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.

Abstract. Introduction. Total knee replacement (TKR) in patients with skeletal dysplasia is technically challenging surgery due to deformity, joint contracture, and associated co-morbidities. The aim of this study is to follow up patients with skeletal dysplasia following a TKR. Methodology. We retrospectively reviewed 22 patients with skeletal dysplasia who underwent 31 TKRs at our institution between 2006 and 2022. Clinical notes, operative records and radiographic data were reviewed. Results. Achondroplasia was the most common skeletal dysplasia (8), followed by Chondrodysplasia punctata (7) and Spondyloepiphyseal dysplasia (5). There were fourteen men and eight women with mean age of 51 years (28 to 73). The average height of patients was 1.4 metres (1.16–1.75) and the mean weight was 64.8 Kg (34.3–100). The mean follow up duration was 68.32 months (1–161). Three patients died during follow up. Custom implants were required in twelve patients (38.71%). Custom jigs were utilised in six patients and two patients underwent robotic assisted surgery. Hinged TKR was used in seventeen patients (54.84%), posterior stabilised TKR in nine patients (29.03%), and cruciate retaining TKR in five patients (16.13%). One patient underwent a patella resurfacing for persistent anterior knee pain and another had an intra-operative medial tibial plateau fracture which was managed with fixation. No revisions occurred during the follow up period. Conclusion. Despite the technical challenges and complexity of TKR within this unique patient group, we demonstrate good implant survivorship during the study period. Cross sectional imaging is recommended preoperatively for precise planning and templating

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 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

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. 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

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.

Objectives

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

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!

Purpose. The correction obtained in the coronal plane knee deformity using guided growth was assessed in children with sick physes (Height<1SD of the normal children) to define the limits of this technique in sick physis. Methods. We retrospectively assessed deformity correction following guided growth using 8 plates in children with coronal plane deformity and metabolically abnormal physis- renal rickets in 6 and bone dysplasias (multiple epiphyseal dyspasia, spondyloepiphyseal dysplasia, metaphyseal dysplasia, mesomelic dysplasia, chondrodysplasia punctata) in 10 children aged 2 to 14 years. Lateral distal femoral angle(LDFA) and medial proximal tibia(MPTA) angles were serially assessed after eight plate application as a day care procedure. We noted correction achieved rate of correction, complications and additional surgical procedures. Fischer's exact test and multiple regression analysis was done to assess the effect of modifiers. Results. Sixteen children mean aged 7.8 years (2–14 years) with 9 boys and seven girls were followed for a mean of 16.125 months. Deviations from normal MPTA(n=6) were a mean of 11.82(range 8.4–16.9) and from normal LDFA(n=15) a mean of 13.96 (range 5.7–35.1). Mean rate of correction was 0.8°(range 0.1–2.65) per month in dysplasia group and 1.09°(range 0.5–1.6)per month. Complete correction was achieved in 2 out of 6 in renal and 4 out of 10 in dysplasia group. Complication included backing out of screw (1), suboptimal screw placement (2) in dysplasia group and stiffness of the knee (1). Multiple regression analysis found sex, severity of deformity >15° and duration of follow up to be not significant, however, age less than 6 years had a significantly higher chance of deformity correction(P=0.001). Conclusion. The rate of deformity correction with eight plates for guided growth is very variable even within the same patient in dysplasia group, chondrodyspalsia and SED having very slow correction rates. The deformities however correct completely before the age of 6 years irrespective of etiology. Bone dysplasias and metabolic causes require further study to understand their growth pattern and possibilities of relapse

Patients with skeletal dysplasia are prone to developing advanced degenerative knee disease 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 due to the bone deformity, flexion contracture, generalised hypotonia and ligamentous laxity. We set out to retrospectively review the outcome of 11 TKR's performed in eight patients with skeletal dysplasia at our institution using the SMILES custom-made rotating-hinge total knee system. There were 3 males and 5 females with mean age 57 years (range, 41–79 years), mean height 138 cm (range, 122–155 cm) and mean weight 56 kg (range, 40–102 kg). Preoperative diagnoses included achondroplasia, spondyloepiphyseal dysplasia, pseudoachondroplasia, multiple epiphyseal dysplasia, morquio syndrome, diastrophic dysplasia and Larson's Syndrome. Patients were followed clinically and radiographically for a mean of 7 years (range, 3–11.5 years). Knee pain and function improved in all 11 joints. Mean Knee Society clinical and function scores improved from 24 (range, 14–36) and 20 points (range, 5–40) preoperatively to 68 (range, 28–80) and 50 points (range, 22–74) respectively at final follow-up. Four complications were recorded (36%), including a patellar fracture following a fall, a tibial periprosthetic fracture, persistent anterior knee pain and a femoral component revision for aseptic loosening. Our results suggest that custom rotating-hinge TKR in patients with skeletal dysplasia is effective at relieving pain, optimising movement and improving function. It compensates for bony deformity and ligament deficiency and reduces the need for corrective osteotomy. Patellofemoral joint complications are frequent and functional outcome is worse than primary TKR in the general population. Submission endorsed by Mr Peter Calder, Consultant Orthopaedic Surgeon and Society member

STUDY DESIGN. Retrospective study of 8 children treated with vertical expandable prosthetic titanium rib (VEPTR) for correction of early onset spinal scoliotic deformities. METHODS. 8 children with progressive scoliosis due to a variety of conditions, 6 congenital (2 Goldenhar syndrome, 2 VACTERL syndrome, 2 congenital thoracic abnormalities), 1 spondyloepiphyseal dysplasia, 1 early onset of scoliosis, underwent the index procedure and subsequent lengthening procedures at 6 months intervals (1 patient had 11 lengthening procedures). Mean age was 4 years (2-6 years) and mean follow up 3.8 years (2-6 years). Mean preoperative Cobb angle was 64,8° (51-108) and mean postoperative angle 40° (31-50). RESULTS. There were no neurological complications. Three patients developed infection with wound breakdown. One patient underwent removal of one of the two VEPTR rods. In the other two patients the rods were removed followed by antibiotics and the VEPTR converted to another growing rod system. Overall, patients tolerated the multiple procedures well. Three patients experienced significant distress with multiple surgical procedures. CONCLUSION. VEPTR offers a viable treatment option for children with severe congenital and early onset scoliosis where there are no viable alternatives. It achieves and maintains spinal deformity correction, while allowing for continued spine and chest-wall growth. Complication rate is acceptable in view of the benefits. None of the complications have lead to long term complications to date, but the repeated lengthening have resulted in psychological disturbance which we are investigating further. An implantable driver would offer very significant advantages and would avoid some of the repeated operations

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.

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 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.

Purpose: Use of six-axis analysis and computer assisted deformity correction via a circular external fixator is a new method for deformity correction. We investigated its accuracy and safety in reconstruction of femoral deformity in children and young adults. Method: We retrospectively reviewed all cases including the indications for use and the methodology of application of the computer assisted six-axis analysis and circular external fixator for reconstruction of 22 femora in 20 patients. Twelve patients were female, and 8 were male. The average age was 13.9 (range, 5.9–24.6). Etiology included traumatic (7), idiopathic (6), multiple enchondromatosis (2), rickets (2), congenital femoral deficiency (2), spondyloepiphyseal dysplasia (1), congenital pseudohypoparathyroidisim (1), and multifocal osteomyelitis (1). Clinical and radiographic data were analyzed. Results: Average follow-up was 14.4 months (range, 4.5–32). Average time in frame was 6.2 months (range, 2.6–19). Bone lengthening of 3.9 cm (range, 1–8.5) was performed in 12 femora. In genu valgum patients, the mLDFA improved from a mean of 73.7° to a mean of 89°. In genu varum patients, the mLDFA improved from a mean of 99.8° to a mean of 89.5°. Complications included pin tract infection in 6, knee stiffness in 3, delayed union in 2, skin irritation in 1, posterior knee subluxation in 2, both of which had stable knees preoperatively. One patient was lost to follow-up and returned back with deformity. No complications occurred in 8 patients. Conclusion: Computer assisted femoral deformity correction with six-axis analysis and application of circular external fixator is a useful technique with the advantage of managing multiplanar deformities in children and young adults. It has the potential complications of the use of any external fixator. Close follow-up is necessary to avoid subluxation of the knee joint even in patients with stable knees. Accurate and safe correction can be achieved in almost all patients