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

Introduction. To evaluate the results of correction of knee deformities based on deformity analysis in Achondroplasia, the commonest skeletal dysplasia as some have concomitant ligamentous deformities. Materials and Methods. Retrospective study from a prospective database (2007–2020) of achondroplasts who underwent growth modulation. Analysis of medical records with objective measurement of mechanical axis radiographs was done (Traumacad). Satisfactory alignment was defined as neutral to slightly varus (0–15 mm MAD) so that the MCL/LCL laxity is not revealed. Results. 23 patients, 41 limbs, 34 bilateral, 6 unilateral underwent multiple growth modulation procedures. 2 had valgus knees. 15 patients underwent proximal fibular epiphysiodesis in addition for LCL laxity with one isolated fibular epiphysiodesis. Mechanical axis deviation (MAD) improved or normalised in 16 patients (70%). 4 patients were still undergoing correction. 4 patients needed further surgery out of which 2 patients were over 13 years when growth modulation was attempted and 2 needed correction of ankle varus. JLCA improved/ normalised in 12 patients (75%) with evidence of indirect LCL tightening and no improvement was seen in 4. The rate of correction was MAD 0.61mm/month, LDFA 0.29°/month and MPTA 0.13°/month; expectedly lower in achondroplasia due to lower growth velocity. Conclusions. This study highlights the pathology, application of growth modulation as per deformity analysis unlike previous studies. Proximal fibular epiphysiodesis improves LCL laxity in a majority of these children and is a simple procedure compared to our published series with indirect LCL tightening with frames

The use of total hip arthroplasty in adolescents for end-stage degenerative hip disease is controversial with few reported outcomes in the literature. The purpose of the study was to report the outcomes of total hip arthroplasty in a cohort of adolescents managed by a multidisciplinary team of paediatric and adult orthopaedic surgeons from a single centre. A multidisciplinary clinic consisting of paediatric and adult hip surgeons was established in 2013 to identify, treat and prospectively follow adolescents who would potentially beneift from a total hip arthoplasty. To date 29 consecutive adolescent patients (20 female, 9 male, 32 hips) with end stage degenerative hip disease have undergone cementless primary total hip arthroplasty. Mean age at decision to treat was 15.7 years (age 11.4–18.5). Ten different underlying paediatric specific pathologies were documented including DDH, cerebral palsy, JIA, skeletal dysplasias, SCFE and idiopathic chondrolysis. Three patients had bilateral staged procedures. Clinical baseline and outcomes were measured using Oxford Hip Score (OHS), WOMAC score and ASKp scores. Radiographic follow-up was conducted at 6 weeks, 6 months and yearly thereafter. Post-operative complications were recorded. Twenty-five hips were eligible for minimum 2-year follow-up scoring at a mean 2.7 years (2.0–3.6 years). No intraoperative complications occurred. One case of recalcitrant postoperative psoas tendonitis occured. OHS improved from 25 (7–43) to 41 (23–48). WOMAC improved from 48 (12–8) to 12 (1–44), and ASKp improved from 78% (33–98%) to 93% (73–100%). Radiographic review revealed acceptable alignment of all components with no signs of loosening or wear. No revision of components was required. One patient died at 6 months post op from an unrelated condition. The early term results of total hip arthroplasty in adolescents from our multidisciplinary program are encouraging. Improvements in quality of life to normal values were seen as were hip scores consistent with satisfactory joint function. Depite the range of complex and varied causes of arthritis seen in this population the establishment of combined paediatric and adult orthopaedic surgical teams appears to be a successful model to manage end-stage degenerative hip disease in adolescents

Aims

As an alternative to external fixators, intramedullary lengthening nails (ILNs) can be employed for distraction osteogenesis. While previous studies have demonstrated that typical complications of external devices, such as soft-tissue tethering, and pin site infection can be avoided with ILNs, there is a lack of studies that exclusively investigated tibial distraction osteogenesis with motorized ILNs inserted via an antegrade approach.

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.