Aims. Periacetabular osteotomy (PAO) is an established treatment for acetabular dysplasia. It has also been proposed as a treatment for patients with acetabular retroversion. By reviewing a large cohort, we aimed to test whether outcome is equivalent for both types of morphology and identify factors that influenced outcome. Methods. A single-centre, retrospective cohort study was performed on patients with acetabular retroversion treated with PAO (n = 62 hips). Acetabular retroversion was diagnosed clinically and radiologically (presence of a crossover sign, posterior wall sign, lateral centre-edge angle (LCEA) between 20° and 35°). Outcomes were compared with a control group of patients undergoing PAO for dysplasia (LCEA < 20°; n = 86 hips). Femoral version was recorded. Patient-reported outcome measures (PROMs), complications, and reoperation rates were measured. Results. The mean Non-Arthritic Hip Score (NAHS) preoperatively was 58.6 (SD 16.1) for the dysplastic hips and 52.5 (SD 12.7) for the retroverted hips (p = 0.145). Postoperatively, mean NAHS was 83.0 (SD 16.9) and 76.7 (SD 17.9) for dysplastic and retroverted hips respectively (p = 0.041). Difference between pre- and postoperative NAHS was slightly lower in the retroverted hips (18.3 (SD 22.1)) compared to the dysplastic hips (25.2 (SD 15.2); p = 0.230). At mean 3.5 years’ follow-up (SD 1.9), one hip needed a revision PAO and no hips were converted to total hip arthroplasty (THA) in the retroversion group. In the control group, six hips (7.0%) were revised to THA. No differences in complications (p = 0.106) or in reoperation rate (p = 0.087) were seen. Negative predictors of outcome for patients undergoing surgery for retroversion were female sex, obesity, hypermobility, and severely decreased femoral anteversion. Conclusion. A PAO is an effective surgical intervention for acetabular retroversion and produces similar improvements when used to treat dysplasia. Femoral version should be routinely assessed in these patients and when extremely low (< 0°), as an additional procedure to address this abnormality may be necessary. Females with signs of hypermobility should also be consulted of the likely guarded improvement. Cite this article: Bone Jt Open 2021;2(9):757–764

Aims. Radiological residual acetabular dysplasia (RAD) has been reported in up to 30% of children who had successful brace treatment of infant developmental dysplasia of the hip (DDH). Predicting those who will resolve and those who may need corrective surgery is important to optimize follow-up protocols. In this study we have aimed to identify the prevalence and predictors of RAD at two years and five years post-bracing. Methods. This was a single-centre, prospective longitudinal cohort study of infants with DDH managed using a published, standardized Pavlik harness protocol between January 2012 and December 2016. RAD was measured at two years’ mean follow-up using acetabular index-lateral edge (AI-L) and acetabular index-sourcil (AI-S), and at five years using AI-L, AI-S, centre-edge angle (CEA), and acetabular depth ratio (ADR). Each hip was classified based on published normative values for normal, borderline (1 to 2 standard deviations (SDs)), or dysplastic (> 2 SDs) based on sex, age, and laterality. Results. Of 202 infants who completed the protocol, 181 (90%) had two and five years’ follow-up radiographs. At two years, in 304 initially pathological hips, the prevalence of RAD (dysplastic) was 10% and RAD (borderline) was 30%. At five years, RAD (dysplastic) decreased to 1% to 3% and RAD (borderline) decreased to < 1% to 2%. On logistic regression, no variables were predictive of RAD at two years. Only AI-L at two years was predictive of RAD at five years (p < 0.001). If both hips were normal at two years’ follow-up (n = 96), all remained normal at five years. In those with bilateral borderline hips at two years (n = 21), only two were borderline at five years, none were dysplastic. In those with either borderline-dysplastic or bilateral dysplasia at two years (n = 26), three (12%) were dysplastic at five years. Conclusion. The majority of patients with RAD at two years post-brace treatment, spontaneously resolved by five years. Therefore, children with normal radiographs at two years post-brace treatment can be discharged. Targeted follow-up for those with abnormal AI-L at two years will identify the few who may benefit from surgical correction at five years’ follow-up. Cite this article: Bone Joint J 2024;106-B(7):744–750

Aims. The purpose of this study was to explore the correlation between femoral torsion and morphology of the distal femoral condyle in patients with trochlear dysplasia and lateral patellar instability. Methods. A total of 90 patients (64 female, 26 male; mean age 22.1 years (SD 7.2)) with lateral patellar dislocation and trochlear dysplasia who were awaiting surgical treatment between January 2015 and June 2019 were retrospectively analyzed. All patients underwent CT scans of the lower limb to assess the femoral torsion and morphology of the distal femur. The femoral torsion at various levels was assessed using the a) femoral anteversion angle (FAA), b) proximal and distal anteversion angle, c) angle of the proximal femoral axis-anatomical epicondylar axis (PFA-AEA), and d) angle of the AEA–posterior condylar line (AEA-PCL). Representative measurements of distal condylar length were taken and parameters using the ratios of the bianterior condyle, biposterior condyle, bicondyle, anterolateral condyle, and anteromedial condyle were calculated and correlated with reference to the AEA, using the Pearson Correlation coefficient. Results. The femoral torsion had a strong correlation with distal condylar morphology. The FAA was significantly correlated with the ratio of the bianterior condyle (r = 0.355; p = 0.009), the AEA-PCL angle (r = 0.340; p = 0.001) and the ratio of the anterolateral condyle and lateral condyle (ALC-LC) (r = 0.309; p = 0.014). The PFA-AEA angle was also significantly correlated with the ratio of the bianterior condyle (r = 0.319; p = 0.008), the AEA-PCL angle (r = 0.231; p = 0.031), and the ratio of ALC-LC (r = 0.261; p = 0.034). In addition, the bianterior condyle ratio showed a significant correlation with the biposterior condyle ratio (r = -0.324; p = 0.027) and the AEA-PCL angle (r = 0.342; p = 0.021). Conclusion. Increased femoral torsion correlated with a prominent anterolateral condyle and a shorter posterolateral condyle compared with the medial condyle. The deformities of the anterior and posterior condyles are combined deformities rather than being isolated and individual deformities in patients with trochlear dysplasia and patella instability. Cite this article: Bone Joint J 2020;102-B(7):868–873

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. Methods. We retrospectively identified 20 patients with skeletal dysplasias and 292 with other syndromes (control group) who had completed surgical growth-friendly EOS treatment between 1 January 2000 and 31 December 2018. We compared radiological parameters, complications, and health-related quality of life (HRQoL) at mean follow-up of 8.6 years (SD 3.3) in the dysplasia group and 6.6 years (SD 2.6) in the control group. Results. Mean major curve correction per patient did not differ significantly between the dysplasia group (43%) and the control group (28%; p = 0.087). Mean annual spinal height increase was less in the dysplasia group (9.3 mm (SD 5.1) than in the control group (16 mm (SD 9.2); p < 0.001). Mean annual spinal growth adjusted to patient preoperative standing height during the distraction period was 11% in the dysplasia group and 14% in the control group (p = 0.070). The complication rate was 1.6 times higher (95% confidence interval (CI) 1.3 to 2.0) in the dysplasia group. The following complications were more frequent in the dysplasia group: neurological injury (rate ratio (RR) 5.1 (95% CI 2.3 to 11)), deep surgical site infection (RR 2.2 (95% CI 1.2 to 4.1)), implant-related complications (RR 2.0 (95% CI 1.5 to 2.7)), and unplanned revision (RR 1.8 (95% CI 1.3 to 2.5)). Final fusion did not provide additional spinal height compared with watchful waiting (p = 0.054). There were no significant differences in HRQoL scores between the groups. Conclusion. After growth-friendly EOS treatment, patients with skeletal dysplasias experienced a higher incidence of complications compared to those with other syndromes. Surgical growth-friendly treatment for skeletal dysplasia-associated EOS should be reserved for patients with severe, progressive deformities that are refractory to nonoperative treatment. Cite this article: Bone Joint J 2024;106-B(6):596–602

Aims. There has been limited literature regarding outcomes of acetabular rim syndrome (ARS) with persistent acetabular os in the setting of acetabular dysplasia. The purpose of this study was to characterize a cohort of adolescent and young adult patients with ARS with persistent os and compare their radiological and clinical outcomes to patients with acetabular dysplasia without an os. Methods. We reviewed a prospective database of patients undergoing periacetabular osteotomy (PAO) for symptomatic acetabular dysplasia between January 1999 and December 2021 to identify hips with preoperative os acetabuli, defined as a closed triradiate cartilage but persistence of a superolateral os acetabulum. A total of 14 hips in 12 patients with persistent os acetabuli (ARS cohort) were compared to 50 randomly selected ‘control’ hips without persistent os acetabuli. Preoperative and postoperative radiographs were measured for markers of dysplasia: lateral centre-edge angle, anterior centre-edge angle, acetabular inclination, and migration index. Union of the os was determined in patients with ≥ six months’ follow-up. Patient-reported outcome measures (PROMs) included the University of California, Los Angeles (UCLA) activity score and modified Harris Hip Score (mHHS, maximum score 80) completed at one year postoperatively. Results. There was no significant difference between the ARS and control cohorts with regard to sex distribution (p = 0.270), age at surgery (p = 0.186), or BMI (p = 0.442). Preoperatively, the ARS cohort had more severe dysplasia, including lower lateral centre-edge angle (-9.3° (SD 12.5°) vs 7.6° (SD 10.7°); p < 0.001) and greater acetabular inclination (33.9° (SD 8.2°) vs 21.8° (SD 8.7°); p < 0.001). Postoperatively, 6/13 (46%) hips with ≥ six months of radiological follow up demonstrated union of the os. There was no difference between the ARS and control cohorts at one year in UCLA score (9.1 (SD 1.5) vs 8.2 (SD 1.9); p = 0.167) or mHHS (73.4 (SD 9.5) vs 69.9 (SD 8.8); p = 0.312). Conclusion. ARS with persistent acetabular os may occur in more severe dysplasia. Union occurs in 50% cases following corrective PAO. Cite this article: Bone Joint J 2024;106-B(12):1393–1398

Aims. We investigated the prevalence of late developmental dysplasia of the hip (DDH), abduction bracing treatment, and surgical procedures performed following the implementation of universal ultrasound screening versus selective ultrasound screening programmes. Methods. A systematic search of PubMed, Embase, The Cochrane Library, OrthoSearch, and Web of Science from the date of inception of each database until 27 March 2022 was performed. The primary outcome of interest was the prevalence of late detection of DDH, diagnosed after three months. Secondary outcomes of interest were the prevalence of abduction bracing treatment and surgical procedures performed in childhood for dysplasia. Only studies describing the primary outcome of interest were included. Results. A total of 31 studies were identified, of which 13 described universal screening and 20 described selective screening. Two studies described both. The prevalence of late DDH was 0.10 per 1,000 live births (95% confidence interval (CI) 0.00 to 0.39) in the universal screening group and 0.45 per 1,000 live births (95% CI 0.31 to 0.61) in the selective screening group. Abduction bracing treatment was performed on 55.54 per 1,000 live births (95% CI 24.46 to 98.15) in the universal screening group versus 0.48 per 1,000 live births (95% CI 0.07 to 1.13) in the selective screening group. Both the universal and selective screening groups had a similar prevalence of surgical procedures in childhood for dysplasia being performed (0.48 (95% CI 0.32 to 0.63) vs 0.49 (95% CI 0.31 to 0.71) per 1,000 live births, respectively). Conclusion. Universal screening showed a trend towards lower prevalence of late DDH compared to selective screening. However, it was also associated with a significant increase in the prevalence of abduction bracing without a significant reduction in the prevalence of surgical procedures in childhood for dysplasia being performed. High-quality studies comparing both treatment methods are required, in addition to studies into the natural history of missed DDH. Cite this article: Bone Joint J 2023;105-B(2):198–208

Aims. Hip arthroscopy has gained prominence as a primary surgical intervention for symptomatic femoroacetabular impingement (FAI). This study aimed to identify radiological features, and their combinations, that predict the outcome of hip arthroscopy for FAI. Methods. A prognostic cross-sectional cohort study was conducted involving patients from a single centre who underwent hip arthroscopy between January 2013 and April 2021. Radiological metrics measured on conventional radiographs and magnetic resonance arthrography were systematically assessed. The study analyzed the relationship between these metrics and complication rates, revision rates, and patient-reported outcomes. Results. Out of 810 identified hip arthroscopies, 359 hips were included in the study. Radiological risk factors associated with unsatisfactory outcomes after cam resection included a dysplastic posterior wall, Tönnis grade 2 or higher, and over-correction of the α angle. The presence of acetabular retroversion and dysplasia were also significant predictors for worse surgical outcomes. Notably, over-correction of both cam and pincer deformities resulted in poorer outcomes than under-correction. Conclusion. We recommend caution in performing hip arthroscopy in patients who have three positive acetabular retroversion signs. Acetabular dysplasia with a lateral centre-edge angle of less than 20° should not be treated with isolated hip arthroscopy. Acetabular rim-trimming should be avoided in patients with borderline dysplasia, and care should be taken to avoid over-correction of a cam deformity and/or pincer deformity. Cite this article: Bone Joint J 2024;106-B(8):775–782

Aims. Metal-on-metal hip resurfacing (MoM-HR) has seen decreased usage due to safety and longevity concerns. Joint registries have highlighted the risks in females, smaller hips, and hip dysplasia. This study aimed to identify if reported risk factors are linked to revision in a long-term follow-up of MoM-HR performed by a non-designer surgeon. Methods. A retrospective review of consecutive MoM hip arthroplasties (MoM-HRAs) using Birmingham Hip Resurfacing was conducted. Data on procedure side, indication, implant sizes and orientation, highest blood cobalt and chromium ion concentrations, and all-cause revision were collected from local and UK National Joint Registry records. Results. A total of 243 hips (205 patients (163 male, 80 female; mean age at surgery 55.3 years (range 25.7 to 75.3)) with MoM-HRA performed between April 2003 and October 2020 were included. Mean follow-up was 11.2 years (range 0.3 to 17.8). Osteoarthritis was the most common indication (93.8%), and 13 hips (5.3%; 7M:6F) showed dysplasia (lateral centre-edge angle < 25°). Acetabular cups were implanted at a median of 45.4° abduction (interquartile range 41.9° - 48.3°) and stems neutral or valgus to the native neck-shaft angle. In all, 11 hips (4.5%; one male, ten females) in ten patients underwent revision surgery at a mean of 7.4 years (range 2.8 to 14.2), giving a cumulative survival rate of 94.8% (95% confidence interval (CI) 91.6% to 98.0%) at ten years, and 93.4% (95% CI 89.3% to 97.6%) at 17 years. For aseptic revision, male survivorship was 100% at 17 years, and 89.6% (95% CI 83.1% to 96.7%) at ten and 17 years for females. Increased metal ion levels were implicated in 50% of female revisions, with the remaining being revised for unexplained pain or avascular necrosis. Conclusion. The Birmingham MoM-HR showed 100% survivorship in males, exceeding the National Institute for Health and Care Excellence ‘5% at ten years’ threshold. Female sex and small component sizes are independent risk factors. Dysplasia alone is not a contraindication to resurfacing. Cite this article: Bone Jt Open 2023;4(11):853–858

Aims. Pelvic tilt is believed to affect the symptomology of osteoarthritis (OA) of the hip by alterations in joint movement, dysplasia of the hip by modification of acetabular cover, and femoroacetabular impingement by influencing the impingement-free range of motion. While the apparent role of pelvic tilt in hip pathology has been reported, the exact effects of many forms of treatment on pelvic tilt are unknown. The primary aim of this study was to investigate the effects of surgery on pelvic tilt in these three groups of patients. Methods. The demographic, radiological, and outcome data for all patients operated on by the senior author between October 2016 and January 2020 were identified from a prospective registry, and all those who underwent surgery with a primary diagnosis of OA, dysplasia, or femoroacetabular impingement were considered for inclusion. Pelvic tilt was assessed on anteroposterior (AP) standing radiographs using the pre- and postoperative pubic symphysis to sacroiliac joint (PS-SI) distance, and the outcomes were assessed with the Hip Outcome Score (HOS), International Hip Outcome Tool (iHOT-12), and Harris Hip Score (HHS). Results. The linear regression model revealed a significant negative predictive association between the standing pre- and postoperative PS-SI distances for all three groups of patients (all p < 0.001). There was a significant improvement in all three outcome measures between the pre- and postoperative values (p < 0.05). Conclusion. There is a statistically significant decrease in pelvic tilt after surgery in patients with OA of the hip, dysplasia, and femoroacetabular impingement. These results confirm that surgery significantly alters the pelvic orientation. Pelvic tilt significantly decreased after total hip arthroplasty, periacetabular osteotomy, and arthroscopy/surgical hip dislocation. The impact of surgery on pelvic tilt should be considered within the therapeutic plan in order to optimize pelvic orientation in these patients. Cite this article: Bone Joint J 2022;104-B(9):1025–1031

Aims. The Oswestry-Bristol Classification (OBC) was recently described as an MRI-based classification tool for the femoral trochlear. The authors demonstrated better inter- and intraobserver agreement compared to the Dejour classification. As the OBC could potentially provide a very useful MRI-based grading system for trochlear dysplasia, it was the aim to determine the inter- and intraobserver reliability of the classification system from the perspective of the non-founder. Methods. Two orthopaedic surgeons independently assessed 50 MRI scans for trochlear dysplasia and classified each according to the OBC. Both observers repeated the assessments after six weeks. The inter- and intraobserver agreement was determined using Cohen’s kappa statistic and S-statistic nominal and linear weights. Results. The OBC with grading into four different trochlear forms showed excellent inter- and intraobserver agreement with a mean kappa of 0.78. Conclusion. The OBC is a simple MRI-based classification system with high inter- and intraobserver reliability. It could present a useful tool for grading the severity of trochlear dysplasia in daily practice. Cite this article: Bone Joint Open 2020;1-7:355–358

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. Patients and Methods. A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. Results. Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). Conclusion. Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563–1569

Aims. The aim of this study was to investigate the agreement in interpretation of the quality of the paediatric hip ultrasound examination, the reliability of geometric and morphological assessment, and the relationship between these measurements. Methods. Four investigators evaluated 60 hip ultrasounds and assessed their quality based the standard plane of Graf et al. They measured geometric parameters, described the morphology of the hip, and assigned the Graf grade of dysplasia. They analyzed one self-selected image and one randomly selected image from the ultrasound series, and repeated the process four weeks later. The intra- and interobserver agreement, and correlations between various parameters were analyzed. Results. In the assessment of quality, there a was moderate to substantial intraobserver agreement for each element investigated, but interobserver agreement was poor. Morphological features showed weak to moderate agreement across all parameters but improved to significant when responses were reduced. The geometric measurements showed nearly perfect agreement, and the relationship between them and the morphological features showed a dose response across all parameters with moderate to substantial correlations. There were strong correlations between geometric measurements. The Graf classification showed a fair to moderate interobserver agreement, and moderate to substantial intraobserver agreement. Conclusion. This investigation into the reliability of the interpretation of hip ultrasound scans identified the difficulties in defining what is a high-quality ultrasound. We confirmed that geometric measurements are reliably interpreted and may be useful as a further measurement of quality. Morphological features are generally poorly interpreted, but a simpler binary classification considerably improves agreement. As there is a clear dose response relationship between geometric and morphological measurements, the importance of morphology in the diagnosis of hip dysplasia should be questioned. Cite this article: Bone Joint J 2023;105-B(10):1123–1130

We made a prospective longitudinal clinical and radiological study of 18 children diagnosed as having dysplasia epiphysealis capitis femoris. Half the cases were bilateral. Boys were affected five times more often than girls. There were no symptoms or clinical signs in most but some of the bilateral cases had an inconsistent waddling gait. The imaging studies suggest that the cartilaginous proximal femoral epiphysis is hypoplastic, with delayed appearance of single or multiple ossification centres. Progressive improvement occurred and at an average age of five years and six months, there was complete fusion of all the ossific nuclei and normal density and texture of the epiphyseal bone. The end result was a round epiphysis with a slightly diminished height. The dysplasia is attributed to focal hypoplasia of the proximal femoral epiphysis

Aims. The Oswestry-Bristol Classification (OBC) is an MRI-specific assessment tool to grade trochlear dysplasia. The aim of this study is to validate clinically the OBC by demonstrating its use in selecting treatments that are safe and effective. Methods. The OBC and the patellotrochlear index were used as part of the Oswestry Patellotrochlear Algorithm (OPTA) to guide the surgical treatment of patients with patellar instability. Patients were assigned to one of four treatment groups: medial patellofemoral ligament reconstruction (MPFLr); MPFLr + tibial tubercle distalization (TTD); trochleoplasty; or trochleoplasty + TTD. A prospective analysis of a longitudinal patellofemoral database was performed. Between 2012 and 2018, 202 patients (233 knees) with a mean age of 24.2 years (SD 8.1), with recurrent patellar instability were treated by two fellowship-trained consultant sports/knee surgeons at The Robert Jones and Agnes Hunt Orthopaedic Hospital. Clinical efficacy of each treatment group was assessed by Kujala, International Knee Documentation Committee (IKDC), and EuroQol five-dimension questionnaire (EQ-5D) scores at baseline, and up to 60 months postoperatively. Their safety was assessed by complication rate and requirement for further surgery. The pattern of clinical outcome over time was analyzed using mixed regression modelling. Results. In all, 135 knees (mean age 24.9 years (SD 9.4)) were treated using a MPFLr. Ten knees (7.4%) required additional surgery. A total of 50 knees (mean age 24.4 years (SD 6.3)) were treated using MPFLr + TTD. Ten (20%) required additional surgery. A total of 20 knees (mean age 19.5 years (SD 3.0)) were treated using trochleoplasty + TTD. Three patients (15%) required additional surgery. In each treatment group, there was a significant improvement in Kujala, IKDC, and EQ-5D at one year postoperatively (p < 0.001) with a recognized level of overall complication rate. Conclusion. The OBC is a valid assessment tool to grade patients with trochlear dysplasia and, when used as part of the OPTA, helps to determine treatments that are safe and effective. This fulfils the requirements for its application in mainstream clinical practice. Cite this article: Bone Joint J 2021;103-B(10):1586–1594

Aims. The aim of the current study was to assess the reliability of the Ottawa classification for symptomatic acetabular dysplasia. Methods. In all, 134 consecutive hips that underwent periacetabular osteotomy were categorized using a validated software (Hip2Norm) into four categories of normal, lateral/global, anterior, or posterior. A total of 74 cases were selected for reliability analysis, and these included 44 dysplastic and 30 normal hips. A group of six blinded fellowship-trained raters, provided with the classification system, looked at these radiographs at two separate timepoints to classify the hips using standard radiological measurements. Thereafter, a consensus meeting was held where a modified flow diagram was devised, before a third reading by four raters using a separate set of 74 radiographs took place. Results. Intrarater results per surgeon between Time 1 and Time 2 showed substantial to almost perfect agreement among the raters (κappa = 0.416 to 0.873). With respect to inter-rater reliability, at Time 1 and Time 2 there was substantial agreement overall between all surgeons (Time 1 κappa = 0.619; Time 2 κappa = 0.623). Posterior and anterior rating categories had moderate and fair agreement at Time 1 (posterior κappa = 0.557; anterior κappa = 0.438) and Time 2 (posterior κappa = 0.506; anterior κappa = 0.250), respectively. At Time 3, overall reliability (κappa = 0.687) and posterior and anterior reliability (posterior κappa = 0.579; anterior κappa = 0.521) improved from Time 1 and Time 2. Conclusion. The Ottawa classification system provides a reliable way to identify three categories of acetabular dysplasia that are well-aligned with surgical management. The term ‘borderline dysplasia’ should no longer be used. Cite this article: Bone Joint Res. 2020;9(5):242–249

Aims. Classifying trochlear dysplasia (TD) is useful to determine the treatment options for patients suffering from patellofemoral instability (PFI). There is no consensus on which classification system is more reliable and reproducible for the purpose of guiding clinicians’ management of PFI. There are also concerns about the validity of the Dejour Classification (DJC), which is the most widely used classification for TD, having only a fair reliability score. The Oswestry-Bristol Classification (OBC) is a recently proposed system of classification of TD, and the authors report a fair-to-good interobserver agreement and good-to-excellent intraobserver agreement in the assessment of TD. The aim of this study was to compare the reliability and reproducibility of these two classifications. Methods. In all, six assessors (four consultants and two registrars) independently evaluated 100 axial MRIs of the patellofemoral joint (PFJ) for TD and classified them according to OBC and DJC. These assessments were again repeated by all raters after four weeks. The inter- and intraobserver reliability scores were calculated using Cohen’s kappa and Cronbach’s α. Results. Both classifications showed good to excellent interobserver reliability with high α scores. The OBC classification showed a substantial intraobserver agreement (mean kappa 0.628; p < 0.005) whereas the DJC showed a moderate agreement (mean kappa 0.572; p < 0.005). There was no significant difference in the kappa values when comparing the assessments by consultants with those by registrars, in either classification system. Conclusion. This large study from a non-founding institute shows both classification systems to be reliable for classifying TD based on axial MRIs of the PFJ, with the simple-to-use OBC having a higher intraobserver reliability score than that of the DJC. Cite this article: Bone Jt Open 2023;4(7):532–538

Metal-on-metal hip resurfacing was performed for developmental dysplasia in 96 hips in 85 patients, 78 in women and 18 in men, with a mean age at the time of surgery of 43 years (14 to 65). These cases were matched for age, gender, operating surgeon and date of operation with a group of patients with primary osteoarthritis who had been treated by resurfacing, to provide a control group of 96 hips (93 patients). A clinical and radiological follow-up study was performed. The dysplasia group were followed for a mean of 4.4 years (2.0 to 8.5) and the osteoarthritis group for a mean of 4.5 years (2.2 to 9.4). Of the dysplasia cases, 17 (18%) were classified as Crowe grade III or IV. There were five (5.2%) revisions in the dysplasia group and none in the osteoarthritic patients. Four of the failures were due to acetabular loosening and the other sustained a fracture of the neck of femur. There was a significant difference in survival between the two groups (p = 0.02). The five-year survival was 96.7% (95% confidence interval 90.0 to 100) for the dysplasia group and 100% (95% confidence interval 100 to 100) for the osteoarthritic group. There was no significant difference in the median Oxford hip score between the two groups at any time during the study. The medium-term results of metal-on-metal hip resurfacing in all grades of developmental dysplasia are encouraging, although they are significantly worse than in a group of matched patients with osteoarthritis treated in the same manner

In a longitudinal case-control study, we followed 81 subjects with dysplasia of the hip and 136 control subjects without dysplasia for ten years assessing radiological evidence of degeneration of the hip at admission and follow-up. There were no cases of subluxation in the group with dysplasia. Neither subjects with dysplasia nor controls had radiological signs of ongoing degenerative disease at admission. The primary radiological discriminator of degeneration of the hip was a change in the minimum joint space width over time. There were no significant differences between these with dysplasia and controls in regard to age, body mass index or occupational exposure to daily repeated lifting at admission. We found no significant differences in the reduction of the joint space width at follow-up between subjects with dysplasia and the control subjects nor in self-reported pain in the hip. The association of subluxation and/or associated acetabular labral tears with dysplasia of the hip may be a conditional factor for the development of premature osteoarthritis in mildly to moderately dysplastic hips

Patients with acetabular dysplasia commonly undergo peri-acetabular osteotomy after skeletal maturity to reduce the risk of the late development of osteoarthritis. Several studies have suggested that deformity of the femoral head influences the long-term outcome. We radiologically examined 224 hips in 112 patients with acetabular dysplasia and early-stage osteoarthritis. There were 103 women and nine men with a mean age of 37.6 years (18 to 49). A total of 201 hips were placed in the acetabular dysplasia group and 23 in a normal group. The centre–edge angle and acetabular head index were significantly smaller (both p < 0.001), and the acetabular angle, acetabular roof angle and roundness index were significantly greater in the acetabular dysplasia group than those in the normal group (all p < 0.001). There were significant correlations between the roundness index and other parameters. Femoral head shape may be influenced by the severity of the acetabular dysplasia. Cite this article: Bone Joint J 2013;95-B:1192–6

Monostotic fibrous dysplasia of the proximal femur has a variable clinical course, despite its reported limited tendency to progress. We investigated the natural history and predisposing factors for progression of dysplasia in a group of 76 patients with a mean follow-up of 8.5 years (2.0 to 15.2). Of these, 31 (41%) presented with an asymptomatic incidental lesion while 45 (59%) presented with pain or a pathological fracture. A group of 23 patients (30%) underwent early operative treatment for pain (19: 25%) or pathological fracture (4: 5%). Of the 53 patients who were initially treated non-operatively, 45 (85%) remained asymptomatic but eight (15%) needed surgery because of pain or fracture. The progression-free survival of the observation group was 81% (. sd. 6.4%) at five-years follow-up. An initial presentation of pain (p < 0.001), a limp (p < 0.001), radiological evidence of microfracture (p = 0.001) and younger age (< 17 years) (p = 0.016) were significant predisposing factors for disease progression. The risk of experiencing pain or pathological fracture is considerable in monostotic fibrous dysplasia of the proximal femur. Patients presenting with pain, a limp or radiological evidence of microfracture have a high chance of needing surgical treatment. Cite this article: Bone Joint J 2014;96-B:673–6

Aims. The objective of this study was to present the outcomes of rotational acetabular osteotomy (RAO) over a 30-year period for osteoarthritis (OA) secondary to dysplasia of the hip in pre- or early-stage OA. Methods. Between September 1987 and December 1994, we provided treatment to 47 patients (55 hips) with RAO for the management of pre- or early-stage OA due to developmental hip dysplasia. Of those, eight patients (11 hips) with pre-OA (follow-up rate 79%) and 27 patients (32 hips) with early-stage OA (follow-up rate 78%), totalling 35 patients (43 hips) (follow-up rate 78%), were available at a minimum of 28 years after surgery. Results. In the pre-OA group, the mean Merle d'Aubigné score improved significantly from 14.5 points (SD 0.7) preoperatively to 17.4 points at final follow-up (SD 1.2; p = 0.004) and in the early-stage group, the mean score did not improve significantly from 14.0 (SD 0.3) to 14.6 (SD 2.4; p = 0.280). Radiologically, the centre-edge angle, acetabular roof angle, and head lateralization index were significantly improved postoperatively in both groups. Radiological progression of OA was observed in two patients (two hips) in the pre-OA group and 17 patients (18 hips) in the early-stage group. Kaplan-Meier survival analysis, with radiological progression of OA as the primary outcome, projected a 30-year survival rate of 81.8% (95% confidence interval (CI) 0.59 to 1.00) for the pre-OA group and 42.2% (95% CI 0.244 to 0.600) for the early-stage group. In all cases, the overall survival rate stood at 51.5% (95% CI 0.365 to 0.674) over a 30-year period, and when the endpoint was conversion to total hip arthroplasty, the survival rate was 74.0% (95% CI 0.608 to 0.873). Conclusion. For younger patients with pre-OA, joint preservation of over 30 years can be expected after RAO. Cite this article: Bone Joint J 2024;106-B(5 Supple B):25–31

Aims. Some patients presenting with hip pain and instability and underlying acetabular dysplasia (AD) do not experience resolution of symptoms after surgical management. Hip-spine syndrome is a possible underlying cause. We hypothesized that there is a higher frequency of radiological spine anomalies in patients with AD. We also assessed the relationship between radiological severity of AD and frequency of spine anomalies. Methods. In a retrospective analysis of registry data, 122 hips in 122 patients who presented with hip pain and and a final diagnosis of AD were studied. Two observers analyzed hip and spine variables using standard radiographs to assess AD. The frequency of lumbosacral transitional vertebra (LSTV), along with associated Castellvi grade, pars interarticularis defect, and spinal morphological measurements were recorded and correlated with radiological severity of AD. Results. Out of 122 patients, 110 (90.2%) were female and 12 (9.8%) were male. We analyzed the radiographs of 122 hips (59 (48.4%) symptomatic left hips, and 63 (51.6%) symptomatic right hips). Average age at time of presentation was 34.2 years (SD 11.2). Frequency of LSTV was high (39% to 43%), compared to historic records from the general population, with Castellvi type 3b being the most common (60% to 63%). Patients with AD have increased L4 and L5 interpedicular distance compared to published values. Frequency of pars interarticularis defect was 4%. Intraclass correlation coefficient for hip and spine variables assessed ranged from good (0.60 to 0.75) to excellent (0.75 to 1.00). Severity of AD did not demonstrate significant correlation with frequency of radiological spine anomalies. Conclusion. Patients with AD have increased frequency of spinal anomalies seen on standard hip radiographs. However, there exists no correlation between radiological severity of AD and frequency of spine anomalies. In managing AD patients, clinicians should also assess spinal anomalies that are easily found on standard hip radiographs. Cite this article: Bone Joint J 2021;103-B(8):1351–1357

Aims. The aim of this study was to evaluate cartilaginous patellotrochlear congruence and patellofemoral alignment parameters after deepening trochleoplasty in severe trochlear dysplasia. Patients and Methods. The study group comprised 20 patients (two male, 18 female; mean age 24 years (16 to 39)) who underwent deepening trochleoplasty and medial patellofemoral ligament (MPFL) reconstruction for the treatment of recurrent lateral patellar dislocation due to severe trochlear dysplasia (Dejour type B to D). Pre- and postoperative MRI investigations of the study group were compared with MRI data of 20 age- and gender-matched control patients (two male, 18 female; mean age 27 years (18 to 44)) regarding the patellotrochlear contact ratio, patellotrochlear contact area, posterior patellar edge-trochlear groove ratio, and patellar tilt. Results. Preoperatively, all parameters in the study group indicated significant patellar malalignment, including a reduced patellotrochlear contact ratio (mean 0.38 (0.13 to 0.59) vs mean 0.67 (0.48 to 0.88); p < 0.001), reduced contact area (mean 128 mm. 2. (87 to 190) vs mean 183 mm. 2. (155 to 227); p < 0.001), increased patellar lateralization (mean 0.82 (0.55 to 1.0) vs mean 0.99 (0.93 to 1.1); p < 0.001) and increased patellar tilt (mean 25.8° (8.1° to 43.0°) vs mean 9.1° (0.5° to 16.8°); p < 0.001). Postoperatively, all parameters in the study group improved significantly and reached normal values compared with the control group. The patellotrochlear contact ratio increased to a mean of 0.63 (0.38 to 0.85) (p < 0.001), the contact area increased to a mean of 187 mm. 2. (101 to 255) (p < 0.001), the posterior patellar edge-trochlear groove ratio improved to a mean of 0.96 (0.83 to 0.91) (p < 0.001), and the patellar tilt decreased to a mean of 13.9° (8.1° to 22.1°) (p < 0.001) postoperatively. Conclusion. Deepening trochleoplasty and MPFL reconstruction normalized the patellotrochlear congruence and patellofemoral alignment parameters. Modifying the dysplastic trochlea involves approximating the trochlear morphology to the shape of the patella rather than inducing a patella and trochlea mismatch. Cite this article: Bone Joint J 2019;101-B:325–330

Objectives. We have previously investigated an association between the genome copy number variation (CNV) and acetabular dysplasia (AD). Hip osteoarthritis is associated with a genetic polymorphism in the aspartic acid repeat in the N-terminal region of the asporin (ASPN) gene; therefore, the present study aimed to investigate whether the CNV of ASPN is involved in the pathogenesis of AD. Methods. Acetabular coverage of all subjects was evaluated using radiological findings (Sharp angle, centre-edge (CE) angle, acetabular roof obliquity (ARO) angle, and minimum joint space width). Genomic DNA was extracted from peripheral blood leukocytes. Agilent’s region-targeted high-density oligonucleotide tiling microarray was used to analyse 64 female AD patients and 32 female control subjects. All statistical analyses were performed using EZR software (Fisher’s exact probability test, Pearson’s correlation test, and Student’s t-test). Results. CNV analysis of the ASPN gene revealed a copy number loss in significantly more AD patients (9/64) than control subjects (0/32; p = 0.0212). This loss occurred within a 60 kb region on 9q22.31, which harbours the gene for ASPN. The mean radiological parameters of these AD patients were significantly worse than those of the other subjects (Sharp angle, p = 0.0056; CE angle, p = 0.0076; ARO angle, p = 0.0065), and all nine patients required operative therapy such as total hip arthroplasty or pelvic osteotomy. Moreover, six of these nine patients had a history of operative or conservative therapy for developmental dysplasia of the hip. Conclusions. Copy number loss within the region harbouring the ASPN gene on 9q22.31 is associated with severe AD. A copy number loss in the ASPN gene region may play a role in the aetiology of severe AD. Cite this article: T. Sekimoto, M. Ishii, M. Emi, S. Kurogi, T. Funamoto, Y. Yonezawa, T. Tajima, T. Sakamoto, H. Hamada, E. Chosa. Copy number loss in the region of the ASPN gene in patients with acetabular dysplasia: ASPN CNV in acetabular dysplasia. Bone Joint Res 2017;6:439–445. DOI: 10.1302/2046-3758.67.BJR-2016-0094.R1

The aim of this study was to investigate the incidence of dysplasia in the ‘normal’ contralateral hip in patients with unilateral developmental dislocation of the hip (DDH) and to evaluate the long-term prognosis of such hips. A total of 48 patients (40 girls and eight boys) were treated for late-detected unilateral DDH between 1958 and 1962. After preliminary skin traction, closed reduction was achieved at a mean age of 17.8 months (4 to 65) in all except one patient who needed open reduction. In 25 patients early derotation femoral osteotomy of the contralateral hip had been undertaken within three years of reduction, and later surgery in ten patients. Radiographs taken during childhood and adulthood were reviewed. The mean age of the patients was 50.9 years (43 to 55) at the time of the latest radiological review. In all, eight patients (17%) developed dysplasia of the contralateral hip, defined as a centre-edge (CE) angle < 20° during childhood or at skeletal maturity. Six of these patients underwent surgery to improve cover of the femoral head; the dysplasia improved in two after varus femoral osteotomy and in two after an acetabular shelf operation. During long-term follow-up the dysplasia deteriorated to subluxation in two patients (CE angles 4° and 5°, respectively) who both developed osteoarthritis (OA), and one of these underwent total hip replacement at the age of 49 years. In conclusion, the long-term prognosis for the contralateral hip was relatively good, as OA occurred in only two hips (4%) at a mean follow-up of 50 years. Regular review of the ‘normal’ side is indicated, and corrective surgery should be undertaken in those who develop subluxation. Cite this article: Bone Joint J 2014; 96-B:1161–6

1. The examination of fifty-one infants presenting signs of unilateral dysplasia (preluxation) of the hip disclosed the existence of an abduction-contracture in the opposite "normal" hip. 2. The abduction-contracture was not infrequently found in the newborn, but seemed to attract attention mostly between the second and fifth month of life. It was seldom found after the sixth month. 3. The dysplasia in the contralateral hip showed the usual features. 4. The relationship between the degree of abduction-contracture and the degree of dysplasia was a direct one only occasionally; in most cases they seemed unrelated to each other. 5. Observation of these patients showed that the abduction-contracture followed a constant course towards gradual and spontaneous regression. The dysplasia progressed in one of the following directions: spontaneous recovery, rapid or slow recovery with treatment by abduction splint, subluxation, or true dislocation. 6. It is submitted that the coexistence of unilateral dysplasia of the hip and abduction-contracture in the opposite hip is not fortuitous. It is believed that the abduction-contracture determines the development of the dysplasia in the opposite hip through the faulty mechanics caused by "fixed pelvic obliquity.". 7. The discovery of unilateral abduction-contracture soon after birth should be a warning that dysplasia may develop in the opposite hip. A careful watch should therefore be kept for signs of abduction-contracture and for the later development of dysplasia

Aims. The aim of this study was to investigate the changes in femoral trochlear morphology following surgical correction of recurrent patellar dislocation associated with trochlear dysplasia in children. Patients and Methods. A total of 23 patients with a mean age of 9.6 years (7 to 11) were included All had bilateral recurrent patellar dislocation associated with femoral trochlear dysplasia. The knee with traumatic dislocation at the time of presentation or that had dislocated most frequently was treated with medial patellar retinacular plasty (Group S). The contralateral knee served as a control and was treated conservatively (Group C). All patients were treated between October 2008 and August 2013. The mean follow-up was 48.7 months (43 to 56). Axial CT scans were undertaken in all patients to assess the trochlear morphological characteristics on a particular axial image which was established at the point with the greatest epicondylar width based on measurements preoperatively and at the final follow-up. Results. Preoperatively, there were no statistically significant differences between the trochlear morphology in the two groups (sulcus angle, p 0.852; trochlear groove depth, p 0.885; lateral trochlear inclination, p 0.676; lateral-to-medial facet ratio, p 0.468; lateral condylar height, p 0.899; medial condylar height, p 0.816). Many radiological parameters of trochlear morphology were significantly different between the two groups at the final follow-up, including well-known parameters, such as the mean sulcus angle (Group S, 146.27° (. sd. 7.18); Group C, 160.61° (. sd. 9.29); p < 0.001), the mean trochlear groove depth (Group S, 6.25 mm (. sd. 0.41); Group C, 3.48 mm (. sd. 0.65); p < 0.001) and the mean lateral trochlear inclination (Group S, 20.99° (. sd. 3.87); Group C, 12.18° (. sd. 1.85); p < 0.001). Lesser known parameters such as the ratio of the lateral to medial trochlear length (Group S, 1.46 (. sd. 0.19); Group C, 2.14 (. sd. 0.42); p < 0.001), which is a measurement of facet asymmetry, and the lateral and medial condylar height were also significantly different between the two groups (p < 0.001). Conclusion. The femoral trochlear morphology can be improved by early (before epiphyseal closure) surgical correction in children with recurrent patellar dislocation associated with femoral trochlear dysplasia. Cite this article: Bone Joint J 2018;100-B:811–21

We investigated the clinical and radiological outcome of trochleaplasty for recurrent patellar dislocation in association with trochlear dysplasia in 38 consecutive patients (45 knees) with a mean follow-up of 8.3 years (4 to 14). None had recurrence of dislocation after trochleaplasty. Post-operatively, patellofemoral pain, present pre-operatively in only 35 knees, became worse in 15 (33.4%), remained unchanged in four (8.8%) and improved in 22 (49%). Four knees which had no pain pre-operatively (8.8%) continued to have no pain. A total of 33 knees were available for radiological assessment. Post-operatively, all but two knees (93.9%) had correction of trochlear dysplasia radiologically but degenerative changes of the patellofemoral joint developed in 30% (10) of the knees. We conclude that recurrent patellar dislocation associated with trochlear dysplasia can be treated successfully by trochleaplasty, but the impact on patellofemoral pain and the development of patellofemoral osteoarthritis is less predictable. Overall, subjective patient satisfaction with restored patellofemoral stability after trochleaplasty appeared to outweigh its possible sequelae

Since this paper was submitted for publication three additional reports have appeared, two of which represent typical examples of the syndrome. Husson and Parkman (1961) reported the case of a female infant dying at the age of four months with chondroectodermal dysplasia, obliteration of the upper labiogingival sulcus, and congenital heart disease. The heart lesions included anomalous pulmonary venous return, single atrium and a persistent left superior vena cava. There was no known consanguinity. In reviewing the cardiac lesions described in chondroectodermal dysplasia they include another case with anomalous pulmonary venous return described by Darling, Rothney and Craig (1957), which also showed chondroectodermal dysplasia and polydactyly, though details of the extracardiac malformations are not given. Nabrady (1961) described a four-year-old Hungarian girl with ectodermal defects involving the teeth and nails but not the hair, polydactyly, distal shortening of the extremities producing dwarfing, and typical radiological appearances of the long bones. There was presumptive evidence of a septal defect of the heart. There was no consanguinity, but the mother was considered to show a "forme fruste" of the condition. A necropsy report by Meitner (1961) of a newly born premature infant with multiple congenital malformations of organs of ectodermal, mesodermal and endodermal origin is of interest because these malformations included extreme polydactyly of hands and feet, absence of nails, chondrodysplasia, and trilocular heart. In many respects, however, the case is atypical of chondroectodermal dysplasia

We followed up 76 consecutive hips with symptomatic acetabular dysplasia treated by acetabular shelf augmentation for a mean period of 11 years. Survival analysis using conversion to hip replacement as an end-point was 86% at five years and 46% at ten years. Forty-four hips with slight or no narrowing of the joint space pre-operatively had a survival of 97% at five and 75% at ten years. This was significantly higher (p = 0.0007) than that of the 32 hips with moderate or severe narrowing of the joint-space, which was 76% at five and 22% at ten years. There was no significant relationship between survival and age (p = 0.37) or the pre- and post-operative centre-edge (p = 0.39) and acetabular angles (p = 0.85). Shelf acetabuloplasty is a reliable, safe procedure offering medium-term symptomatic relief for adults with acetabular dysplasia. The best results were achieved in patients with mild and moderate dysplasia of the hip with little arthritis

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

1 . Twenty patients with fibrous dysplasia, confirmed histologically, are reported and discussed in regard to classification, etiology, pathogenesis and treatment. The various fibrous or fibrocystic lesions of bone are characterised briefly for purpose of contrast, and the position of fibrous dysplasia in this heterogeneous collection is suggested. 2. The classification of fibrous dysplasia based on the degree of skeletal involvement is used, and the diagnostic, therapeutic and prognostic implications of this classification emphasised. The authors endorse the opinion that fibrous dysplasia is a developmental defect. Clinical, histological and radiographic evidence is presented to point to the distinct evolution of the monostotic lesions, to which a positive and aggressive approach is recommended

Our aim was to determine whether abnormalities noted on MRI immediately after reduction for developmental dysplasia of the hip could predict the persistance of dysplasia and aid surgical planning. Scans of 13 hips in which acetabular dysplasia had resolved by the age of four years were compared with those of five which had required pelvic osteotomy for persisting dysplasia. The scans were analysed by two consultant musculoskeletal radiologists who were blinded to the outcome in each child. The postreduction scans highlighted a number of anatomical abnormalities secondary to developmental dysplasia of the hip, but statistical analysis showed that none were predictive of persisting acetabular dysplasia in the older child, suggesting that the factors which determine the long-term outcome were not visible on these images

There is controversy whether congenital foot abnormalities are true risk factors for pathological dysplasia of the hip. Previous United Kingdom screening guidelines considered congenital talipes equinovarus (CTEV) to be a risk factor for hip dysplasia, but present guidelines do not. We assessed the potential relationship between pathological dysplasia of the hip and fixed idiopathic CTEV. We present a single-centre 21-year prospective longitudinal observational study. All fixed idiopathic CTEV cases were classified (Harrold and Walker Types 1 to 3) and the hips clinically and sonographically assessed. Sonographic Graf Type III, IV and radiological irreducible hip dislocation were considered to be pathological hip dysplasia. Over 21 years there were 139 children with 199 cases of fixed idiopathic CTEV feet. Sonographically, there were 259 normal hips, 18 Graf Type II hips, 1 Graf Type III hip and 0 Graf Type IV hip. There were no cases of radiological or sonographic irreducible hip dislocation. Fixed idiopathic CTEV should not be considered as a significant risk factor for pathological hip dysplasia. This conclusion is in keeping with the current newborn and infant physical examination guidelines in which the only risk factors routinely screened are family history and breech presentation. Our findings suggest CTEV should not be considered a significant risk factor in pathological dysplasia of the hip. Cite this article: Bone Joint J 2014;96-B:1553–5

Aims. Acetabular dysplasia is frequently associated with intra-articular pathology such as labral tears, but whether labral tears should be treated at the time of periacetabular osteotomy (PAO) remains controversial. The purpose of this study was to compare the clinical outcomes and radiographic corrections of PAO for acetabular dysplasia between patients with and without labral tears pre-operatively. Patients and Methods. We retrospectively reviewed 70 hips in 67 patients with acetabular dysplasia who underwent PAO. Of 47 hips (45 patients) with labral tears pre-operatively, 27 (25 patients) underwent PAO alone, and were classified as the labral tear alone (LT) group, and 20 (20 patients) underwent combined PAO and osteochondroplasty, and were classified as the labral tear osteochondroplasty (LTO) group. The non-labral tear (NLT) group included 23 hips in 22 patients. Results. There were no significant differences between groups for post-operative Harris hip scores, degree of progression of osteoarthritis or rate of reoperation. The pre-operative alpha angle was significantly larger in the LTO group compared with the other groups (p < 0.0001). Conclusion. PAO provides equivalent short-term relief of pain and functional outcome in patients with or without labral tears. The rate of progression of osteoarthritis and reoperation was not significantly increased in patients with labral tears. Take home message: PAO provides equivalent short-term pain relief and functional outcomes in patients with acetabular dysplasia with and without labral tears. We did not find significantly increased risks of progression of osteoarthritis or re-operation in those with labral tears. Cite this article: Bone Joint J 2016;98-B:741–6

Untreated acetabular dysplasia following treatment for developmental dysplasia of the hip (DDH) leads to early degenerative joint disease. Clinicians must accurately and reliably recognise dysplasia in order to intervene appropriately with secondary acetabular or femoral procedures. This study sought early predictors of residual dysplasia in order to establish empirically-based indications for treatment. DDH treated by closed or open reduction alone was reviewed. Residual hip dysplasia was defined according to the Severin classification at skeletal maturity. Future hip replacement in a subset of these patients was compared with the Severin classification. Serial measurements of acetabular development and subluxation of the femoral head were collected, as were the age at reduction, type of reduction, and Tonnis grade prior to reduction. These variables were used to predict the Severin classification. The mean age at reduction in 72 hips was 16 months (1 to 46). On the final radiograph, 47 hips (65%) were classified as Severin I/II, and 25 as Severin III/IV (35%). At 40 years after reduction, five of 43 hips (21%) had had a total hip replacement (THR). The Severin grade was predictive for THR. Early measurements of the acetabular index (AI) were predictive for Severin grade. For example, an AI of 35° or more at two years after reduction was associated with an 80% probability of becoming a Severin grade III/IV hip. This study links early acetabular remodelling, residual dysplasia at skeletal maturity and the long-term risk of THR. It presents evidence describing the diagnostic value of early predictors of residual dysplasia, and therefore, of the long-term risk of degenerative change

Aims

The primary aims of this study were to determine the time to sonographic correction of decentred hips during treatment with Pavlik harness for developmental dysplasia of the hip (DDH) and investigate potential risk factors for a delayed response to treatment.

1. The occurrence of bilateral double-layer patellae in association with multiple epiphysial dysplasia is described in three siblings. 2. Twelve cases of bilateral double-layer patellae have been reported previously, and in all there was an accompanying skeletal dystrophy which, although not diagnosed as such, is likely to have been multiple epiphysial dysplasia. 3. It is suggested that although they occur only in a minority of cases, double-layer patellae when present are a feature of considerable diagnostic value in multiple epiphysial dysplasia. Lateral radiography of the knees may therefore be of assistance in the diagnosis of multiple epiphysial dysplasia

Aims

Periacetabular osteotomy (PAO) is widely recognized as a demanding surgical procedure for acetabular reorientation. Reports about the learning curve have primarily focused on complication rates during the initial learning phase. Therefore, our aim was to assess the PAO learning curve from an analytical perspective by determining the number of PAOs required for the duration of surgery to plateau and the accuracy to improve.

Aims

Early detection of developmental dysplasia of the hip (DDH) is associated with improved outcomes of conservative treatment. Therefore, we aimed to evaluate a novel screening programme that included both the primary risk factors of breech presentation and family history, and the secondary risk factors of oligohydramnios and foot deformities.

Objectives. To determine the pattern of mutations of the WISP3 gene in clinically identified progressive pseudorheumatoid dysplasia (PPD) in an Indian population. Patients and Methods. A total of 15 patients with clinical features of PPD were enrolled in this study. Genomic DNA was isolated and polymerase chain reaction performed to amplify the WISP3 gene. Screening for mutations was done by conformation-sensitive gel electrophoresis, beginning with the fifth exon and subsequently proceeding to the remaining exons. Sanger sequencing was performed for both forward and reverse strands to confirm the mutations. Results. In all, two of the 15 patients had compound heterozygous mutations: one a nonsense mutation c.156C>A (p.C52*) in exon 2, and the other a missense mutation c.677G>T (p.G226V) in exon 4. All others were homozygous, with three bearing a nonsense mutation c.156C>A (p.C52*) in exon 2, three a missense mutation c.233G>A (p.C78Y) in exon 2, five a missense mutation c.1010G>A (p.C337Y) in exon 5, one a nonsense mutation c.348C>A (p.Y116*) in exon 3, and one with a novel deletion mutation c.593_597delATAGA (p.Y198*) in exon 4. Conclusion. We identified a novel mutation c.593_597delATAGA (p.Y198*) in the fourth exon of the WISP3 gene. We also confirmed c.1010G>A as one of the common mutations in an Indian population with progressive pseudorheumatoid dysplasia. Cite this article: V. Madhuri, M. Santhanam, K. Rajagopal, L. K. Sugumar, V. Balaji. WISP3 mutational analysis in Indian patients diagnosed with progressive pseudorheumatoid dysplasia and report of a novel mutation at p.Y198* Bone Joint Res 2016;5:301–306. DOI: 10.1302/2046-3758.57.2000520

Aims. This study aimed to explore whether intraoperative nerve monitoring can identify risk factors and reduce the incidence of nerve injury in patients with high-riding developmental dysplasia. Patients and Methods. We conducted a historical controlled study of patients with unilateral Crowe IV developmental dysplasia of the hip (DDH). Between October 2016 and October 2017, intraoperative nerve monitoring of the femoral and sciatic nerves was applied in total hip arthroplasty (THA). A neuromonitoring technician was employed to monitor nerve function and inform the surgeon of ongoing changes in a timely manner. Patients who did not have intraoperative nerve monitoring between September 2015 and October 2016 were selected as the control group. All the surgeries were performed by one surgeon. Demographics and clinical data were analyzed. A total of 35 patients in the monitoring group (ten male, 25 female; mean age 37.1 years (20 to 46)) and 56 patients in the control group (13 male, 43 female; mean age 37.9 years (23 to 52)) were enrolled. The mean follow-up of all patients was 13.1 months (10 to 15). Results. The two groups had no significant differences in preoperative data. In the monitoring group, ten nerve alerts occurred intraoperatively, and no neural complications were detected postoperatively. In the control group, six patients had neural complications. The rate of nerve injury was lower in the monitoring group than in the control group, but this did not achieve statistical significance. The degree of leg lengthening was significantly greater in the monitoring group than in the control group. In further analyses, patients who had previous hip surgery were more likely to have intraoperative nerve alerts and postoperative nerve injury. Conclusion. Nerve injury usually occurred during the processes of exposure and reduction. The use of intraoperative nerve monitoring showed a trend towards reduced nerve injury in THA for Crowe IV DDH patients. Hence, we recommend its routine use in patients undergoing leg lengthening, especially in those with previous hip surgery. Cite this article: Bone Joint J 2019;101-B:1438–1446

Aims

To analyze whether the addition of risk-based criteria to clinical examination-based selective ultrasound screening would increase the rates of early detected cases of developmental dysplasia of the hip (DDH) and decrease the rate of late detected cases.

1. Familial metaphysial dysplasia occurring in two families is recorded. 2. One case with unusual skull changes and webbing of the digits is described in detail. 3. The similarity between the shape of the bones in familial metaphysial dysplasia and other bone disease is discussed

The dysplasia cup, which was devised as an adjunct to the Birmingham Hip Resurfacing system, has a hydroxyapatite-coated porous surface and two supplementary neutralisation screws to provide stable primary fixation, permit early weight-bearing, and allow incorporation of morcellised autograft without the need for structural bone grafting. A total of 110 consecutive dysplasia resurfacing arthroplasties in 103 patients (55 men and 48 women) performed between 1997 and 2000 was reviewed with a minimum follow-up of six years. The mean age at operation was 47.2 years (21 to 62) and 104 hips (94%) were Crowe grade II or III. During the mean follow-up of 7.8 years (6 to 9.6), three hips (2.7%) were converted to a total hip replacement at a mean of 3.9 years (2 months to 8.1 years), giving a cumulative survival of 95.2% at nine years (95% confidence interval 89 to 100). The revisions were due to a fracture of the femoral neck, a collapse of the femoral head and a deep infection. There was no aseptic loosening or osteolysis of the acetabular component associated with either of the revisions performed for failure of the femoral component. No patient is awaiting a revision. The median Oxford hip score in 98 patients with surviving hips at the final review was 13 and the 10th and the 90th percentiles were 12 and 23, respectively

Fifty-one infants with limited abduction of the hip and acetabular dysplasia were, between 1969 and 1975, treated with abduction-adduction exercises, administered by the parents; no abduction devices were used. In 1983 a follow-up examination was carried out on 41 of these patients. Although at birth these children had characteristics similar to patients with congenital dislocation of the hip, none of their hips dislocated. At birth acetabular measurements showed that half the children had severe dysplasia and the other half slight dysplasia; the difference between the affected and the healthy hips was significant. At follow-up the gait was normal in all the patients. Movements at the hips were symmetrical and within normal limits in all but one patient. The acetabular angle, the centre-edge angle, the shaft-neck angle, the hip ratio, and the size of the femoral head were the same on the previously affected side as on the normal side. Nineteen of the children followed up had preferred lying on one side and dysplasia of the upper hip had developed. The significance of the sleeping position on the development of acetabular dysplasia is discussed

A brief review of the literature on malignant change occurring in fibrous dysplasia is given and a further case of a sarcoma arising in a patient with polyostotic fibrous dysplasia is reported

Tibial dysplasia is a rare congenital deformity which must be distinguished from the more common fibular dysplasia. We have reviewed 24 patients with 35 affected legs. The classification system of Kalamchi and Dawe (1985) was found to be preferable to that of Jones, Barnes and Lloyd-Roberts (1978) as a guide to prognosis and management. We discuss the outcome of surgical treatment, recommending selective amputation for most cases

We examined clinically and radiologically the knees of 46 patients (27 females and 19 males) with diastrophic dysplasia. The age of the patients varied from newborn to 38 years. A total of 18 patients was followed during their growth until adolescence. The knees of two legally aborted fetuses appeared on examination to be macroscopically normal and congruous. Excessive valgus deformity of the tibiofemoral weight-bearing angle with a mean of 14° was noted in infancy. Most of the patients had marked instability of the knees. The range of movement of the knee began to decrease before the age of five years. There were signs of early degeneration and deformation of the bony epiphyses before the age of six years. The patellofemoral joint was abnormal from an early age. A marked patella infera, often associated with a lateral position of the patella with bony fragmentation, was noted. The knee in diastrophic dysplasia is basically unstable, showing early deformation of the subchondral bone and degeneration of the joint

Three amputated legs with tibial dysplasia were studied by radiography, arteriography and anatomical dissection. The radiographic appearances were the same as the Type 1b tibial dysplasia described by Jones, Barnes and Lloyd-Roberts (1978) in that the tibiae were absent but the lower femoral epiphyses were normal. However, our anatomical findings differed from those of Jones et al. since no bony or cartilaginous anlage of the proximal tibia was found in any of the three legs. The pattern of vascular anomaly was identical in the three legs and similar to the findings of Hootnick et al. (1980) in congenital short fibula. Congenital fusion of the subtalar joint was a constant finding. These results support the hypothesis that the arterial and skeletal systems are vulnerable to a teratogenic insult in the fifth week of embryonic life. The bony and arterial anomalies should be borne in mind by the surgeon attempting reconstructive surgery for this condition

Aims. The influence of identifiable pre-operative factors on the outcome of eccentric rotational acetabular osteotomy (ERAO) is unknown. We aimed to determine the factors that might influence the outcome, in order to develop a scoring system for predicting the prognosis for patients undergoing this procedure. Patients and Methods. We reviewed 700 consecutive ERAOs in 54 men and 646 women with symptomatic acetabular dysplasia or early onset osteoarthritis (OA) of the hip, which were undertaken between September 1989 and March 2013. The patients’ pre-operative background, clinical and radiological findings were examined retrospectively. Multivariate Cox regression analysis was performed using the time from the day of surgery to a conversion to total hip arthroplasty (THA) as an endpoint. A risk score was calculated to predict the prognosis for conversion to THA, and its predictive capacity was investigated. Results. The congruity of the hip, age, the pre-operative minimum width of the joint space and range of abduction were identified as factors predicting conversion to THA. For three groups of patients (scoring 0 to 5, 6 to 7, and 8 to 9 points), the Kaplan-Meier event-free rates of survival at 15 years post-operatively for conversion to THA were 99.6%, 85.2% and 67.3%, respectively. Conclusion. These four pre-operative factors are easily measured and predict the prognosis for patients following ERAO. They may be used for decision making when offering surgical treatment to patients with acetabular dysplasia and early onset osteoarthritis. Cite this article: Bone Joint J 2016;98-B:1326–32

We performed rotational acetabular osteotomy in order to treat dysplasia of the hip in five ambulatory adults with cerebral palsy. There was one man and four women, with a mean age of 21 years (16 to 27) who were followed up for a mean of 12 years and two months. The mean Sharp angle improved from 52° to 43°, the mean acetabular index from 30.2° to 2.8°, the mean centre-edge angle from −5.6° to 29.2°, and the mean acetabular head index from 49.2 to 88.2. There was no progression of joint degeneration and relief from pain was maintained. Our results suggest that rotational acetabular osteotomy is a valuable option for the treatment of acetabular dysplasia in adults with cerebral palsy who have incapacitating pain in the hip

We describe a new technique of reconstruction of the deficient acetabulum in cementless total hip arthroplasty. The outer iliac table just above the deficient acetabulum is osteotomised and slid downwards. We have termed this an iliac sliding graft. Between October 1997 and November 2001, cementless total hip arthroplasty with an iliac sliding graft was performed on 19 patients (19 hips) with acetabular dysplasia. The mean follow-up was 3.4 years (2 to 6). The mean pre-operative Harris hip score was 45.1 which improved significantly to 85.3 at the time of the final follow-up. No patient had post-operative abductor dysfunction. Incorporation of the graft was seen after two to three months in all patients. Resorption of the graft and radiolucencies were infrequent. This technique is a useful alternative to femoral head autografting when the patient’s own femoral head cannot be used

We describe the clinical and radiological results of cementless primary total hip replacement (THR) in 25 patients (18 women and seven men; 30 THRs) with severe developmental dysplasia of the hip (DDH). Their mean age at surgery was 47 years (23 to 89). In all, 21 hips had Crowe type III dysplasia and nine had Crowe type IV. Cementless acetabular components with standard polyethylene liners were introduced as close to the level of the true acetabulum as possible. The modular cementless S-ROM femoral component was used with a low resection of the femoral neck. A total of 21 patients (25 THRs) were available for review at a mean follow-up of 18.7 years (15.8 to 21.8). The mean modified Harris hip score improved from 46 points pre-operatively to 90 at final follow up (p < 0.001). . A total of 15 patients (17 THRs; 57%) underwent revision of the acetabular component at a mean of 14.6 years (7 to 20.8), all for osteolysis. Two patients (two THRs) had symptomatic loosening. No patient underwent femoral revision. Survival with revision of either component for any indication was 81% at 15 years (95% CI 60.1 to 92.3), with 21 patients at risk. . This technique may reduce the need for femoral osteotomy in severe DDH, while providing a good long-term functional result. Cite this article: Bone Joint J 2014;96-B:1449–54

Hereditary multiple exostoses is an autosomal dominant disorder characterised by multiple osteochondromata, most commonly affecting the forearm, knee and ankle. Osteochondromata of the proximal femur have been reported to occur in 30% to 90% of affected patients with coxa valga in 25%. Acetabular dysplasia is rare but has been described. This is the first report of a patient requiring surgical intervention. A girl was seen at the age of nine with hereditary multiple exostoses and when 12 developed bilateral pain in the groin. Radiographs showed severely dysplastic acetabula with less than 50% coverage of the femoral heads and widening of the medial joint space. Large sessile osteochondromata were present along the medial side of the femoral neck proximal to the lesser trochanter, with associated coxa valga. The case illustrates the importance of obtaining initial skeletal surveys in children with hereditary multiple exostoses to identify potential problems such as acetabular dysplasia and subluxation of the hip

1. Two cases are reported showing the syndrome of chondrodysplasia, manual polydactyly, ectodermal dysplasia affecting the teeth and nails, and congenital heart disease. 2. Particulars of thirty-eight cases are tabulated, and the features of the syndrome are discussed. 3. The syndrome is regarded as showing an autosomal recessive mode of inheritance

We investigated the variables which determine the outcome after triple osteotomy of the pelvis for the treatment of congenital dysplasia of the hip. We reviewed 51 patients (61 hips) with a median age at operation of 23 years who were treated with a Tönnis triple osteotomy. The median follow-up was six years with a minimum of two years. Eight patients (eight hips) required a revision procedure. Of the remaining 53 hips, the results were good or excellent in 36 (68%) when evaluated according to the Harris hip score (median 90 points), and 33 patients (65%) were satisfied with the procedure. Logistic regression analysis indicated that the incidence of complications such as nonunion at an osteotomy site influenced patient satisfaction (p = 0.079). The incidence of complications correlated positively with increasing patient age at operation (p = 0.004). The amount of acetabular correction did not correlate with patient satisfaction. In univariate analysis, the groups of ’satisfied’ and ‘not satisfied’ patients differed significantly in Harris hip score, age, incidence of nonunion at the osteotomy sites, complications and late revisions. In conclusion, the patient’s age at operation and the incidence of complications influence patient satisfaction after triple osteotomy, but the amount of radiologically evident acetabular correction shows no correlation to outcome

We report the case of a 12-year-old boy with polyostotic fibrous dysplasia of the upper femur in whom a massive customised polyethylene prosthesis functioned successfully for more than fifty years

The exceptionally high prevalence of diastrophic dysplasia in Finland has enabled us to analyse the foot deformities of 102 patients at their first orthopaedic evaluation and classify 204 feet into five categories. The most common finding (43%) was a foot with tarsal valgus deformity and metatarsus adductus; 37% showed either equinovarus adductus (29%) or equinus (8%) deformities. At the first examination 13% showed metatarsus adductus deformity alone, and 7% were clinically normal. The expression 'club foot', generally used for the foot deformity in diastrophic dysplasia is a misnomer. There is a wide spectrum of deformities, some of them specific for the condition

1. A survey of genetic and other etiological factors has been carried out in 589 index patients with congenital dislocation of the hip and their families, with special investigation of acetabular dysplasia, familial joint laxity and a comparison of neonatal and late-diagnosis cases. 2. It is believed that there are two etiological groups with congenital dislocation of the hip, i) a group with acetabular dysplasia which is inherited as a multiple gene system and is responsible for a high proportion of cases diagnosed late, and ii) a group with joint laxity which is responsible for a high proportion of neonatal cases. 3. Evidence is presented to show that acetabular dysplasia is a separate heritable system in some families. 4. Other findings relating to the genetic aspects of the survey are summarised

The acetabular rim syndrome is a pathological entity which we illustrate by reference to 29 cases. The syndrome is a precursor of osteoarthritis of the hip secondary to acetabular dysplasia. The symptoms are pain and impaired function. All our cases were treated by operation which consisted in most instances of re-orientation of the acetabulum by peri-acetabular osteotomy and arthrotomy of the hip. In all cases, the limbus was found to be detached from the bony rim of the acetabulum. In several instances there was a separated bone fragment, or 'os acetabuli' as well. In acetabular dysplasia, the acetabular rim is subject to abnormal stress which may cause the limbus to rupture, and a fragment of bone to separate from the adjacent bone margin. Dysplastic acetabuli may be classified into two radiological types. In type I there is an incongruent shallow acetabulum. In type II the acetabulum is congruent but the coverage of the femoral head is deficient

Malignant transformation of fibrous dysplasia to chondrosarcoma is rare. We report a case in which malignancy developed in an area of fibrous dysplasia in the ilium. We believe this to be the second reported case at this site. Treatment was by excision of the hemipelvis including the ala of the sacrum. A review of the literature is presented

Radiological and histological examinations are described in a full term stillborn infant with punctate epiphysial dysplasia diagnosed in utero as achondroplasia

A patient with polyostotic fibrous dysplasia had several fractures of the right lower limb. An above-knee amputation was eventually performed, followed by arthrodesis of the hip. Five years later the stump became painful and swelled with dramatic rapidity. Biopsy showed that this was not due to malignant change, but that an aneurysmal bone cyst had developed in association with the fibrous dysplasia

Three cases of multiple epiphysial dysplasia in three generations of one family are described. The history indicates that three further members, one in a fourth generation. have been affected

1. A case of dysplasia epiphysialis punctata is described, with some histological observations. 2. Both parents were syphilitic. 3. The etiology is not yet fully determined. Some suggestions are put forward for further investigation

1. A case is reported of a Jamaican boy of fifteen months with typical and florid manifestations of dysplasia epiphysialis hemimelica associated with a number of atypical features not previously recorded. 2. The progress of the disorder over a limited period of two years is described and the development of the atypical features is discussed

After late reduction of the hip in children with developmental dysplasia the acetabular contour slowly improves and it is difficult to know if and when acetabular reconstruction is required. We studied the radiographs of 19 patients with unilateral dislocated or subluxated hips which had been reduced between the ages of one and two years. Preoperatively, all the affected hips showed acetabular dysplasia. After reduction they steadily improved for three years by which time none was dysplastic as measured by the acetabular index. After the age of ten years, when assessed by the more sensitive centre-edge angle, two were found to be dysplastic. It was not possible to predict these from early radiographs. We conclude that the decision to reconstruct an acetabulum should not be taken until three years after reduction, and that a few hips which appear to be developing satisfactorily at that time will, nevertheless, become dysplastic

1. Monostotic fibrous dysplasia in the long bones occurs most frequently in adolescence. In the jaws it is found mainly in early adult life. It presents later in the ribs, probably because it is often asymptomatic in this site. 2. The disease is equally distributed in both sexes. 3. Reactivation may occasionally occur in later life and in pregnancy. 4. Successful surgical treatment is by no means always easily achieved, and requires, in addition to the problems of fracture fixation and the correction of deformity, careful consideration of the age of the patient, the activity of the lesion and the extent to which it involves the cortical bone

We carried out a morphometric analysis of acetabular dysplasia in patients with cerebral palsy requiring hip surgery using three-dimensional CT. We evaluated 54 hips in 27 patients. The contralateral normal hips of ten patients with unilateral Perthes’ disease were used as a control group. The acetabular defects were assessed qualitatively as anterior, posterior or global. Quantitative assessments were made using three-directional acetabular indices (anterosuperior, superolateral and posterosuperior) and measured by multiplanar reformation, from which we calculated the acetabular volume. In the qualitative study, posterior defects were most common in the subluxation group whereas global defects predominated in the dislocation group. In the quantitative study, all acetabular indices in both the subluxation and dislocation groups were higher than those in the control group and the superolateral indices showed a tendency to elevation in the dislocation group. The acetabular volume was largest in the control group, smallest in the dislocation group, and intermediate between the two in the subluxation group

We reviewed 12 patients with primary glenoid dysplasia. Ten were assessed clinically and two from case notes and radiographs. We identified two groups according to the age at onset of symptoms. The first (seven patients) consisted of boys and younger men, all of whom developed symptoms before the age of 40 years. All four children were free from pain, whereas the three adults in this group had varying degrees of this. Four patients had symptoms of instability. The second group consisted of older men (five patients) all of whom had noted the onset of symptoms, in the form of pain and stiffness, after the age of 40 years. All five had radiological evidence of osteoarthritis. Although the four children in our study had minimal symptoms, all eight adults had ongoing shoulder pain and dysfunction, despite a specific rehabilitation programme. Four patients required surgery; one had posterior stabilisation for instability and three arthroplasties of the shoulder for osteoarthritis

The Fairbank Collection in the Radiology Department of the Royal National Orthopaedic Hospital contains details of 15 patients in the section labelled "Metaphysial Dysplasia and Dysostosis". This material has been reviewed, long-term follow-up of the patients carried out and the diagnosis revised, where indicated, according to current concepts. Of the 15 patients, all children, seven had recognisable bone dysplasias. The remaining eight, all listed under the heading "metaphysial fragility", had the characteristic radiographic features of "battered" babies

1. A case of metaphysial dysplasia observed for eleven years is reported. 2. Only one case with similar features has previously been reported. 3. The literature on the subject is reviewed

We examined 50 patients with diastrophic dysplasia both clinically and radiologically. Two legally aborted fetuses were dissected. The mean age of the patients was 16.2 years (newborn to 38) and the mean follow-up was 11.4 years (3 months to 34 years). The fetal hips and MRI of newborn infants showed congruity and no significant joint deformity. Flexion contracture of the hip became evident later in 93% and was progressive. The radiological appearance of the proximal femoral ossific nuclei was delayed and in 17% of males and 28% of females the ossific nuclei had not appeared by the age of 12 years. Radiological measurements differed considerably from reference values and were related to the rapid and progressive restriction of rotational movement and the increase in flexion contracture. The typical findings were flattening and inferomedial bulking of the femoral head and a double-hump deformation. The changes in the hip led to secondary osteoarthritis before early middle age. We describe the clinical and radiological measurements which define the early degeneration of the joint

1. A condition of fibrous dysplasia of the jaws occurring in four brothers and a sister has been under observation since 1931. 2. Three of the five patients have been operated upon for correction of grotesque deformity, with satisfactory results. 3. The pathological nature of the disorder is discussed

Craniodiaphyseal dysplasia (CDD) is a rare sclerosing bone disorder, the severity of which depends on its phenotypic expression. Hyperostosis can cause progressive foraminal stenosis leading to palsy of cranial nerves, epilepsy and mental retardation. We report the only case of CDD in an adult, with stenosis of the cervical canal leading to quadriparesis as a late complication of hyperostosis, and describe the problems associated with its treatment. Although the syndrome is rare, its pathophysiological and therapeutic considerations may be applicable to the management of stenosis of the spinal canal in other hyperostotic bone disorders

In order to clarify how intra-articular lesions influence the survival of a periacetabular osteotomy in patients with dysplasia of the hip, we performed an observational study of 121 patients (121 hips) who underwent a transposition osteotomy of the acetabulum combined with an arthroscopy. Their mean age was 40.2 years (13 to 64) and the mean follow-up was 9.9 years (2 to 18). Labral and cartilage degeneration tended to originate from the anterosuperior part of the acetabulum, followed by the femoral side. In all, eight hips (6.6%) had post-operative progression to Kellgren–Lawrence grade 4 changes, and these hips were associated with the following factors: moderate osteoarthritis, decreased width of the joint space, joint incongruity, and advanced intra-articular lesions (subchondral bone exposure in the cartilage and a full-thickness labral tear). Multivariate analysis indicated subchondral bone exposure on the femoral head as an independent risk factor for progression of osteoarthritis (p = 0.003). In hips with early stage osteoarthritis, femoral subchondral bone exposure was a risk factor for progression of the grade of osteoarthritis. . Although the outcome of transposition osteotomy of the acetabulum was satisfactory, post-operative progression of osteoarthritis occurred at a high rate in hips with advanced intra-articular lesions, particularly in those where the degenerative process had reached the point of femoral subchondral bone exposure

1. A family of six generations, consisting of 114 members affected by epiphysial dysplasia of the lower extremities, is described. 2. Twenty-two males and twenty-three females were affected, while thirty-two males and thirty females were unaffected. Seven members of the family could not be traced. 3. Variations from Fairbank's description of dysplasia epiphysialis multiplex are discussed. In the family here reported the lower limbs only were affected and the severity of the disease was very variable. 4. Long term follow-up studies are presented

Reorientation of the acetabulum may be required in adolescents and young adults with developmental dysplasia of the hip. We have carried out a retrospective review of 51 hips after triple osteotomy with an average follow-up of ten years (8 to 15). Forty-eight hips (94%) were available for review and of these 39 (81%) were improved compared with before operation, 29 (60%) scoring good or excellent. Radiographic assessment showed improvement of the average centre-edge angle by 19°, the acetabular index by 12° and the anterior centre-edge angle by 26°. The degree of osteoarthritis progressed by one grade in ten hips (21%) over a period of ten years. The satisfactory long-term clinical and radiographic results have encouraged us to continue this treatment for symptomatic acetabular dysplasia in these patients

We report 40 cases in one family of an autosomal dominant bone dysplasia, which, though similar in some aspects to Paget's disease, seems unique in some features and in its natural history. The disease shows both general and focal skeletal changes, the latter being mainly in the limbs with an onset from the second decade. Progressive osteoclastic resorption is accompanied by medullary expansion which leads to pain, severe deformity and a tendency to pathological fracture. The serum alkaline phosphatase and urinary hydroxyproline are variably elevated, while other biochemical indices are normal. Most patients had an associated deafness of early onset and loss of dentition. No previous description of this disease has been found in the literature

We performed Charnley total hip arthroplasties on 64 patients (71 hips) between 1976 and 1984 for moderate congenital acetabular dysplasia in which a superolateral cement thickness of less than 20 mm was expected when the cup was placed in the true acetabulum at an angle of 45°. Of these, 59 hips were examined 10 to 17 years after operation; 37 (group A) had been operated on between 1976 and 1982 using Charnley’s original technique of cementing the acetabulum and 22 (group B) between 1983 and 1984 using more modern techniques. In group A, aseptic loosening of the socket was observed in ten hips (27.0%) and the 17-year survival rate was 81.5%. In group B, loosening was noted in only one socket (4.5%) and the 13-year survival rate was 100%. The improved techniques produced significantly better long-term results in fixation of the cup in dysplastic hips without bone grafting

We describe our experience with vascularised bone grafting for the treatment of fibrous dysplasia of the upper limb in eight patients, five men and three women, aged between 17 and 36 years. The site was in the humerus in six and the radius in two. Persistent pain, progression of the lesion and pathological fracture with delayed union were the indications for surgical intervention. We used a vascularised fibular graft after curettage of the lesion. Function and radiological progress were serially monitored. Early radiological union of the graft occurred at periods ranging from 8 to 14 weeks. The mean period for reconstitution of the diameter of the bone was 14 months (12 to 18) predominantly through inductive formation of bone around the vascularised graft, which was a prominent feature in all patients. There were no recurrences and none of the grafts sustained a fracture or failed to unite. After operation function was excellent in three patients and good in five. Vascularised bone grafts provide a safe and reliable means of ensuring good continuity of bone with little risk of recurrence and failure

Although the technique of autogenous acetabular bone grafting has been widely used to augment containment of the acetabulum in total hip arthroplasty (THA) for developmental dysplasia, the role of this technique in improving long-term results remains controversial. We present the long-term results of cemented THA with acetabular bone grafting in 112 patients (133 hips) in order to clarify the factors which affect the outcome. The mean follow-up was for 12.3 years (8 to 24). Kaplan-Meier survivorship analysis predicted a rate of survival of the acetabular component at 15 years of 96% (95% confidence interval (CI) 92 to 99) with revision for aseptic loosening as the endpoint, and of 75% (95% CI 65 to 85) when radiological loosening was used. Parametric survivorship analysis using the Cox proportional-hazards model indicated that trochanteric nonunion, lateral placement of the socket, and delayed trabecular reorientation of the bone graft were risk factors for loosening of the acetabular component. Our findings have shown that autologous acetabular bone grafting is of value for long-term success provided that the risk factors are reduced

1. A family is described of which several members in four generations suffered epiphysial growth disturbances of an unusual kind in the capital epiphyses of the femur, with shortness of stature. 2. This disorder forms one of a group of epiphysial growth disturbances which embraces several recognised disorders, whose effects on the epiphyses vary in severity. They may occur sporadically or they may be inherited as recessive or dominant characters. 3. Differentiation depends on the behaviour of the joint line–in other words, whether the epiphysis starts to show defects through inability to sustain the stress of weight bearing. In the most serious cases the picture is that of Morquio-Brailsford's disease; when the changes are less severe the appearance may be that of bilateral Perthes' disease. Differential diagnosis is no longer possible in later life, because the ultimate condition of the defective femoral heads is the same. Only in the hereditary cases is one able, by studying the capital epiphyses of the hip in childhood, to differentiate between the changes like those of Perthes' disease on the one hand, and micro-epiphysial dysplasia without necrosis on the other

An area of fibrous dysplasia of bone may undergo rapid enlargement which may be due to either cystic degeneration or malignant transformation. These complications may be clinically and radiologically indistinguishable and, unless both are borne in mind, incorrect management may follow. Magnetic resonance imaging was used in one of our cases and was the only imaging modality to demonstrate the true nature of the condition

1. A case of fibrous dysplasia in a woman aged fifty, with unilateral skeletal lesions, cutaneous pigmentation and possibly accelerated skeletal growth, is described. 2. At necropsy, the bony changes were found to be complicated by many of the changes of lipoid granulomatosis. Disseminated plaques of demyelinisation were found in the central nervous system. 3. The implications of the case are discussed

Between 1986 and 1990, we carried out 55 rotational acetabular osteotomies in 54 patients with acetabular dysplasia. Five hips were lost to follow-up. Of the 50 remaining, the degenerative changes were classified according to the criteria of Tönnis as grade 0 in 23, grade 1 in 16 and grade 2 in 11. The mean age of the three men and 46 women at the time of operation was 31.8 years (13 to 53). The mean follow-up was 137 months (120 to 174). At the most recent follow-up, 48 patients had satisfactory relief from pain. There was a slight decrease in the range of movement, particularly of flexion, in 18 hips. Radiologically, all osteotomies had united satisfactorily. There was radiological evidence of improvement in degenerative changes in 13 hips (5 grade 1 and 8 grade 2). Ten deteriorated (5 grade 0, 3 grade 1, and 2 grade 2) and one required total hip arthroplasty ten years after osteotomy. The osteoarthritis in the two hips with an associated valgus osteotomy progressed. The changes in radiological indices such as the centre-edge angle, acetabular femoral head index, acetabular root obliquity and horizontal or vertical displacement of the femoral head showed no statistical difference (unpaired Student’s t-test) between the patients with radiological progression and those with and without improvement

We treated 26 hips (24 consecutive patients) with residual dysplasia by a technique of incomplete triple pelvic osteotomy. The mean age of the patients was 21.6 years. The mean values for the pre-operative centre-edge angle of Wiberg, the refined centre-edge angle, the acetabular angle of Sharp, the modified acetabular angle and femoral head lateralisation were 7.7°, −3.1°, 49.3°, 53.2° and 17.2 mm, respectively. After a mean follow-up of 3.3 years they were 27.0°, 13.0°, 38.9°, 44.3° and 15.9 mm, respectively (p < 0.05). The osteoarthritic grading changed adversely in one hip. The mean pre-operative and latest Harris hip scores were 74.9 and 93.0, respectively (p < 0.05). This technique provides a stable osteotomy with maintenance of the posterior column which allows early mobilisation and minimal internal fixation. The technique is not complex and requires minimal blood transfusion. The use of an image intensifier is not necessary and harvesting of a subcristal bone graft avoids post-operative complications at the donor site

1. The results of Chiari pelvic osteotomy have been examined two to six years after operation in a group of eighteen patients with persisting acetabular dysplasia in the second and third decades. 2. The analysis suggests that for a good clinical result it is essential to create a relationship of the new outer acetabular lip to the original lip so that the CE angle is between 20 and 40 degrees and the roof angle is between 10 degrees below and 20 degrees above the horizontal. 3. This may be achieved by a pelvic osteotomy immediately above the joint capsule, angled 10 degrees upwards and inwards and displaced by 50 per cent of the pelvic thickness. 4. Details of the operative technique using a Smith-Petersen approach are presented

Seventeen patients with congenital spondylo-epiphysial dysplasia from six centres in Britain have been investigated and two variants delineated. There is wide clinical and radiological variability in each group with overlap between them, but 12 of the patients had very short stature and grossly disorganised hips with severe coxa vara, and the five remaining patients were less seriously affected with height only a little below the third percentile and only mild coxa vara. Both groups can be diagnosed at birth but the two cannot be differentiated on clinical and radiological grounds until after the age of three to four years when the developing severe coxa vara and difference in stature become apparent. All cases were sporadic with the exception of a concordant twin-pair

We have reviewed the outcome of 134 hips in 96 children with Graf type-III or type-IV dysplasia of the hip on ultrasound examination. We treated 28 affected hips in 22 children with the Craig splint, 43 hips in 30 children with the Pavlik harness, and 26 hips in 16 children with the von Rosen splint. A total of 37 affected hips in 28 children was not splinted. All children were less than three months of age at referral. Those treated with the von Rosen splint had a significantly better ultrasound appearance at 12 to 20 weeks of age and fewer radiological abnormalities than those not splinted or treated with the Pavlik harness. In the von Rosen group no hip required further treatment with an abduction plaster or operation compared with ten in the Pavlik harness group, three in the Craig splint group and eight in the group without splintage. Our results suggest that the von Rosen splint is more likely to improve the outcome of neonatal dysplasia of the hip and a definitive, large-scale randomised trial is therefore indicated

Three brothers with the X-linked variety of spondylo-epiphyseal dysplasia tarda are presented. Their clinical courses differed: one was relatively free of symptoms, the second had slowly progressive osteoarthritis of the hip, and the third had an acute, disabling arthritis necessitating total hip arthroplasty. The clinical features of this disorder are reviewed

The clinical features of nine new patients with dysplasia epiphysialis hemimelica are reported, with a long-term follow-up on a further seven patients who were described in the earliest case reports of this disease. Each of these 16 patients had only one leg involved, but 12 had multiple epiphyses affected. The distal femur, distal tibia and talus were the commonest sites and most patients presented with painless swelling or deformity. Wasting of the muscles of the affected leg was a common finding, and was occasionally disproportionate to the degree of disuse. One patient had the unique combination of involvement of the lateral and medial halves of different epiphyses in the same limb and another had unusual metaphysial changes. Diagnosis was often delayed despite typical radiographic appearances. There was no evidence for a genetic component in the aetiology nor was any common environmental factor identified. Treatment by local excision was generally effective for lesions in the vicinity of the knee, but some patients with involvement of the talus required arthrodesis around the ankle. The long-term prognosis appears to be good and so far only two of these patients have developed premature osteoarthritis

We carried out the Bernese periacetabular osteotomy for the treatment of 13 dysplastic hips in 11 skeletally mature patients with an underlying neurological diagnosis. Seven hips had flaccid paralysis and six were spastic. The mean age at the time of surgery was 23 years and the mean length of follow-up was 6.4 years. Preoperatively, 11 hips had pain and two had progressive subluxation. Before operation the mean Tönnis angle was 33°, the mean centre-edge angle was −10°, and the mean extrusion index was 53%. Postoperatively, they were 8°, 25° and 15%, respectively. Pain was eliminated in 7 patients and reduced in four in those who had preoperative pain. One patient developed pain secondary to anterior impingement from excessive retroversion of the acetabulum. Four required a varus proximal femoral osteotomy at the time of the pelvic procedure and one a late varus proximal femoral osteotomy for progressive subluxation. Before operation no patient had arthritis. At the most recent follow-up one had early arthritis of the hip (Tönnis grade I) and one had advanced arthritis (Tönnis grade III). Our results suggest that the Bernese periacetabular osteotomy can be used successfully to treat neurogenic acetabular dysplasia in skeletally mature patients

1. An account is given of a family in which five members in three generations were affected by osteochondritis involving the hips, in three cases bilaterally. 2. One patient showed aseptic osseous necrosis of the epiphyses of the ankles and fingers. 3. The differential diagnosis between Perthes' disease and multiple epiphysial dysplasia is discussed, but it is not certain into which category these patients fall. 4. The assistance of a family history and skeletal survey in diagnosis is illustrated

1. Early recognition of femoro-pelvic instability in proximal femoral dysplasia is essential to rational management. 2. Certain radiological signs helpful in identifying such patients within the first year are described. 3. Early operation is recommended to prevent displacement of a pseudarthrosis and to encourage healing. Alternative methods are described for established defects. 4. Radiological signs are described which indicate that healing without loss of femoro-pelvic stability will occur. Such patients may be managed expectantly

A Salter innominate osteotomy is used to treat acetabular dysplasia, but reports of its effects on the position of the femoral head are few and conflicting. Lateral shift would increase the resultant forces acting on the joint and be detrimental. We studied 15 Salter innominate osteotomies and demonstrated that a correctly performed osteotomy does not significantly alter the distance from the centre of the femoral head to the midline of the body. Stereophotogrammetry was used in three patients to delineate the axis of rotation of the distal acetabular fragment and determine the locus of movement of the centre of the femoral head about it. Our results explain why the Salter osteotomy does not lateralise the femoral head

Between May 1992 and April 1997, there were 20 452 births in the Blackburn District. In the same period 1107 infants with hip ‘at-risk’ factors were screened prospectively by ultrasound. We recorded the presence of dislocation and dysplasia detected under the age of six months using Graf’s alpha angle. Early dislocation was present in 36 hips (34 dislocatable and 2 irreducible). Of the 36 unstable hips, 30 (83%) were referred as being Ortolani-positive or unstable; 25 (69%) of these had at least one of the risk factors. Only 11 (31%) were identified from the ‘at-risk’ screening programme alone (0.54 per 1000 live births). Eight cases of ‘late’ dislocation presented after the age of six months (0.39 per 1000 live births). The overall rate of dislocation was 2.2 per 1000 live births. Only 31% of the dislocated hips belonged to a major ‘at-risk’ group. Statistical analysis confirmed that the risk factors had a relatively poor predictive value if used as a screening test for dislocation. In infants referred for doubtful clinical instability, one dislocation was detected for every 11 infants screened (95% confidence interval (CI) 8 to 17) whereas in infants referred because of the presence of any of the major ‘at-risk’ factors the rate was one in 75 (95% CI 42 to 149). Routine ultrasound screening of the ‘at-risk’ groups on their own is of little value in significantly reducing the rate of ‘late’ dislocation in DDH, but screening clinically unstable hips alone or associated with ‘at-risk’ factors has a high rate of detection

In acetabular dysplasia, fixation of the acetabular component of a cemented total hip prosthesis may be insecure and superolateral bone grafts are often used to augment the acetabular roof. We used finite element analysis to study the mechanical importance of the lateral acetabular roof and found that the lateral acetabular rim plays an important role in the load transfer of the pelvic bone. When the superlateral rim was lacking, the load shifted to the posterosuperior rim and to the area of pubic support, and the stresses in all materials, especially in the cement and in the trabecular bone, increased greatly. At the cement-bone interface the tilting component of the shear stress increased threefold. In a model in which the dysplastic acetabulum was augmented by a rigidly fixed, load-transmitting bone graft, the stresses were considerably diminished