante uncemented acetabular components implanted as hybrid hip replacements with cemented stems between 1991 and 1995 in 53 patients whose mean age was 40 years (19 to 49). The mean follow-up was for 13.6 years (12 to 16) with no loss to follow-up. We assessed the patients both clinically and radiologically.

The mean Oxford hip score was 20 (12 to 46) and the mean Harris hip score 81 (37 to 100) at the final review. Radiologically, 27 hips (39%) had femoral osteolysis, 13 (19%) acetabular osteolysis, and 31 (44%) radiolucent lines around the acetabular component. Kaplan-Meier survival curves were constructed for the outcomes of revision of the acetabular component, revision of the component and polyethylene liner, and impending revision for progressive osteolysis.

The cumulative survival for revision of the acetabular component was 94% (95% confidence interval 88.4 to 99.7), for the component and liner 84% (95% confidence interval 74.5 to 93.5) and for impending revision 55.3% (95% confidence interval 40.6 to 70) at 16 years.

Uncemented acetabular components with polyethylene liners undergo silent lysis and merit regular long-term radiological review.