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

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

Accurate skeletal age and final adult height prediction methods in paediatric orthopaedics are crucial for determining optimal timing of growth-guiding interventions and minimizing complications in treatments of various conditions. This study aimed to evaluate the accuracy of final adult height predictions using the central peak height (CPH) method with long leg X-rays and four different multiplier tables.

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

Genetic skeletal disorders constitute a rare and heterogeneous bone diseases often leading to poor quality of life. Several surgical options are available. The surgeon must deal with specific features (bone deformity, previous procedures, abnormal bone quality, stiffness or instability, muscle weakness). The questions concern the feasibility of the procedures and the surgical strategy. 55 patients (26 W, 29 M) were reviewed between 2016 and 2022. The mean age of the patients was 35 years (17–71). The diagnosis included 9 hereditary multiple exostoses, 8 osteogenesis imperfecta, 6 multiple epiphyseal dysplasia congenita, 6 achondroplasia, 4 osteopetrosis, 3 pycnodysostosis, 3 hypophosphatemic rickets, 3 fibrous dysplasia, 2 mucopolysaccharidosis, and 10 miscellaneous. 25 patients were referred for hip problems (40 hips). 4 patients (7 hips) requiring a THA have not been operated (4 planned). 4 patients (6 hips) had a proximal femoral fixation (2 osteotomies, 4 fracture fixations). 17 patients (27 hips) sustained a THA (25 primary, 2 revisions). All of them were operated by one operator, using a posterolateral approach and standard implants (including 7 dysplastic and 2 short stems). No customized implant has been used. As regard the 27 THAs, the mean follow up was 4.2 years (1–12). The early complications included 2 femoral cracks and 1 femur fracture. There were 2 revisions (1 cup loosening at 2 years, 1 stem loosening at 4 years). No infection nor dislocation occurred. All the patients were satisfied with their treatment and regain some autonomy. 3 THA were considered as unfeasible. Constitutional bone diseases need a multidisciplinary program of care. The indication for surgery is based on a mutual trust patient/surgeon, a careful evaluation of benefits/risks, and an accurate imaging to anticipate the difficulties. The expected results are a better function and quality of life, and a stability over time

Aims

Temporary hemiepiphysiodesis (HED) is applied to children and adolescents to correct angular deformities (ADs) in long bones through guided growth. Traditional Blount staples or two-hole plates are mainly used for this indication. Despite precise surgical techniques and attentive postoperative follow-up, implant-associated complications are frequently described. To address these pitfalls, a flexible staple was developed to combine the advantages of the established implants. This study provides the first results of guided growth using the new implant and compares these with the established two-hole plates and Blount staples.

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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.

Introduction

The effect of lower extremity lengthening on physeal growth is still controversial. We aim to compare data of the patients who had bilateral simultaneous femur and tibia lengthening with the patients who underwent the lengthening surgery separately for the femur and tibia at the end of completed skeletal maturation in terms of the effect of physeal growth.

Introduction. Angular deformity in the lower extremities can result in pain, gait disturbance, deformity and joint degeneration. Guided growth modulation uses the tension band principle with the goal of treatment being to normalise the mechanical axis. To assess the success of this procedure we reviewed our results in an attempt to identify patients who may not benefit from this simple and elegant procedure. Materials and Methods. We reviewed the surgical records and imaging in our tertiary children's hospital to identify all patients who had guided growth surgery since 2007. We noted the patient demographics, diagnosis, peri-operative experience and outcome. All patients were followed until skeletal maturity or until metalwork was removed. Results. 173 patients with 192 legs were assessed for eligibility. Six were excluded due to inadequate follow-up or loss of records. Of the 186 treated legs meeting criteria for final assessment 19.8% were unsuccessful, the other 80.2% were deemed successful at final follow up. Complications included infection and metal-work failure. Those with a pre-treatment diagnosis of idiopathic genu valgum/ varum had a success rate of 83.6%. Conclusions. In our hands, guided growth had an 80-percent success rate when all diagnosis were considered. Those procedures that were unlikely to be successful included growth disturbances due to mucopolysaccharide storage disease, Blounts disease and achondroplasia. Excluding those three diagnoses, success rate was 85.4%. We continue to advocate the use of guided growth as a successful treatment option for skeletally immature patients with limb deformity

Introduction. This study aims to evaluate the effect of using different types of fixator on the quality of callus and complications during distraction osteogenesis in patients with achondroplasia. Materials and Methods. Forty-nine achondroplasia patients with a minimum follow-up of 36 months who underwent limb lengthening between 2005 and 2017 with external fixator only were included. Thirty-three of the patients underwent lengthening using classical Ilizarov frame, while spatial frame used for sixteen. Regenerate quality is evaluated according to the Li classification on the X-ray taken one month after the end of the distraction. Complications were noted in the follow-up period. Results. The mean age at the time of surgery was 8,6 years. The mean external fixation index (EFI) was 34,3 and 30,1 day/cm for spatial frame and Ilizarov frame respectively. Mean follow-up period of 161,62 months and mean fixator period of 257 days. Amount of lengthening was 7,2 cm for Ilizarov frame, and 7,5 cm for spatial frame. Rate of callus with good morphological quality seen at consolidation was 72,4% and 50% for Ilizarov and spatial frames respectively. Two groups show similar results of complication rates in terms of pin site infection, premature fibular consolidation, regenerate fracture, plastic deformation, knee contracture. However fibular nonunion rates were higher for Ilizarov-type fixator. Conclusions. Although spatial frame with computer assistance brings easier follow-up for deformity correction, Ilizarov-type external fixator show slightly higher rates of good quality callus during consolidation for patients with achondroplasia

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

Introduction and Objective. Virtual Surgical Planning (VSP) is becoming an increasingly important means of improving skills acquisition, optimizing clinical outcomes, and promoting patient safety in orthopedics and traumatology. Pediatric Orthopedics (PO) often deals with the surgical treatment of congenital or acquired limbs and spine deformities during infancy. The objective is to restore function, improve aesthetics, and ensure proper residual growth of limbs and spine, using osteotomies, bone grafts, age-specific or custom-made hardware and implants. Materials and Methods. Three-dimensional (3D) digital models were generated from Computed Tomography (CT) scans, using free open-source software, and the surgery was planned and simulated starting from the 3D digital model. 3D printed sterilizable models were fabricated using a low-cost 3D printer, and animations of the operation were generated with the aim to accurately explain the operation to parents. All procedures were successfully planned using our VSP method and the 3D printed models were used during the operation, improving the understanding of the severely abnormal bony anatomy. Results. The surgery was precisely reproduced according to VSP and the deformities were successfully corrected in eight cases (3 genu varum in Blount disease, 2 coxa vara in pseudo achondroplasia, 1 SCFE, 1 missed Monteggia lesion and 1 post-traumatic forearm malunion deformity). In one case, a focal fibrocartilaginous dysplasia, the intraoperative intentional undersizing of the bone osteotomy produced an incomplete correction of a congenital forearm deformity. Conclusions. Our study describes the application of a safe, effective, user-friendly, VSP process in PO surgery. We are convinced that our study will stimulate the widespread adoption of this technological innovation in routine clinical practice for the treatment of rare congenital and post-traumatic limb deformities during childhood

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.

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The STRYDE nail is an evolution of the PRECICE Intramedullary Limb Lengthening System, with unique features regarding its composition. It is designed for load bearing throughout treatment in order to improve patient experience and outcomes and allow for simultaneous bilateral lower limb lengthening. The literature published to date is limited regarding outcomes and potential problems. We report on our early experience and raise awareness for the potential of adverse effects from this device.

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Severe spinal deformity in growing patients often requires surgical management. We describe the incidence of spinal deformity surgery in a National Health Service.

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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.

Although achondroplasia has been cited as the most common form of rhizomelic dwarfism, no report in the literature has given the data on the ratio of their upper and lower limb segments. We performed a paired study of 91achondroplasia patients with age and gender matched normal control group. Their upper and lower extremity radiographs were evaluated, and their radio-humeral and tibia-femoral ratios were compared. The ratios were compared using the Wilcoxon rank sum test. A p value of <0.05 was considered significant. The mean age of the patients was 15.8 years (95% confidence interval (CI), 13.1–18.6), and there were 45 males and 46 females. The radio-humeral ratio for the upper extremity of achondroplasia patients was 0.76 (95% CI, 0.75–0.76) which was significantly different from the normal control group of 0.79 (95% CI, 0.77–0.80) (p=0.001). The tibio-femoral ratio of the achondroplasia patients was 0.79 (95% CI, 0.78–0.79), which was not significantly different from the normal control group of 0.78 (95% CI, 0.77–0.79) (p=0.346). Rhizomelia is the predominant form of dwarfism in upper extremity, but not in lower extremity in achondroplasia. The functional deficits from these patients seem to come from the generalized limb shortness, and not specifically from rhizomelia

Introduction. Distraction histogenesis is utilised for cosmetic reasons in patients with constitutional short stature, achondroplasias, or height dysphoria. One of the considerations of patients and surgeons alike, is the preservation of aesthetically acceptable body proportions after the lengthening i.e which segment of the lower limb to lengthen and by how much, in order to preserve the patient's proportions within -or as close as possible to- the normal range of human body proportions. Sound anthropometric data on the normal range of the human body proportions is currently lacking. The aim of this study is to calculate the normal range of the ratio between the lengths of the lower limb, upper limb, femur, tibia, stature and trunk. Methods. Data form standardized measurements of the lengths of the tibia, femur, arm, stature, lower limb, upper limb was obtained from a sample population of 6068 healthy men and women servicing in the US Army. The mean, median, range and standard deviation were calculated for the following ratios of lengths: whole lower limb/leg, Stature/leg, Whole lower limb/thigh, Stature/thigh, Siting height/whole lower limb, Stature/Whole lower limb, Arm Span/Stature, Whole upper limb/Whole lower limb. Results. The ratios show normal distribution. Results are presented separately for men and women for the total sample population and for subgroups based on ethnic origin (White, Black, Hispanic and Asian). Discussion. This study is one of the first to provide specific anthropometric data from a large sample population relevant to the evidence-based preoperative planning and patient counselling before cosmetic limb lengthening procedures

Purpose. Angular deformity in the lower extremities can result in pain, gait disturbance, cosmetic deformity and joint degeneration. Up until the introduction of guided growth in 2007, which has since become the gold standard, treatment for correcting angular deformities in skeletally immature patients had been either an osteotomy, a hemiepiphysiodesis, or the use of staples. Methods. We reviewed the surgical records and diagnostic imaging in our childrens hospital to identify all patients who had guided growth surgery since 2007. All patients were followed until skeletal maturity or until their metalwork was removed. Results. 113 patients, with 147 legs were assessed for eligibility. Three were excluded for various reasons including inadequate follow-up or loss of records. Of the 144 treated legs which met the criteria for final assessment 32 (22.2%) were unsuccessful, the other 112 (77.8%) were deemed successful at final follow up. Complications were few, but included infection in one case and metal failure in another. Those with a pre-treatment diagnosis of idiopathic genu valgum/genu varum had a success rate of 83.6%. Conclusions. In our hands, guided growth had a seventy-eight percent success rate when all diagnosis were considered. Those procedures that were unlikely to be successful included growth disturbances due to mucopolysaccharide storage disease (28% failure rate), Blounts disease (66.6% failure rate) and achondroplasia (37.5% failure rate). If you exclude those three diagnoses, success rate for all other conditions was 81.4%. We continue to advocate the use of guided growth as a successful treatment option for skeletally immature patients with limb deformity