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

The aim of this study was to evaluate the long-term clinical and radiographic outcomes of the Birmingham Interlocking Pelvic Osteotomy (BIPO).

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. 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. 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

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

Introduction. Trochleoplasty is an effective surgical procedure for patients with severe trochlear dysplasia and recurrent patella instability. Previous work has suggested patients demonstrate early improvements in knee function and quality of life. However, concerns regarding longer term outcomes due to the development of stiffness and patellofemoral osteoarthritis remain a concern for these patients. Our aim was to assess mid-term patient-reported outcome and quality of life measures for trochleoplasty performed at a single centre for severe trochlear dysplasia. Methods. Retrospective review of 28 knees (23 patients) having undergone trochleoplasty for severe trochlear dysplasia were reviewed. Due to the non-parametric nature of the data, median and interquartile range (IQR) were determined for pre-operative and mid-term follow-up scores. Statistically significant differences between groups were assessed using paired Wilcoxon-signed rank test with statistical significance set at p<0.05. Data were analysed using a statistical software package (IBM® SPSS® Statistics 26.0). Results. Median time from surgery to follow-up for all patients in the series was 5.3 years (IQR 3.9 – 7.0 years). Median pre-operative Kujala score improved from 57.0 (IQR 45 – 66) to 96.3 (IQR 83 -100). Median pre-operative IKDC score improved from 42.6 (IQR 35.9 – 51.3) to 92.6 (IQR 71.6 – 98.7). Median EQ-5D score also improved from 0.691 (IQR 0.414 – 0.727) to 1.000 (IQR 0.8178 – 1.000). Improvement in Kujala scores, IKDC scores and EQ-5D were all statistically significant with p<0.001 in all domains. Discussion. Our data suggests patients experience significant improvements in knee function and quality of life following trochleoplasty surgery for severe dysplasia. We demonstrate an absolute improvement in scores at a mean of 5.3 years follow-up of 39.3 points for Kujala, 50 for IKDC and 0.309 for EQ-5D. Minimal clinically important differences (MCID) have been reported to be in the region of 10 for the Kujala score, 8.8–15.6 for IKDC and 0.085 for EQ-5D. Our data shows improvements which far exceed the published MCID, suggesting trochleoplasty confers a large treatment effect and patients benefiting from sustained improvements in knee function and overall quality of life at mid-term follow-up. Conclusion. Following trochleoplasty for severe trochlear dysplasia, patient reported outcomes demonstrate continued improvements in knee function and quality of life at mid-term (5-year) follow-up. There is a large absolute treatment effect which likely impacts on both physical and psychological wellbeing for these patients. Continued surveillance of patient reported outcomes in this clinically complex cohort is indicated

Traditional radiographic criteria might underestimate or fail to detect subtle types of acetabular dysplasia. Acetabular sector angles (ASA) can measure the degree of anterior and posterior coverage of the femoral head on computed tomography (CT). This study aims to determine ASA values at different axial levels in a cohort of (1) asymptomatic, high-functioning hips without underlying hip pathology (controls); and (2) symptomatic, dysplastic hips that underwent periacetabular osteotomy (PAO). Thereby, we aimed to define CT-based thresholds for hip dysplasia and its subtypes. This is an IRB approved cross-sectional study of 51 high functioning, asymptomatic patients (102 hips) (Oxford Hip Score >43), without signs of osteoarthritis (Tönnis grade≤1), who underwent a CT scan of the pelvis (mean age: 52.1±5.5 years; 52.9% females); and 66 patients (72 hips) with symptomatic hip dysplasia treated with peri-acetabular osteotomy (PAO) (mean age: 29.3±7.3 years; 85.9% females). Anterior and posterior acetabular sector angles (AASA & PASA) were measured by two observers at three CT axial levels to determine equatorial, intermediate, and proximal ASA. Inter- and intra-observer reliability coefficient was high (between 0.882–0.992). Cut-off values for acetabular deficiency were determined based on Receiver Operating Characteristic (ROC) curve analysis, area under the curve (AUC) was calculated. The dysplastic group had significantly smaller ASAs compared to the Control Group, AUC was the highest at the proximal and intermediate PASA. Controls had a mean proximal PASA of 162°±17°, with a cut-off value for dysplasia of 137° (AUC: 0.908). At the intermediate level, the mean PASA of controls was 117°±11°, with a cut-off value of 107° (AUC 0.904). Cut-off for anterior dysplasia was 133° for proximal AASA (AUC 0.859) and 57° for equatorial AASA (AUC 0.868). Cut-off for posterior dysplasia was 102° for intermediate PASA (AUC 0.933). Measurement of ASA on CT is a reliable tool to identify dysplastic hips with high diagnostic accuracy. Posterior ASA less than 137° at the proximal level, and 107° at the intermediate level should alert clinicians of the presence of dysplasia

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. 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

The periacetabular osteotomy (PAO) is a well-described procedure for symptomatic acetabular dysplasia. For severe acetabular deformities, the efficacy of acetabular reorientation remains controversial and the literature on mid to long-term outcomes is limited. The purpose of this study was to analyze average 10-year clinical and radiographic results of the PAO for severe acetabular dysplasia. We retrospectively analyzed a consecutive series of patients undergoing PAO for severe acetabular dysplasia as defined by LCEA < 5˚. Patient demographics, radiographic measurements, modified Harris Hip score (MHHS), UCLA activity, SF-12, and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) were assessed. Sixty-eight patients (82 hips; 54 females) with an average age 20.7 and BMI of 24.4 kg/m2 were included. Mean follow-up was follow up was 10.3 years. . The LCEA and ACEA improved a mean of 32.8˚ (8.4˚ to 24.4˚, p<0.0001) and 31.6˚ (−4.9˚ to 26.7˚, p< 0.0001), respectively. MHHS improved an average of 17.5 points (64.6 to 82.3, p<0.0001), WOMAC Pain subscore improved an average of 21.1 points (65.7 to 85.0, p = 0.004), and SF-12 physical improved 11.8 points (from 40.3 to 50.6, p = 0.006). Activity improved as indicated by a 1.5-point increase in the UCLA Activity score (6.4 to 7.9, p=0.005). Six hips (9.1%) converted to THA at average 6.8 years post-PAO. Kaplan-Meier survival analysis with THA as the endpoint was 92% at 15 years (95% confidence interval [CI] (81%–96%). Multivariable linear regression analysis revealed concurrent osteochondroplasty was associated with a decreased risk of PAO failure. PAO is an effective treatment for severe acetabular dysplasia. At average 10.3 years, clinical and radiographic outcomes demonstrate pain relief, improved hip function, and major deformity correction. We observed minimal clinical deterioration over time

Finite element analysis (FEA) has been applied for the biomechanical analysis of acetabular dysplasia, but not for biomechanical studies of periacetabular osteotomy (PAO) or those performing analysis taking into consideration the severity of acetabular dysplasia. This study aimed to perform biomechanical evaluation of changes in stress distribution following PAO and to determine the effect of the severity of developmental dysplasia of the hip (DDH) using three-dimensional FEA. A normal model was designed with a 25° center-edge (CE) angle and a 25° vertical-center-anterior margin (VCA) angle. DDH models were designed with CE and VCA angles each of 10, 0, or −10°. Post-PAO models were created by separating each DDH model and rotating the acetabular bone fragment in the anterolateral direction so that the femoral head was covered by the acetabular bone fragment, with CE and VCA angles each at 25°. Compared to the normal hip joint model, the DDH models showed stress concentration in the acetabular edge and contacting femoral head, and higher stress values; stress increased with decreasing CE and VCA angles. Compared to the DDH models, the post-PAO models showed near-normal patterns of stress distribution in the acetabulum and femoral head, with stress concentration areas shifted from the lateral to medial sides. Stress dispersion was especially apparent in the severe acetabular dysplasia models. PAO provided greater decreases in the maximum values of von Mises stress in the load-bearing area of the acetabulum and femoral head when applied to the DDH models of higher degrees of severity, although the values increased with increasing severity of DDH. PAO is expected to provide biomechanical improvement of the hip joint, although the results also suggest a limitation in the applicability of PAO for the patients with severe acetabular dysplasia

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

Recurrent patellar instability is a common problem and there are multiple demographic and pathoanatomic risk factors that predispose patients to dislocating their patella. The most common of these is trochlear dysplasia. In cases of severe trochlear dysplasia associated with patellar instability, a sulcus deepening trochleoplasty combined with a medial patellofemoral ligament reconstruction (MPFLR) may be indicated. Unaddressed trochlear pathology has been associated with failure and poor post-operative outcomes after stabilization. The purpose of this study is to report the clinical outcome of patients having undergone a trochleoplasty and MPFLR for recurrent lateral patellofemoral instability in the setting of high-grade trochlear dysplasia at a mean of 2 years follow-up. A prospectively collected database was used to identify 46 patients (14 bilateral) who underwent a combined primary MPFLR and trochleoplasty for recurrent patellar instability with high-grade trochlear dysplasia between August 2013 and July 2021. A single surgeon performed a thin flap trochleoplasty using a lateral para-patellar approach with lateral retinaculum lengthening in all 60 cases. A tibial tubercle osteotomy (TTO) was performed concomitantly in seven knees (11.7%) and the MPFLR was performed with a gracilis tendon autograft in 22%, an allograft tendon in 27% and a quadriceps tendon autograft in 57% of cases. Patients were assessed post-operatively at three weeks and three, six, 12 and 24 months. The primary outcome was the Banff Patellar Instability Instrument 2.0 (BPII 2.0) and secondary outcomes were incidence of recurrent instability, complications and reoperations. The mean age was 22.2 years (range, 13 to 45), 76.7% of patients were female, the mean BMI was 25.03 and the prevalence of a positive Beighton score (>4/9) was 40%. The mean follow-up was 24.3 (range, 6 to 67.7) months and only one patient was lost to follow-up before one year post-operatively. The BPII 2.0 improved significantly from a mean of 27.3 pre-operatively to 61.1 at six months (p < 0 .01) and further slight improvement to a mean of 62.1 at 12 months and 65.6 at 24 months post-operatively. Only one patient (1.6%) experienced a single event of subluxation without frank dislocation at nine months. There were three reoperations (5%): one for removal of the TTO screws and prominent chondral nail, one for second-look arthroscopy for persistent J-sign and one for mechanical symptoms associated with overgrowth of a lateral condyle cartilage repair with a bioscaffold. There were no other complications. In this patient cohort, combined MPFLR and trochleoplasty for recurrent patellar instability with severe trochlear dysplasia led to significant improvement of patient reported outcome scores and no recurrence of patellar dislocation at a mean of 2 years. Furthermore, in this series the procedure demonstrated a low rate (5%) of complications and reoperations

Introduction. Fibrous dysplasia is a pathological condition, where normal medullary bone is replaced by fibrous tissue and small, woven specules of bone. Fibrous dysplasia can occur in epiphysis, metaphysis or diaphysis. Occationally, biopsy is necessary to establish the diagnosis. We present a review of operative treatment using the Ilizarov technique. The management of tibial fibrous dysplasia in children are curettage or subperiosteal resection to extra periosteal wide resection followed by bone transport. Materials and Methods. A total of 18 patients were treated between 2010 – 2020; 12 patients came with pain and 6 with pain and deformity. All patients were treated by Ilizarov technique. Age ranges from 4–14 years. 12 patients by enbloc excision and bone transportation and 6 patients were treated by osteotomy at the true apex of the deformity by introducing the k/wires in the medullary cavity with stable fixation by Ilizarov device. The longest duration for bone transport was 16 weeks (14–20 weeks) for application, after deformity correction was 20 weeks. We have never used any kind of bone grafts. Results. All the 18 patients were treated successfully by Ilizarov compression distraction device. The patients with localized tibial pathology with deformity had the shortest period on the Ilizarov apparatus, 14 weeks. Conclusions. Preservation and bone regeneration by distraction histogenesis constitutes a highly conservative limb saving surgery. Patients with bone defects of <10 cm, a great deal of preserved healthy tissue and good prognosis are good candidates for these methods

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

Hip dysplasia has traditionally been classified based on the lateral centre edge angle (LCEA). A recent meta-analysis demonstrated no definite consensus and a significant heterogeneity in LCEA values used in various studies to define hip dysplasia and borderline dysplasia. To overcome the shortcomings of classifying hip dysplasia based on just LCEA, a comprehensive classification for adult acetabular dysplasia (CCAD) was proposed to classify symptomatic hips into three discrete prototypical patterns of hip instability, lateral/global, anterior, or posterior. The purpose of this study was to assess the reliability of this recently published CCAD. One thirty four consecutive hips that underwent a PAO were categorized using a validated software (Hip2Norm) into four categories of normal, lateral/global, anterior or psosterior. Based on the prevalence of individual dysplasia and using KappaSize R package version 1.1, seventy four cases were necessary for reliability analysis: 44 dysplastic and 30 normal hips were randomly selected. Six blinded fellowship trained raters were then provided with the classification system and they looked at the x-rays (74 images) at two separate time points (minimum two weeks apart) to classify the hips using standard PACS measurements. Thereafter, a consensus meeting was held where a simplified flow diagram was devised before a third reading by four raters using a separate set of 74 radiographs took place. Intra-rater results per surgeon between Time 1 and Time 2 showed substantial to almost perfect agreement amongst the raters. With respect to inter-rater reliability, at time 1 and time 2, there was substantial agreement overall between all surgeons (kappa of 0.619 for time 1 and 0,623 for time 2). Posterior and anterior rating categories had moderate and fair agreement at time 1 and time 2, respectively. At time 3, overall reliability (kappa of 0.687) and posterior and anterior rating improved from Time 1 and Time 2. The comprehensive 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

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

Introduction. Acetabular dysplasia, also known as developmental dysplasia of the hip, has been shown to contribute to the onset of osteoarthritis. Surgical correction involves repositioning the acetabulum in order to improve coverage of the femoral head. However, ideal placement of the acetabular fragment can often be difficult due to inadequate visualization. Therefore, there has been an increased need for pre-operative planning and navigation modalities for this procedure. Methods. PubMed and EBSCO Host databases were queried using keywords (preoperative, pre-op, preop, before surgery, planning, plan, operation, surgery, surgical, acetabular dysplasia, developmental dysplasia of the hip, and Hip Dislocation, Congenital [Mesh]) from 1974 to March 2019. The search generated 411 results. We included all case-series, English, full-text manuscripts pertaining to pre-operative planning for congenital acetabular dysplasia. Exclusion criteria included: total hip arthroplasty (THA) planning, patient population mean age over 35, and double and single case studies. Results. A total of 12 manuscripts met our criteria for a total of 186 hips. Preoperative planning modalities described were: Amira (Thermo Fischer Scientific; Waltham, MA, USA) − 12.9%, OrthoMap (Stryker Orthopaedics; Mahwah, NJ, USA) − 36.5%, Amira + Biomechanical Guidance System (Johns Hopkins University) − 5.9%, Mills et al. method − 16.1%, Klaue et al. method − 16.1%, Armand et al. method − 6.5%, Tsumura et al. method − 3.8%, and Morrita et al. method − 2.2%. Virtual implementation of the Amira software yielded increases in femoral head coverage (p<0.05) and a significant decrease in lateral center edge angle (LCEA) (p<0.05). A significant decrease in post-surgical complications (0.0% navigated group vs. 8.7% non-navigated group, p<0.01) was found with usage of OrthoMap related planning. Conclusion. There was a notable lack of prospective studies demonstrating the efficacy of these modalities, with decreased post-surgical complications being the only added benefit of their use. Additionally, small sample sizes and lack of commercial availability for many of these programs further diminishes their applicability. Future studies are needed to compare computer assisted planning with traditional radiographic assessment of ideal osteotomy orientation. Furthermore, these programs must be readily accessible rather than be solely available to the researchers who wrote the program. For any figures or tables, please contact authors directly

Pelvic bone defect in patients with severe congenital dysplasia of the hip (CDH) lead to abnormalities in lumbar spine and lower limb alignment that can determine total hip arthroplasty (THA) patients' outcome. These variables may be different in uni- or bilateral CDH. We compared the clinical outcome and the spinopelvic and lower limb radiological changes over time in patients undergoing THA due to uni- or bilateral CHD at a minimum follow-up of five years. Sixty-four patients (77 hips) undergoing THA due to severe CDH between 2006 and 2015 were analyzed: Group 1 consisted of 51 patients with unilateral CDH, and group 2, 113 patients (26 hips) with bilateral CDH. There were 32 females in group 1 and 18 in group 2 (p=0.6). The mean age was 41.6 years in group 1 and 53.6 in group 2 (p<0.001). We compared the hip, spine and knee clinical outcomes. The radiological analysis included the postoperative hip reconstruction, and the evolution of the coronal and sagittal spinopelvic parameters assessing the pelvic obliquity (PO) and the sacro-femoro-pubic (SFP) angles, and the knee mechanical axis evaluating the tibio-femoral angle (TFA). At latest follow-up, the mean Harris Hip Score was 88.6 in group 1 and 90.7 in group 2 (p=0.025). Postoperative leg length discrepancy of more than 5 mm was more frequent in group 1 (p=0.028). Postoperative lumbar back pain was reported in 23.4% of the cases and knee pain in 20.8%, however, there were no differences between groups. One supracondylar femoral osteotomy and one total knee arthroplasty were required. The radiological reconstruction of the hip was similar in both groups. The PO angle improved more in group 1 (p=0.01) from the preoperative to 6-weeks postoperative and was constant at 5 years. The SFP angle improved in both groups but there were no differences between groups (p=0.5). 30 patients in group 1 showed a TFA less than 10º and 17 in group 2 (p=0.7). Although the clinical outcome was better in terms of hip function in patients with bilateral CDH than those with unilateral CDH, the improvement in low back and knee pain was similar. Patients with unilateral dysplasia showed a better correction of the PO after THA. All spinopelvic and knee alignment parameters were corrected and maintained over time in most cases five years after THA

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

MCID and PASS are thresholds driven from PROMS to reflect clinical effectiveness. Statistical significance can be derived from a change in PROMS, whereas MCID and PASS reflect clinical significance. Its role has been increasingly used in the world of young adult hip surgery with several publications determining the thresholds for Femoro-acetabular impingement FAI. To our knowledge MCID and PASS for patient undergoing PAO for dysplasia has not been reported. 593 PAOs between 1/2013 and 7/2023 were extracted from the Northumbria Hip Preservation Registry. Patients with available PROMS at 1year and/or 2years were included. PAOs for retroversion, residual Perthes and those combined with FO were excluded. MCID was calculated using the distribution method 0.5SD of baseline score(1). PASS was calculated using anchor method, ROC analysis performed, and value picked maximizing Youden index. A Logistic Regression analysis was performed to determine which independent variables correlated with achieving MCID and PASS. The MCID threshold for iHOt12 was 8.6 with 83.4 and 86.3 % of patients achieved it at 1 and 2 years respectively. The PASS score at 1 and 2 year follow up was 43 and 44 respectively, with 72.6 and 75.2% achieving it at 1 and 2 year postop. At 2 years a Higher preop iHOT 12 was associated with not achieving MCID and PASS (p<0.05). Preop acetabular version was negatively correlated with achieving MCID and previous hip arthroscopy was negatively correlated with PASS. The % of patients achieving MCID and PASS mimics that of FAI surgery (2). The negative correlation with preop iHOT12 reaffirms the importance of patient selection. The negative correlation of hip arthroscopy highlights the importance of having a high index of suspicion for dysplasia prior to hip arthroscopy and poorer outcomes of patients with mixed CAM and dysplasia pathology

Introduction. Acetabular dysplasia is typically characterised by insufficient antero-superior femoral head coverage. It is postulated (yet unproven) that patients with dysplasia compensate by reducing pelvic tilt (anterior pelvic rotation), effectively retroverting their acetabulum to improve antero-superior cover. We aimed to 1) define pelvic tilt (PT) in patients with bilateral and unilateral dysplasia, and 2) quantify PT changes following a successful periacetabular osteotomy (PAO). Patients/Materials and Methods. We retrospectively reviewed 16 patients (14 females) who underwent successful, bilateral, staged, PAOs (32 PAOs). These cases were matched for age and gender with 32 unilaterally dysplastic hips that underwent successful PAO for a similar degree of acetabular dysplasia as per pre-operative imaging. Supine and AP radiographs pre-PAO and at latest follow-up were used to measure centre-edge-angle (CEA) and Tönnis-angle (TA). PT was measured using two validated methods (Sacro-Femoral-Pubic (SFP) angle and Pubic-Symphysis to Sacro-Iliac (PS-SI) Index with excellent correlation (ρ=0.8, p<0.001). Results. Post-operatively, the TA/CEA improved from 20°±8/11°±9 to 5°±5/33°±6, respectively, with similar improvements between the unilateral and bilateral hips (p=0.9). PT pre-operatively was similar between unilaterally (8°±5) and bilaterally dysplastic hips (7°±5) (p=0.87). The change in PT was −1°±3; changing by >5° (all increased) in 6 patients (13%; 3 with unilateral PAOs and 3 with bilateral PAOs). Discussion. Patients with unilateral and bilateral hip dysplasia demonstrate similarities in PT. In 87% of cases PT remained the same following PAO, therefore was unlikely to have been compensatory. PT increased in 13% of cases post-PAO illustrating that a change in PT is possible and suggesting that the reduced tilt pre-PAO may have been compensatory to improve functional femoral head coverage in these patients. Conclusion. This is the first clinical and radiological evidence that patients with bilateral or unilateral dysplasia demonstrate similar pre- and post-operative PT, which remains unchanged in almost 90% of cases

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 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

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

Background. Hip dysplasia has traditionally been classified based on the lateral center edge angle (LCEA). A recent meta-analysis demonstrated no definite consensus and a significant heterogeneity in LCEA values used in various studies to define hip dysplasia and borderline dysplasia. To overcome the shortcomings of classifying hip dysplasia based on just LCEA, a comprehensive classification for adult acetabular dysplasia (CCAD) was proposed to classify symptomatic hips into three discrete prototypical patterns of hip instability; lateral/global, anterior, or posterior. The purpose of this study was to assess the reliability of this recently published CCAD. Methods. One hundred thirty-four consecutive hips that underwent a PAO were categorized using a validated software (Hip2Norm) into four categories of normal, lateral/global, anterior or posterior. Based on the prevalence of individual dysplasia and using KappaSize R package version 1.1, seventy-four cases were necessary for reliability analysis: 44 dysplastic and 30 normal hips were randomly selected. Five surgeons (3 fellowship trained in hip preservation) did a first reading (Time 1) to classify the hips, followed by four raters for a second reading (Time 2) minimum two weeks apart. Thereafter, a consensus meeting was held where a simplified flow diagram was devised before a third reading by four raters using a separate set of 74 radiographs took place. Results. There was substantial and almost perfect intra-rater agreement for 4 surgeons between Time 1 and Time 2. At time 1 and time 2, there was substantial agreement overall between all surgeons; however, posterior and anterior rating categories had moderate and fair agreement at time 1 and time 2, respectively. At time 3, overall reliability and posterior and anterior rating improved from Time 1 and Time 2. Conclusion. This comprehensive 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

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

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

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 this purpose to guide clinicians in order to treat PFI. There are also concerns about 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 intra-observer agreement in the assessment of TD. The aim of this study was to compare the reliability and reproducibility of these two classifications. 6 assessors (4 consultants and 2 registrars) independently evaluated 100 magnetic resonance axial images of the patella-femoral joint for TD and classified them according to OBC and DJC. These assessments were again repeated by all raters after 4 weeks. The inter and intra-observer reliability scores were calculated using Cohen's kappa and Cronbach's alpha. Both classifications showed good to excellent interobserver reliability with high alpha scores. The OBC classification showed a substantial intra-observer 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 to those by registrars, in either classification systems. This large study from a non-founding institute shows both classification systems to be reliable for classifying TD based on magnetic resonance axial images of the patella-femoral joint, with the simple to use OBC having a higher intra-observer reliability score compared to the DJC

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

A risk factor for patellofemoral instability is trochlear dysplasia. Trochleoplasty is a surgical procedure used to reshape the trochlear groove to improve patellar stability. This study seeks to compare pre-op MRI measurements and post operative MRI measurements for patients who have undergone trochleoplasty in correlation with their clinical outcomes scores. Data was collected from a database of patients known to have trochlear dysplasia who underwent trochleoplasty. Radiological Data was collected pre-op and subsequent post op MRI data collected included TT-TG, Patella Tilt, IS, sulcus angle. Data score sheets pre-op and post op trochleoplasty completed by patients were also collected. 10 patients had pre and post op MRI's documented. 80%(8/10) females and 20%(2/10) males, average age of 30 years old (range 23 – 32 years old). Average MRI pre-op scores: IS ratio: 1.2, Patella tilt: 24.14, sulcus angle 160.13, and TT-TG distance of 16.94. 1 year average MRI post-op scores: IS ratio: 1.28, Patella tilt 15.56, sulcus angle 148.66 and TT-TG distance 16.78. 1 year post op Kujala and Norwich instability scores patient reported improved stability, function and confidence post op compared to pre-op. Subjective and objective scores reflected an improvement of stability. MRI demonstrated a deeper trochlear groove post-operatively which should provide resistance against lateral patella movement and patellar dislocations. TT-TG pre and post op remained constant. Pre op and post op Kujala scores reflected improved function. The Norwich instability scores pre and post op reflected satisfaction of treatment. There are not a lot of studies published on trochleoplasty. Based on this study it is clear that patients with patellofemoral instability with severe trochlear dysplasia will benefit from trochleoplasty. The sample size of the data analysis was only 10. However it reflected that function 1 year post procedure remained stable

In Japan, osteoarthritis (OA) of the hip secondary to acetabular dysplasia is very common, and there are few data concerning the pathogeneses and incidence of femoroacetabular impingement (FAI). We have attempted to clarify the radiological prevalence of painful FAI in a cohort of Japanese patients and to investigate the radiological findings. We identified 176 symptomatic patients (202 hips) with Tönnis grade 0 or 1 osteoarthritis, whom we prospectively studied between August 2011 and July 2012. There were 61 men (65 hips) and 115 women (137 hips) with a mean age of 51.8 years (11 to 83). Radiological analyses included the α-angle, centre–edge angle, cross-over sign, pistol grip deformity and femoral head neck ratio. Of the 202 hips, 79 (39.1%) had acetabular dysplasia, while 80 hips (39.6%) had no known aetiology. We found evidence of FAI in 60 hips (29.7%). Radiological FAI findings associated with cam deformity were the most common. There was a significant relationship between the pistol grip deformity and both the α-angle (p < 0.001) and femoral head–neck ratio (p = 0.024). Radiological evidence of symptomatic FAI was not uncommon in these Japanese patients. Cite this article: Bone Joint J 2013;96-B:172–6

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

Dysplasia has long been identified as a high-risk group for total hip replacement(THR). The underlying causes include younger age, underlying joint deformity, and greater tissue laxity. A higher failure rate has also been identified for hip resurfacing arthroplasty (HRA) in these patients. Many experts have advised avoiding HRA in these patients, although comparative studies are not available. We do not practice patient selection, because THR has not been proven any more reliable for these patients. Instead, we have taken the approach of studying the causes of failure and finding methods to improve the results of HRA in dysplasia patients. We have identified three primary failure modes for the young women who typically have dysplasia: failure of initial acetabular ingrowth (FAI), adverse wear related failure (AWRF), and early femoral failure (EFF: femoral neck fracture and head collapse). Improvements in technique to address all of these failure modes were in place by 2008: acetabular components with supplemental fixation for severe deformities (trispike), guidelines and intraoperative x-ray techniques to eliminate malpositioned acetabular components resulting in edge-loading, uncemented femoral fixation and a bone management protocol that has eliminated early femoral failure. Group I includes 142 cases done before 2008 and Group II includes 168 cases with minimum 2-year follow-up done after this date. Two-year failure rates improved from 5% (8/142) to 0.6% (1/168) and 5-year Kaplan-Meier survivorship improved from 93% to 99%. In Group II we have had only one failure (femoral neck fracture) in 168 dysplasia cases with 2–5 year follow-up. There have been no failures of acetabular ingrowth, no AWRF, no femoral head collapse, no failures of femoral ingrowth, no femoral loosenings, no dislocations and no nerve palsies. All acetabular components placed since 2008 meet our published RAIL (relative acetabular inclination limit) guidelines, which we have shown to be 99% reliable in avoiding high on levels and AWRF. Both groups were 70% female. With a mean bearing size 48mm (high-risk for HRA). There was also no differences in DEXA scan T score, BMI, ASA score, length of incision (4 inches) HHS, or patients participating in impact sports (UCLA activity score 9&10). In Group II the mean age was 3 years greater (52), the mean operative time was 20 minutes shorter (96 minutes), estimated blood loss was 120 ml less (140ml) and the mean hospital stay was one day shorter (2 days) probably reflective of greater experience in this single surgeon series. We have demonstrated that with sufficient surgeon experience and properly designed implants, hip resurfacing can be performed with a failure rate that is lower than most reports on THR for this disorder

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

We 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

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

The Acetabular Dysplasia creates serious technical problems for the insertion of acetabular socket. In first, cup must have a good primary stable fixation in a shallow acetabulum. In second, smaller cups are usually required in dysplasia hip, but small sockets must have thick polyethylene wall. And in third, cup design must ensuring easy reconstruction of the anterolateral bone defect. In our opinion, all these problems can be achieved by using of cementless acetabular Robert Mathys (RM) cups. Materials and Methods. In the period from 1996 to 2000 168 patients with ace-tabular dysplasia were operated with titanium powder coated RM cups. The patients age was from 18 to 75 years old (average 43,5). In 77 patients with type I dysplasia (AAOS classification) a primary stable fixation of the acetabular component in a good position without of filling bone defect was achieved. In 53 patient with type II dysplasia stable fixation was supplemented by closing of a cup by filler bone grafts in a place of bone defect. In case of type III dysplasia (38 patients) with very shallow acetabulum and extensive bone defects initial stabilization was achieved by the press-fit one or two anchoring pegs and insertion cancellous screws. In type III dysplasia the massive bone transplant was fixed by additional screws. The features of a design of a cup allowed to stop on the small socket sizes without danger of use implant with critically thin polyethylene wall. It considerably improved a covering of a cup. Results. In 166 patients (98,8 %) a good medium-term results (2–6 years) were obtained. The radiologic controls have shown that the prostheses underwent good osteointegration. 2 patients (1,2%) needed revision. Of them one patient had a infected complication, one other had an aceptic necrosis of acetabulum and secondary cup migration. In all other patients no osteolisis was observed. The good primary fixation of the RM cup decreased the risk of aseptic loosing of the autologous bone graft. The temporal partial (not more than 1/3) bone graft resorption was find in 33,9 % at the type II and 42,1 % at the type III dysplasia. After 2 years in all cases we observed improvement of the bone stock quality at the site of bone grafting. Conclusions. The features of a design of RM cup allow to use implants of the small size. The application for cup fixation pegs and screws allows to receive its reliable primary stability even at expressed acetabular dysplasia. The good primary stability and ease of application of bone grafts allows to achieve with RM cup of an overall objective of operation - maximal restoration of anatomy and biomechanics of the dysplastic hip joint

Purpose: Fibrous dysplasia is a rare condition accounting for about 1% of benign bone tumours. Both mono- and polyostotic dysplasia is described. Treatment of small tumours is not particularly difficult, but extensive or complicated tumours are another problem. The purpose of this work was to present the results of surgical treatment of a consecutive series of 64 patients. Particular focus was placed on complications. Material and methods: This retrospective series included 64 patients with fibrous dysplasia. Mean age was 32 years. There were 37 women and 27 men. Monostotic dysplasia was noted in 58 cases. The femur was the most frequent localisation (n=19). Seven patients had a fracture. Histological proof was obtained in 61 patients. In three patients, fibrous dysplasia was associated with another tumour (aneurysmal cyst or adamantinoma). Several therapeutic strategies were proposed: abstention for three patients, biopsy alone for 13, resection for six, curettage for 34. The curettage was filled in 23 cases with osteosynthesis in 11. Eight patients were given other surgical treatments (prosthesis, osteosynthesis, amputation). Results: We had five postoperative complications. At mean follow-up of 45 months, 14% of the patients still suffered from generally mild to moderate pain. Radiologically, there were four recurrences, 12 unchanged, 18 regressions, and 19 remissions (generally after resection or curettage with filling). Long-term follow-up revealed malignant transformation in two patients. Discussion: Lesions treated by biopsy alone cured or regressed in 30% of the cases while curettage with filling led to cure or regression in 70% of the cases. We did not include patients treated by bisphosphonates in this series because of insufficient follow-up. Nevertheless, this treatment appears to have a promising effect on pain. Conclusion: Results of treatment for fibrous dysplasia are not as good as might be suspected for this benign disease. The role of surgery appears to be on the decline, to be replaced by bisphosphonates whose long-term efficacy must be evaluated

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

Objective: To review of an uncommon deformity arising in four patients. Method: A clinical and radiological review. Conclusions: heightened awareness and early treatment with monitoring is required. We describe four cases of distal radial epiphyseal dysplasia associated with a localised area of cutis aplasia congenita (CAC) over the dorsum of the distal forearm. The cutis aplasia was diagnosed at birth in all cases, but the radial dysplasia was not recognised until presentation to our orthopaedic department between the ages of 5 and 10 years. Radial dysplasia describes a spectrum of osseous, musculotendinous, and neurovascular dysplasias of the pre-axial border of the upper limb, and is the most common form of longitudinal deficiency. Cutis aplasia congenita involves an ulcerated area lacking in normal skin formation, present at birth. The most common site is on the scalp, but it has been described on the extremities, and overlying embryological malformations. This association, the long-term implications and the requirement for follow-up until skeletal maturity have not previously been described. We emphasise the importance of continued monitoring of these patients as the effects of radial dysplasia did not become obvious for several years, and the potential benefit from achieving early skin cover with grafting rather than allowing healing by secondary intention is discussed. It is important to increase awareness of this condition so that early orthopaedic and plastic surgical opinions can be sought, in order to reduce the disabling effect on the underlying radius

Introduction. Acetabular dysplasia is associated with an increased risk of hip pain and early development of osteoarthritis (OA). The Bernese peri-acetabular osteotomy (PAO) is the most well-established technique in the Western world for the treatment of symptomatic acetabular dysplasia. This case-control study aims to assess whether the severity of acetabular dysplasia has an effect on outcome following Peri-Acetabular Osteotomy (PAO) and/or the ability to achieve desired acetabular correction. Patients/Materials & Methods. A prospective, multicentre, longitudinal cohort of consecutive PAOs was reviewed. Of the available 381 cases, 61 hips had pre-PAO radiographic features of lesser-dysplasia [Acetabular-Index (AI)<15° and Lateral-Centre-Edge-Angle (LCEA)>15°) and comprised the ‘study-group’. ‘Study-Group’ was matched for all factors known to influence outcome post-PAO [age, gender, BMI, Tönnis-grade and joint congruency (p=0.6–0.9)] with a ‘Comparison-Group’ of pronounced dysplasia (n=183). Clinical outcomes, complications and the ability to achieve optimum correction (LCEA: 25°–40°/AI: 0°–+10°) were compared. Results. At a mean follow-up of 4(±1.5) years, 3 hips had a THA and 13 underwent further procedures; 21 major complications occurred. The mean improvement in HOOS was 28(±23). No differences in complication- or re-operation- rates were detected between study- and comparison groups (p=0.29). Lesser-dysplastic hips had inferior HOOS compared to pronounced dysplastic hips, both pre- (52Vs.59) and post-operatively (73Vs.78); however, similar improvements were seen. Amongst the lesser dysplastic hips, those that required a femoral osteochondroplasty at PAO had significantly inferior pre-operative HOOS (48±18), compared to those that didn't require an osteochondroplasty (60±17) (p=0.04). Increased ability to achieve optimum acetabular correct was seen (80Vs59%, p=0.4) in the lesser dysplastic hip. Discussion. A PAO is safe and efficacious in the treatment of lesser dysplasia. The mildly dysplastic hips with cam deformity that required concurrent FOCP and PAO, were the most symptomatic. Future studies should aim to optimize diagnosis and management in this challenging, combined deformity cohort

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

It is generally agreed that in acetabular dysplasia the acetabulum lies excessively anteverted. Although this is true for the majority of hips, we have found that in some patients with dysplastic hips, the acetabulum lies unexpectedly in retroversion. Aim: To investigate the proportion of dysplastic hips which are retroverted. Method: We studied the radiographs of over seven hundred patients with dysplastic hips and who had had a periacetabular osteotomy in the period 1984–1998. We excluded patients with neuromuscular dysplasia, Perthes disease of the hip, post-traumatic dysplasia and proximal focal femoral deficiency. We selected 232 radiographs of patients with congenital acetabular dysplasia. A number of parameters were measured including, lateral centre edge angle, anterior centre-edge angle, acetabular index of weight bearing surface, femoral head extrusion index and acetabular index of depth to width. Also recorded was acetabular version and congruency between femoral head and acetabulum. Results: The lateral centre-edge angle of Wiberg had a mean value of 6.4° (SD 8.9°), the mean anterior centre-edge angle was 1.3° (SD 13.5°) and the acetabular index of weight bearing surface of the acetabulum had a mean value of 24.5° (SD 9.7°). The majority (192, 82.8%) of acetabula were anteverted as might be expected. However, a significant minority (40,17.2%) were retroverted. The mean anterior centre-edge angle in retroverted hips was 6.7° (SD 9.4°) compared with 0.4° (SD 13.3°) in anteverted hips. Conclusion: The authors have shown that in a typical group of patients with congenital acetabular dysplasia, significant enough to warrant periacetabular osteotomy, the majority of hips as expected have anteverted acetabula. However, a significant minority are retroverted. This finding has an important bearing in the performance of the osteotomy

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

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

Purpose. Patients with acetabular dysplasia demonstrate altered biomechanics during gate and other activities. We hypothesized that these patients exhibit a compensatory increase in the anterior pelvic tilt during gait. Materials & Methods. Twelve patients were included in this prospective radiographic and gait analysis study prior to the PAO. All were women. The mean age was 27 years (+/− 8 yrs). Tonnis grade was zero in nine, and one in three hips. All patients performed multiple one-minute walking trials on the level, the incline, and the decline treadmill surfaces in an optical motion capture lab. Anterior pelvic tilt is reported in (+), while the posterior pelvic tilt is reported in (–) values. Results. Radiographic Data. : The mean alpha angle measured from the Dunn and the frog lateral images was 63.0º±17.4, and 54.7º±16.4, respectively. The mean LCEA was 14.9°±6.1, and the mean anterior center edge angle was 18.3°±8.9. the mean acetabular version at 1, 2, and 3 o'clock were 12.1°±11.6, 29.2°±9.9, and 23.3°±7.4, respectively. Intra-class correlation coefficient (ICC) for these measurements were 0.934, 0.895, and 0.971, respectively. The mean femoral anteversion, as measured on the 3D CT scan was 21.3°±16.1. The mean hip flexion range was 107.1°± 7.2. The mean pelvic tilt was 88.7 mm ± 14.4 using the PS-SI distance with an ICC of 0.998. Gait Data. : Baseline measurements were done in the standing position. On the leveled surface, 5 patients had anterior (+) while 7 had posterior (−) pelvic tilt. The mean posterior pelvic tilt was 1.0° with the range of −2.8° to +0.67°. On the inclined surface, all patients had posterior (−) pelvic tilt. The mean pelvic tilt was −4.9° with the range of −6.4° to −3.1°. On the declined surface, 8 patients had anterior (+) while 4 patients had posterior (−) pelvic tilt. The mean pelvic tilt was −0.39° with the range of −1.9° to +1.0°. The pelvic tilt was negatively correlated with the PS-SI distance on all three surfaces with the Spearman coefficients of −0.27, −0.04, and −0.18 on the 3 different surfaces, respectively. Conclusion. Our results demonstrated that the patients with hip dysplasia exhibit variable degrees of the pelvic tilt while walking on different surface inclinations. Weak negative correlation with the standing pelvic tilt measurements from the radiographs suggests that those patients with more anterior standing pelvic tilt tend to have greater compensatory posterior tilt during gait

Important issues related to total hip replacement for dysplasia are: placement of the cup and bone stock, the role of femoral osteotomy, and the choice of acetabular and femoral components. The cup can be placed at the correct or near correct anatomical level with or without a bone graft, in a high position (high hip center) or at the right level in a protruded position. All three techniques can provide adequate coverage of the cup. In the high hip position bone graft is not usually necessary to obtain cup coverage. There is, however, a higher rate of component loosening, a higher dislocation rate, and lengthening is limited to the femoral side. Placing the cup in a protruded position to obtain coverage does not restore bone stock for future surgery, but it does place the hip at the correct level. Placing the cup in the correct anatomical position (i.e. at the right level and not protruded) may require a structural autograft which adds to the complexity of the case. However, bone stock is restored for future surgery. Revision of acetabular components that have had a shelf graft have a survivorship of 96.5% at 10 years and only 5 of 34 required structural support (2 augments, 3 structural allografts). Femoral osteotomy may be used as part of the exposure for diaphyseal shortening or for derotation of excessive anteversion. The osteotomy is carried out in the sub-trochanteric region and may be oblique, step-cut or transverse. Fixation of the osteotomy is achieved via the stem, a plate, or a cortical strut. Cementless components are usually used because of the relatively young age of this patient population. Small components may be necessary. On the femoral side, the stem should be straight or modular so excessive anteversion can be neutralised

To investigate the proportion of dysplastic hips which are retroverted. We studied the radiographs of over seven hundred patients with dysplastic hips who had had a periacetabular osteotomy in the period 1984–1998. We excluded patients with neuromuscular dysplasia, Perthes’ disease of the hip, post-traumatic dysplasia and proximal focal femoral deficiency. We selected 232 radiographs of patients with congenital acetabular dysplasia. A number of parameters were measured including lateral centre-edge angle, anterior centre-edge angle, acetabular index of weight-bearing surface, femoral head extrusion index and acetabular index of depth to width. Also recorded were acetabular version and congruency between femoral head and acetabulum. The lateral centre-edge angle of Wiberg had a mean value of 6.4° (SD 8.9°), the mean anterior centre-edge angle was 1.3° (SD 13.5°) and the acetabular index of weight-bearing surface of the acetabulum had a mean value of 24.5° (SD 9.7°). The majority (192, 82.8%) of acetabula were anteverted as might be expected. However, a significant minority (40, 17.2%) were retroverted. The mean anterior centre-edge angle in retroverted hips was 6.7° (SD 9.4°) compared with 0.4° (SD 13.3°) in anteverted hips. The authors have shown that, in a typical group of patients with congenital acetabular dysplasia significant enough to warrant periacetabular osteotomy, the majority of hips as expected have anteverted acetabula. However, a significant minority are retroverted. This finding has an important bearing on the performance of the osteotomy. We have also found that most if not all the information required prior to and following periac-etabular osteotomy can be obtained from an orthograde view of the pelvis

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

The purpose of our study was to assess the reproducibility and reliability of traditional radiographic methods of diagnosing trochlear dysplasia compared with the gold standard of MRI. Plain radiographs and MRI scans of 36 knees in 28 patients with proven trochlear dysplasia were compared with 18 controls in a single surgeon's practice. The sulcus angle on MRI and axial radiographs was measured. The lateral radiograph was assessed for adequacy, the crossing sign and the trochlear bump sign. The mean axial (radiograph) and MRI sulcus angles in the dysplastic group were 145° and 158° respectively (p=0.0001) compared to 135° and 138° in the control group (p=0.09). The crossing sign was present in 34 out of 36 lateral radiographs in the dysplastic group and 8 out of 18 in the control group equating to a sensitivity of 94% and specificity of 56%. The quality of the lateral radiograph did not significantly alter the sensitivity or specificity of the crossing sign (p=0.01). The bump sign was present in 22 out of 36 lateral radiographs in the dysplastic group and 1 out of 18 in the control group equating to a sensitivity of 61% and specificity of 94%. Our study shows that an increased radiographic sulcus angle is reliable in diagnosing trochlear dysplasia but underestimates it. The bump sign is a reliable predictor of trochlear dysplasia. The crossing sign is sensitive but not specific in diagnosing dysplasia

Ultrasound (US) is the standard imaging modality used to screen for developmental dysplasia of the hip in infants. Currently, radiologists or orthopaedic surgeons review scan images and judge them to be adequate or inadequate for interpretation. If considered adequate, diagnostic dysplasia metrics are determined; however, there is no standardised method for this process. There is significant inter-observer variability in this manual process which can affect misdiagnosis rates. To eliminate this subjectivity, we developed an automatic method to identify adequate US images and extract dysplasia metrics. The purpose of this study was to validate the efficacy of this automatic method by comparing results with observer-determined dysplasia metrics on a set of US images. A total of 693 US images from scans of 35 infants were analysed. Trained clinicians at a single institution labeled each image as adequate or inadequate, and subsequently measured alpha and beta angles on adequate images to diagnose dysplasia. We trained our image classifier on random sets of 415 images and used it to assess the remaining 278 images. Alpha and beta angles were automatically estimated on all adequate images. We compared the manual and automatic methods for discrepancies in adequacy determination, metric variability and incidences of missed early diagnosis or over-treatment. There was excellent agreement between the automatic and manual methods in image adequacy classification (Kappa coefficient = 0.912). On each adequate US image, alpha and beta angle measurements were compared, producing mixed levels of agreement between methods. Mean discrepancies of 1.78°±4.72° and 8.91°±6.437° were seen for alpha and beta angles, respectively. Standard deviations of the angle measures across multiple images from a single patient scan were significantly reduced by the automatic method for both alpha (p<0.05) and beta (p<0.01) angles. Additionally, the automatic method classified three hips (two patients) as Graf type II and two hips (two patients) as type III, while the manual method classified them as type I and II, respectively. Both cases flagged as type III patients by the automatic system subsequently failed Pavlik harness treatment and were booked for surgery. The automatic method produced excellent agreement with radiologists in scan adequacy classification and significantly reduced measurement variability. Good agreement between methods was found in Graf classification. In instances of disagreement, subsequent clinical findings seemed to support the classification of the automatic method. This proposed method presents an alternative automatic, near-real-time analysis for US images that may potentially significantly improve dysplasia metric reliability and reduce missed early diagnoses without increasing over-treatment

The purpose of this study was to examine a cohort of patients with minor acetabular dysplasia features in order to identify the preoperative clinical characteristics and imaging findings that differentiate patients with hip instability from patients with impingement. A retrospective cohort study of patients with borderline acetabular dysplasia was performed. All patients were identified by prospective radiographic evaluation with an LCEA between 20° and 25°. Multivariate statistical analyses were used to identify independent predictors of disease type. Of the 143 hips in the cohort, 39.2% (n=56) had the diagnosis of instability, while 60.8% (n=87) had the diagnosis of impingement. The cohort included 109 females (76.2%) and 34 males (23.8%). Hips with instability had a lower LCEA (21.8° vs. 22.8°; p<0.001), lower ACEA (23.3° vs. 26.6°; p=0.002), a higher AI (11.8° vs. 8.5°; p<0.001), and a lower maximum alpha angle (54.4° vs. 61.1°; p=0.001). The odds of instability increased 1.7 times for each one-degree decrease in LCEA, 1.4 times for each one-degree decrease in ACEA, and 1.1 times for each one-degree increase in acetabular inclination (all p0.003). Female sex was strongly associated with instability. The instability subgroup had greater range of motion (IRF, 22.7° vs. 12.4°, p<0.001) and total arc of motion (IRF+ERF, 61.2° vs. 47.4°, p<0.001). We identified predictors of diagnosis including: acetabular inclination (1.49, p<0.001), ACEA (0.89, p=0.007), crossover sign (0.27, p=0.014), preoperative mHHS (0.96, p=0.014), IRF (1.10, p=0.001), and age (0.88, p=0.001). Patients with symptomatic instability tend to have increased acetabular inclination, decreased ACEA, greater functional limitations, younger, greater IRF, while hips with impingement demonstrate the opposite trends

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

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. 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

Aim: To review an uncommon deformity arising in four patients. Method: A clinical and radiological review. Abstract: We describe four cases of distal radial epiphyseal dysplasia associated with a localised area of cutis aplasia congenita (CAC) over the dorsum of the distal forearm. The cutis aplasia was diagnosed at birth in all cases, but the radial dysplasia was not recognised until presentation to our orthopaedic department between the ages of 5 and 10 years. Radial dysplasia describes a spectrum of osseous, musculotendinous, and neurovascular dysplasias of the pre-axial border of the upper limb, and is the most common form of longitudinal deþ ciency. Cutis aplasia congenita involves an ulcerated area lacking in normal skin formation, present at birth. The most common site is on the scalp, but it has been described on the extremities, and overlying embryological malformations. This association, the long-term implications and the requirement for follow-up until skeletal maturity have not previously been described. We emphasise the importance of continued monitoring of these patients as the effects of radial dysplasia did not become obvious for several years, and the potential beneþt from achieving early skin cover with grafting rather than allowing healing by secondary intention is discussed. It is important to increase awareness of this condition so that early orthopaedic and plastic surgical opinions can be sought, in order to reduce the disabling effect on the underlying radius. Conclusions: Heightened awareness and early treatment with monitoring is required

We report a case of Perthes like changes in the rare disorder of Geleophysic dysplasia and add it to the world literature (24 cases). We found an increased incidence of Perthes at 12% (3/25). Geleophysic dysplasia is characterised by short stature with short limbs distally, normal intelligence, joint stiffness, hepatomegaly and happy facial characteristics. The disease has the clinical and histological appearance of a mucopolysaccharidosis. Perthes changes were seen at the age of 4 years and treated conservatively, with poor clinical results despite the early onset and reasonable radiological improvement. MRI scans of the affected hip did not show any unusual features that differentiate it from normal Perthes disease. The patient also developed carpal tunnel syndrome, which has been seen in with increasing frequency in mucopolysaccharidoses such as Hurlers syndrome, but has not been associated with Geleophysic dysplasia before

Introduction: Anterior Knee pain is a very common presentation in Orthopaedic out patient clinics. However, Trochlear Dysplasia as a condition is still not very well understood. Materials and Methods: Operative findings in patients undergoing Trochleaplasty and in young patients undergoing Patella-femoral arthroplasty were studied. Patients having trochlear dysplasia were identified. All these patients were sent a questionnaire to explore the variety of symptoms they experienced. 87 patients were identified for the study. 71(82%) patients responded to the questionnaire. Results: Two subgroups were identified, one with dislocation of patella and the other without dislocation of the patella. Patients in patella dislocation group were younger (12 years vs 24) than those who presented without dislocation of patella. Patella dislocation group had more patients with anterior knee pain (71% vs 52%) as teenagers as compared to the group without dislocations. Symptoms such as giving way, lack of trust and unable to participate in sports were more commonly seen in the group with patella dislocation whereas, limited walking distance and difficulty with climbing the stairs were more commonly seen in the group without dislocation. Symptoms such as pain, catching and locking were seen equally amongst the two groups. Conclusion: We believe that the patients with trochlear dysplasia have a bimodal presentation. Patients with dislocation of patella present earlier than those without patella dislocation. These two groups also have different symptoms at presentation. It is important to identify these subgroups correctly for appropriate management

The aim of this study was to review the early outcome of the Femoro-Patella Vialla (FPV) joint replacement. A total of 48 consecutive FPVs were implanted between December 2007 and June 2011. Case-note analysis was performed to evaluate the indications, operative histology, operative findings, post-operative complications and reasons for revision. The mean age of the patients was 63.3 years (48.2 to 81.0) and the mean follow-up was 25.0 months (6.1 to 48.9). Revision was performed in seven (14.6%) at a mean of 21.7 months, and there was one re-revision. Persistent pain was observed in three further patients who remain unrevised. The reasons for revision were pain due to progressive tibiofemoral disease in five, inflammatory arthritis in one, and patellar fracture following trauma in one. No failures were related to the implant or the technique. Trochlear dysplasia was associated with a significantly lower rate of revision (5.9% vs 35.7%, p = 0.017) and a lower incidence of revision or persistent pain (11.8% vs 42.9%, p = 0.045). . Focal patellofemoral osteoarthritis secondary to trochlear dysplasia should be considered the best indication for patellofemoral replacement. Standardised radiological imaging, with MRI to exclude overt tibiofemoral disease should be part of the pre-operative assessment, especially for the non-dysplastic knee. Cite this article: Bone Joint J 2013;95-B:793–7

To confirm whether developmental dysplasia of the hip has a risk of hip impingement, we analysed maximum ranges of movement to the point of bony impingement, and impingement location using three-dimensional (3D) surface models of the pelvis and femur in combination with 3D morphology of the hip joint using computer-assisted methods. Results of computed tomography were examined for 52 hip joints with DDH and 73 normal healthy hip joints. DDH shows larger maximum extension (p = 0.001) and internal rotation at 90° flexion (p < 0.001). Similar maximum flexion (p = 0.835) and external rotation (p = 0.713) were observed between groups, while high rates of extra-articular impingement were noticed in these directions in DDH (p < 0.001). Smaller cranial acetabular anteversion (p = 0.048), centre-edge angles (p < 0.001), a circumferentially shallower acetabulum, larger femoral neck anteversion (p < 0.001), and larger alpha angle were identified in DDH. Risk of anterior impingement in retroverted DDH hips is similar to that in retroverted normal hips in excessive adduction but minimal in less adduction. These findings might be borne in mind when considering the possibility of extra-articular posterior impingement in DDH being a source of pain, particularly for patients with a highly anteverted femoral neck. Cite this article: Bone Joint J 2014;96-B:580–9

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

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

Introduction: This study assesses the prevalence of trochlear dysplasia as a cause of patello-femoral arthritis (PFOA), though for many years the condition was scarcely recognised and not recorded. Method: Since 1989 385 primary isolated patello-femoral replacements have been performed in Bristol for patello-femoral arthritis. The diagnoses recorded were:. Lateral facet PFOA 227. Symmetrical PFOA 95. Medial facet PFOA 24. Dislocation 15. Subluxation 33. Post traumatic 6. Trochlear dysplasia was noted either retrospectively or as “other diagnosis” in only 10 cases between 1989 and 2003, however in the last year, the condition became recognised locally, and has been recorded in 11 of the 48 cases performed. A recent review of 40 cases aged under 55 showed evidence of causative trochlear dysplasia in more than 50%. This was based on:. Trochlear dysplasia recorded on operative note 8. A positive crossing sign on a true lateral xray 19. A sulcus angle > 144° 20. Conclusion: Trochlear dysplasia frequently leads to severe symptomatic patello-femoral arthritis, often at an early age, and can be helped by isolated patello-femoral replacement. Since only 38 of the 385 cases had undergone re-alignment procedures it seems likely that the condition initially presents in a variety of ways

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

Introduction. Bulk bone grafting is commonly used in total hip arthroplasty (THA) for developmental dysplasia. However, it is a technically demanding surgery with several critical issues, including graft resorption, graft collapse, and cup loosening. The purpose of this study is to describe our new bone grafting technique and review the radiographic and clinical results. Patients and Methods. We retrospectively reviewed 105 hips in 89 patients who had undergone covered bone grafting (CBG) in total hip arthroplasty for developmental dysplasia. We excluded patients who had any previous surgeries or underwent THA with a femoral shortening osteotomy. According to the Crowe classification, 6 hips were classified as group I, 39 as group II, 40 as group III, and 20 as group IV. Follow-up was at a mean of 4.1 (1 ∼ 6.9) years. The surgery was performed using the direct anterior approach. The acetabulum was reamed as close to the original acetabulum as possible. The pressfit cementless cup was impacted into the original acetabulum. After pressfit fixation of the cup was achieved, several screws were used to reinforce the fixation. Indicating factor for using CBG was a large defect where the acetabular roof angle was more than 45 degrees and the uncovered cup was more than 2 cm (Fig.1). The superior defect of the acetabulum was packed with a sufficient amount of morselized bone using bone dust from the acetabular reamers. Then, the grafted morselized bone was covered with a bone plate from the femoral head. The bone plate was fixed with one screw to compact the morselized bone graft. The patient was allowed to walk bearing full weight immediately after surgery. We measured the height of the hip center from the teardrop line and the pelvic height on anteroposterior roentgenograms of the pelvis and calculated the ratio of the hip center to the pelvic height. We defined the anatomical hip center as the height of the center less than 15 % of the pelvic height, which was nearly equal to 30 mm, because the mean pelvic height was 210 mm. Results. The mean height of the hip center was 9.8 (4.1∼18.0) % of the pelvic height and the 101 (96.2%) cups were placed within the anatomical hip center. Radiographically, in all patients, the host-graft interface became distinct and the new cortical bone in the lateral part of the plate bone appeared within 1 year after surgery (Fig.2, 3). We observed no absorption of the plate bone graft and no migration of the cup at the last follow-up. Conclusion. CBG technique is simple, because the bone graft is always performed after the pressfit of the cup is achieved. Moreover, patients require no partial weight bearing postoperatively, because the cup is supported by the host bone with the pressfit and additional screws. The CBG technique would be an excellent option for the reconstruction of the acetabulum in patients with severe dysplasia to avoid a high hip center and bulky bone grafting

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

Introduction and Aims: Dysplasia of the femoral sulcus is one component that may be present in patients presenting with patellar instability. Rarely the dysplasia is so severe that a dome rather than groove is present. Dejour has reported an operation that deepens the groove, unlike the Albee which elevates it. This study reports the development of a modification of the Dejour trochleoplasty reporting the clinical and radiological results. Method: A consecutive case series of the first 15 patients (17 knees) who underwent a trochleoplasty with a minimum one-year follow-up. There were 11 females and four males with an age range from 15 to 47 years old. Nine patients had had previous operations. The pre-operative length of symptoms ranged from one to 30 years. The patellar instability was managed operatively using the Dejour protocol measuring the patellar height, boss height, tibial tubercle-trochlear groove distance and patellar tilt angle. The Kujala score was used for functional assessment and a subjective assessment was also made. CT scans as well as plain films were used for radiological assessment. The operative technique changed in the light of experience from metallic to absorbable screws as the former abraded the patella. Results: The boss height was reduced from an average of 7.5mm to 1mm (normal 0mm). Tracking became normal in 11 knees and had a slight J-shape in six. Seven knees had a mild residual apprehension. Five patients were very satisfied, eight were satisfied, and two were disappointed. The Kujala score improved from an average of 48 to 73 out of 100. Three patients returned to full sports. Eight patients required further operations apart from the removal of metallic screws in 10 knees. Five of these were arthrolysis for stiffness at about six weeks post-operatively. As a result, patients were placed on a continuous passive motion machine for three to four days to avoid this. One patient went on to have autologous chondrocyte implantation for a defect on the lateral femoral condyle involving the tibial surface. This was after a new injury following a return to sports and not in the area affected by the trochleoplasty. Conclusion: Trochleoplasty for severe dysplasia of the femoral sulcus is a developing procedure. It requires careful attention to detail. For a rare condition the results have been gratifying with an acceptable level of complications. It is not recommended that this procedure be performed by general orthopaedic surgeons

Introduction: Total knee arthroplasty (TKA) in patients with skeletal dysplasia is challenging due to the anatomic variances and deformities. The purpose of this review is to understand the technical issues involved in treating these patients. Methods: Clinical notes, operative reports, and radiographs were retrospectively reviewed of 12 knees in 8 patients: 3 achondroplasia patients (one with bilateral 10° varus deformities, one with a 30° varus deformity in one knee and 25° varus deformity in the other knee, one with a 14° varus deformity); 3 multiple hereditary exostosis patients (one with bilateral 45° valgus deformities, one with a 45° valgus deformity in one knee and 15° valgus deformity in the other, one with a 11° valgus deformity); and 2 osteogenesis imperfecta patients (one with a 25° varus deformity, one with a 17° valgus deformity). Results: Surgical exposure required preoperative placement of soft-tissue expanders to avoid wound complications (1 knee), quadriceps snip (2 knees), and hardware removal (1 knee). Intraoperative balancing of the knee was more complex requiring a lateral epicondylar osteotomy (3 knees), medial release (6 knees), lateral retinacular release (6 knees), and proximal realignment to improve patellar tracking (1 knee). 5 knees required a constrained insert, 2 required tibial augments, one required use of cement and screw technique, and one required modification of an all-polyethylene tibia to accommodate the deformed tibial anatomy. 2 knees required custom tibial components. Complications included 2 peroneal nerve palsies which resolved 3 months postoperatively. Range of motion preoperatively averaged 103° (range 45 to 130°) and 100° postoperatively (range 85 to 120°). All patients were pain-free at their last followup (average follow-up 3.9 years). Conclusion: Special considerations must be made regarding surgical exposure, ligament balancing, implant selection, and anticipation of complications due to the unusual deformities when performing TKA in skeletal dysplasia patients

Background: Hip dysplasia is a complex developmental process. Untreated acetabular dysplasia is the most common cause of secondary hip osteoarthiritis. With increased interest in redirectional pelvic osteotomies, the role of the shelf procedure needs to be re-defined. Aim of the study: to investigate the effectiveness of the shelf procedure in adults with symptomatic acetabular dysplasia by assessing the functional and radiological outcome at a minimum of five years follow-up. Material and Methods: Seventy-six consecutive adults with symptomatic acetabular dysplasia treated with acetabular shelf augmentation, have been followed up for an average period of 11 years (range: 6–14). The mean age was thirty-three years (range: 17–60 years). The Oxford hip score (OHS) was used for clinical assessment. Centre-edge angle (CEA) and acetabular angle (AA) were measured to determine femoral head coverage. Osteoarthiritis severity was based primarily on the width of the joint space using the De Mourgues classification. Survivorship analyses using conversion to THR as an endpoint were performed. logrank test was used to compare the outcome of the shelf against the variables of age, preoperative osteoarthiritis, preoperative and postoperative AA, CEA angles. Results: The shelf procedure improved the mean preoperative CEA from 11° (range: 20° to 17°) to 50° postoperatively (range: 30° to 70°) and the mean preoperative AA from 52° (range: 46° to 64°) to 32° postoperatively (range: 18° to 57°). The Mean OHS was 34.6 (hip score maximum: 48). Thirty percent of hips needed THR at an average duration of 7.3 years. Survival analysis using conversion to THR as an endpoint was 86% (CI, 76%–95%) at five years and 46% (CI, 27%–65%) at ten years. The survival in the 44 patients with only slight or no joint space narrowing was 97% (CI, 93%–100%) at 5 years and 75% (CI, 51%–100%) at 10 years. This was significantly higher (p= 0.0007) than the survival in the 32 patients with moderate or severe osteoarthiritis, which was 76% (CI, 55%–89%) at 5 years and 22% (CI, 5%–38%) at 10 years. There was no significant relationship between survival and age (p= 0.37), pre and postoperative centre-edge angle (p= 0.39), or acetabular angle (p= 0.85). Conclusion: 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 slight or no joint space narrowing

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

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 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

Introduction. Dysplastic hip has not only deficiency of acetabulum but also femoral deformity. Therefore, selection of stem is important in cementless THA for dysplasia. Especially using of short stem should be challenge for deformed femur. We studied clinical performance and radiological findings after THA with triple tapered short stem (Optimys hip) for dysplastic hip. Materials and method. From May 2013, we performed cementless THA for osteoarthritis with dysplasia. Seventy-one hips of 67 patients were examined including four males and 63 females. Age at surgery was from 36 to 88 years old (61.7 in average). Surgical approach was used modified Watson-Jones in all hips. Clinical evaluation was used modified Harris hip score (MHSS), incidence of complications and thig pain. Radiological findings were evaluated according Engh's classification and grade of stress shielding. Follow-up period was minimum one year and was 21.3 months in average. Results. MHHS before surgery was 41 points and that at follow-up was 88 points in average and pain score was significantly improved. Calcar fracture during surgery occurred in two hips and circular wiring was performed. Deep infection, DVT and dislocation were not observed. Incidence of thigh pain was 1.4% of one hip. Radiological findings were assessed as bone in-grown in all of the hips and grade of stress-shielding was very low. There were no aseptic loosening and no revision for any reasons. Conclusions. Cementless THA with triple tapered short stem was useful for treatment of dysplastic hips. Radiological findings of all hip were assessed as bone in-grown

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

Introduction. Bulk bone grafting of the cup is commonly used in total hip arthroplasty (THA) for developmental dysplasia. However, it carries a risk of the graft collapse in the mid-term or long-term results. The purpose of this study is to describe our new bulk bone grafting technique and review the radiographic and clinical results. Patients and Methods. We retrospectively reviewed 85 hips in 74 patients who had undergone bulk bone grafting in total hip arthroplasty for developmental dysplasia between 2008 and 2013. We excluded patients who had any previous surgeries or performed THA with the femoral shortening osteotomy. According to the Crowe classification, 4 hips were classified as Type 1, 28 as Type 2, 35 as Type 3, and 18 as Type 4. Follow-up was at a mean of 4.0 years (1 to 6.1). The surgery was performed using the direct anterior approach on a standard surgical table. The acetabulum was reamed for as close to the original acetabulum as possible. The pressfit cementless cup was impacted into the original acetabulum. After the pressfit fixation of the cup was achieved, two or three screws were used to reinforce the fixation. The superior defect of the acetabulum was packed with sufficient amount of morselized bone graft. Then, the bulk bone was placed on the morselized bone graft and fixed with one screw. Post-operatively, there were no restrictions to movement or position. On the first day after surgery, the patient was allowed to walk with full weight-bearing. We measured the height of the hip center from the interteardrop line and the pelvic height on anteroposterior roentgenograms of the pelvis and calculated the ratio of the hip center to the pelvic height. We defined the anatomical hip center as the height of the center less than 15% of the pelvic height. Results. The mean height of the hip center was 10.2 (4.1∼18.0)% of the pelvic height and the 81 (95.2%) cups were placed within the anatomical hip center. We observed no collapsed grafts, no severe absorption of the grafts, and no migration of the cup at the last follow-up. Conclusion. In our technique, there is no concern of the bulk bone graft collapse even in the long-term results, because the cup is not supported by the bulk bone graft but by the host bone with the pressfit and additional screws. Moreover, 95.2% of all cups were placed within the anatomical hip center. In conclusion, our new bulk bone graft technique would be simple to perform and an excellent option for the reconstruction of the acetabulum in patients with severe dysplasia

A number of measurements of patellar height are in clinical use all of which reference from the tibia. The patellotrochlear index has been proposed recently as a more accurate reflection of the functional height of the patella and described in normal knees. We compared patellar height measurements in patients with patellofemoral dysplasia. In a retrospective analysis of the MRI scans of 33 knees in 29 patients with patellofemoral dysplasia we assessed the inter- and intraobserver reliability of four patellar height measurements: the recently described Patellotrochlear Index (PTI), Insall-Salvati (IS), Blackburne-Peel (BP) and Caton-Deschamps (CD) ratios. We also assessed the correlation between the different measurements in predicting patella alta. Three blinded observers on two separate occasions performed the measurements. There were 21 females and 8 males with a mean age of 21.4 years (13–33). Statistical analysis revealed good inter-observer reliability for all measurements (0.78 for PTI, 0.78 for IS, 0.73 for BP and 0.77 for CD). Intra-observer reliability was also good (0.80, 0.83, 0.75, 0.78 respectively). There was weak correlation between the PTI and the other ratios for patella alta. There was a strong correlation between the CD and BP ratios (0.96) and a moderate correlation between IS and CD and IS and BP ratios (0.594 and 0.539 respectively). We propose the PTI as a more clinically relevant measure

Management of symptomatic residual acetabular dysplasia in adolescence and early adulthood remains a major therapeutic challenge. At our unit the two senior authors review all patients preoperatively and simultaneously perform each procedure. In the four years from 1998 forty-three Bernese osteotomies were performed in 40 patients with residual acetabular dysplasia. The mean average age at surgery was 21 years (range 12 – 43 years) and there were 34 female patients. The indication for surgery was symptomatic hip dysplasia (all idiopathic but for one male with a history of slipped capital femoral epiphysis) presenting with pain and restricted ambulation. 4 patients had previous surgery on the affected hip (2 Salter’s osteotomy, one Shelf procedure and one proximal femoral osteotomy). 27.5% of patients had symptomatic bilateral disease. 42% of patients had Severin class IV or V dysplasia at presentation. 100% of patients had preservation of the hip joint at last follow-up evaluation (mean 2.4 years), with excellent results in 82%, an average post-operative Harris hip score of 96, and an average d’Aubigne hip score of 16.1. The mean post-operative improvements in radiographic measures were as follows: Anterior centre edge angle +19.4°, Lateral centre angle +25.8°, Acetabular Index – 10.7°. Head to Ischial distance – 7.3mm. Surgical operative time decreased from 128 minutes to 43 minutes from the first to the most recent case. Average blood loss has reduced from 1850mls to 420mls over the four years experience. Predonation of 2 units of blood requested from all patients with baseline hemoglobin of > 12g/dl. When combined with intraopera-tive cell salvage the need for transfusion of homologous blood has been eliminated. All complications occurred in the first 9 patients: (one major – iliac vein injury requiring no further treatment; four moderate – lateral cutaneous nerve injuries; four minor – asymptomatic heterotopic ossification). Our experience confirms that the Ganz periacetabular osteotomy is an efficacious procedure for the treatment of the residually dysplastic hip, providing excellent clinical results, where early intervention is the key to improved outcome. It is a technically demanding procedure with a significant early learning curve and we believe that a two

Background. Fibrous dysplasia is a developmental anomaly of bone formation that may exist in a monostotic or polystotic form. Surgical treatment is considered advisable only with presence of significant or progressive deformity or persistent pain. Early surgery is indicated before the tumor expands or fracture occurs. Methods. We reviewed a series of 21 patients, 14 had monostotic whereas 7 had polystotic fibrous dysplasia. There was no case of Mc Cune Albright. We treated all of these patients with curettage and corticocancellous bone graft and also fixation with reconstruction nails. Follow up ranged between 1 and 5 years. Functional and radiographic outcomes were scored. Results. Russel Taylor IM nail and Gamma nail were used in 11 and 10 patients, respectively. Their mean age at the time of diagnosis was 28 years for monostotic for of the disease and 20 years for polystotic ones. Postoperatively, All patients had good bone healing and complete incorporation of the implanted graft, although it last longer in the case of corrective osteotomy for severe varus. Using of Gamma nail was easier for us in addition to shorter operation time. Up to now, no case of recurrency or pathologic fracture has been seen in our patients. Chronic hip pain was the most common problem in these patients but they reported no restriction of activity of daily living. Conclusion. Clinical results of reconstruction nails were safe and satisfactory in patients with fibrous dysplasia of proximal femour

Introduction: Trochlear dysplasia is an important anatomical factor in symptomatic patellar instability. The impression at surgery is that the dysplasia is predominantly an abnormality of the proximal trochlea. There is a mismatch between the bony and cartilaginous trochlear anatomy in normal knees. This study assessed the mismatch in dysplastic trochleas. Methods: The MRI scans of 25 knees in 23 patients with patellar instability and trochlear dysplasia were reviewed retrospectively. Axial fat saturation images where used to assess cartilaginous and bony trochlear morphology. The following parameters were measured in the three most proximal images of each trochlea:. Cartilaginous and bony sulcus angles. Cartilage thickness in the sulcus and on both condyles. Morphology of the bony and cartilaginous surface contour (flat, convex or concave). Horizontal distance of the bony and cartilaginous sulcus measured from the lateral epicondyle. Inter and intra observer error was recorded. Results: There were 15 females and 8 males with an average age of 20.4 years (14 30). The average bony sulcus angle (SA) was 166.9 degrees (141 – 203) whereas the average cartilaginous SA was 184.8 degrees (152 – 222). In 74 of 75 axial images (98.6%) the cartilaginous SA was greater than the bony SA by an average of 17.6 degrees (3 45). The average cartilage thickness in the centre of the trochlea was 3.5 mm (1.4 – 5.2), and the thickness on the lateral and medial condyles were 2.2mm (1.8 3.5) and 1.9mm (0.6 3.3) respectively. In 74 of 75 axial images (98.6%) the cartilaginous contour was different to the osseous contour on subjective assessment. In 58 of 75 images (77.3%) the cartilaginous contour changed the bony morphology from concave to flat or convex, or from flat to convex. In 17 (22.6%) it reduced the concavity. The average horizontal distance of the bony sulcus from the lateral epicondyle was 33.7mm (24 42) and that of the cartilaginous sulcus 31.8mm (23 39). Conclusion: This study demonstrates that the cartilaginous trochlear morphology differs markedly from that of the underlying bony trochlea in patients with trochlear dysplasia. Although the bony trochlea was dysplastic, the cartilage morphology worsened this abnormal shape. Since cartilage is poorly represented on routine radiography and computed tomography, MRI is necessary to demonstrate both the bony and cartilaginous morphology to facilitate surgical planning

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

Introduction: Persistent acetabular dysplasia is a recognized cause of premature hip arthritis. Treatment options include joint preservation (acetabuloplasty/osteotomy) or salvage procedures (THR). Presence of a deficient acetabulum and an elevated acetabular centre make THR technically demanding with uncertain outcome. Shelf ace-tabuloplasty is a viable option, however, most reports in the literature focus on results in children and adolescents. Aim: To investigate the functional and radiological outcome of shelf acetabuloplasty in adults with significantly symptomatic acetabular dysplasia. Material and Methods: 77 consecutive shelf procedures (68 patients) with an average follow-up of 10.9 years (range: 6–17) were reviewed. The Oxford hip score (OHS) was used for clinical assessment. Centre-edge angle (CEA) and acetabular angle (AA) were measured as indicators of joint containment. Results: The average age at surgery was 33 years (range: 17–60). At the time of last follow-up; the mean OHS was 34 (maximum score: 48). Mean postoperative CEA was 59 (Pre-operatively: 16.2 degrees) while mean postoperative AA was 31 (Pre-operatively: 47.5 degrees). Thirty percent of hips needed THR at an average duration of 7.3 years. Pre-operative arthritis was present in 32 hips out of which 17 (53 percent) needed THR. Out of the remaining 45 hips, only 6 (13 percent) needed THR. No correlation was found between the acetabular indices and the outcome. Conclusion: Shelf-acetabuloplasty offers symptomatic relief to adults with acetabular dysplasia and can delay the need for THR for over 10 years. Best results with shelf-acetabuloplasty were achieved in patients without preoperative arthritis

Fibrous dysplasia is a genetic not hereditary disease of the skeleton, most frequently located at the femoral bone. The extension and the radiographic morphology of the femoral lesion are variable, and the prognosis is unpredictable. The purpose of this study is to propose a radiographic classification of the femoral lesion, related to the prognosis and the natural history of the disease. The authors reviewed the radiographic examinations of 25 patients affected by fibrous dysplasia of bone with femoral involvement, who were followed-up at least two years after diagnosis. Eleven patients had bilateral localization of the disease, for a total of 36 affected femurs. A radiographic classification of the femoral lesion was proposed based on the localization, the extension and the type of the lesion. The authors classify the femoral lesions as lytic, lytic with surrounding sclerosis, lytic and sclerotic, sclerotic and complex. They observed a prevalence of the lytic lesions and involvement of the proximal part of the femur. Many lesions (40%) expanded with age, and the sclerotic component also increased. The lytic lesions worsened with age, causing severe deformity of the femur. However the mostly sclerotic lesions turned out to be stable at follow-up. The worsening of the lesion was not related to the sex of the subjects; 77% of the worsened lesions were observed in patients younger than 20 years of age, while 66% of the stable lesions were found in patients older than 20 years. In conclusion, this study demonstrated that the prognosis of the femoral lesion in fibrous dysplasia is related to the type of lesion, as per the radiographic classification here in described. Prognosis is also related to the age of the patient and the presence of a sclerotic component, which suggested a greater stability of the lesion

Introduction. Secondary osteoarthritis in a dysplastic hip is a surgical challenge. Severe leg length discrepancies and torsional deformities add to the problem of inadequate bony support available for the socket. Furthermore, many of these patients are young and wish to remain active, thereby jeopardising the long-term survival of any arthroplasty device. For such severely dysplastic hips, the Birmingham Hip Resurfacing (BHR) device provides the option of a dysplasia component, a hydroxyapatite-coated porous uncemented socket with two lugs to engage neutralisation screws for supplementary fixation into the solid bone of the ilium more medially. The gap between the superolateral surface of the socket component and the false acetabulum is filled with impacted bone graft. Methods and results. One hundred and thirteen consecutive dysplasia BHRs performed by the senior author (DJWM) for the treatment of severely arthritic hips with Crowe grade II and III dysplasia between 1997 and 2000 have been reviewed at a minimum five year follow-up. There were 106 patients (59M and 47F). Eighty of the 113 hips were old CDH or DDH, 29 were destructive primary or secondary arthritis with wandering acetabulae and four were old fracture dislocations of the hip. Mean age at operation was 47.5 years (range 21 to 68 years – thirty-six men and forty-four women were below the age of 55 years). There were two failures (1.8%) out of the 113 hips at a mean follow-up of 6.5 years (range 5 to 8.3 years). One hip failed with a femoral neck fracture nine days after the operation and another failed due to deep infection at 3.3 years. Conclusion. The dysplasia resurfacing device offers a good conservative arthroplasty option for these severely deficient hips

Although fibrous dysplasia is a benign bone disease, in few cases patient are suffering from severe pain of the skeletal system. The aim of this study was to evaluate the current state regarding pain of patients with fibrous dysplasia treated at our hospital. We searched our digital database since 1990 for patients with fibrous dysplasia. Subsequent we verified the histological diagnosis by reviewing the final pathologic report. Additional we called the identified patients by phone to make an enquiry about their pain course and associated treatment. For rating pain intensity we used a numeric rating scale with a range within zero to ten. We identified 43 patients (21 male, 22 female) with an average age at initial diagnosis of 40 years (range 10 to 72years). The mean follow up was 6 years (range 1 to 23 years). Among these 43 patients we were able to contact 33 by phone. Initial diagnosis was made due to pain in 23 cases, nearly coequal by coincidental examination in 20 cases, for fracture in two cases and for local swelling and bone deformity each time in two cases. Thirty-six patients revealed monostotic and seven patients polyostotic involvement. The following locations were found: three times craniofacial, four times within the spine, eight times at the upper extremity, ten times in the pelvis and 31 times at the lower limb. Two patients were suffering additionally from Mazabraud Syndrome. Actual values at the numeric rating scale regarding pain ranged from 0 to 9 with a mean value of 1. Specific in the polyostotic group we found an average value of 3 and three of seven patients stated a value greater than 5 for persistent pain. Five patients with polyostotic involvement were treated with bisphosphonat for pain control with good response. It is remarkable that patients with polyostotic involvement have marked higher values for pain intensity at the numeric rating scale. So therefore we should have a closer look for potential reasons explaining that fact. In accordance with previous published studies we found that pain decreased by intermittent intravenous application of bisphosphonates

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

Introduction: The purpose was to present a new osteotomy technique (trochleoplasty) and its preliminary results for the treatment of femoral trochlear dysplasia with recurrent patellar dislocation. Methods: Between 1990 and 2002, 59 knees of 51 patients (mean age 224 years) with recurrent patellar dislocation due to femoral trochlear dysplasia were treated uniformly at a single institution with a new osteotomy technique developed by the senior author. A distally connected osteochondral flake is released from the dysplastic trochlea and refixed after the osseous trochlear groove has been reconstructed. 44 patients with 50 involved knees returned at a mean follow-up of 37 months (range 6 to 139) for a physical examination, assessment of knee pain and function, radiographic examination of the knee, and in selectived cases for CT scan, MR imaging and follow-up knee arthroscopy. Results: Postoperative complications were limited to hemarthros-1, arthrofibrosis-1, and sudeck‘s disease-1. Postoperatively, no further patellar dislocations were reported. All patients experienced a sensation of significantly improved knee stability resulting in higher levels of activity. Retropatellar pain as found in 34 knees preoperatively was better-24, unchanged-7, worse-6 (3 additional cases) after surgery. Positive apprehension sign, as preoperatively found in all patients, turned negative in all cases. Radiographically, osseous healing of the reconstructed trochlea was noted without evidence of subsequent arthrosis. MRI and knee arthroscopy including histological analysis of osteochondral biopsies did not provide any evidence for osteonecrosis or chondropathia. Conclusion: Recurrent patellar dislocation due to femoral trochlear dysplasia can be treated successfully using the presented technique of trochleoplasty

Aim: To systematically review the present evidence-base, assessing the clinical and radiological outcomes following trochleoplasty for trochlear dysplasia. Methods: An electronic literature search was performed using the AMED, British Nursing Index, CINAHL, Cochrane, EMBASE, ovid Medline, Physiotherapy Evidence Database, PsycINFO, Pubmed and Zetoc databases from their inception to August 2007. Reference lists of identified papers and a hand search of specialist knee journals was also undertaken. All English language, human subject clinical studies, detailing the clinical and/or radiological outcomes of patellar instability patients following a trochleoplasty were included. Two independent reviewers appraised each paper using the CASP tool. Results: Six papers comprising of 117 trochleoplasty procedures on 99 patients were reviewed. Clinically and radiologically, trochleoplasty was shown to be a safe and effective procedure to correct patellar instability in trochlear dysplasia patients. However, the CASP tool identified that the evidence-base reviewed presented with a number of methodological limitations. These included: limited use of inferential statistics; not applying reliable outcome measurements; poor description of patient recruitment; and evaluating small samples. Conclusion: Although trochleoplasty may be an effective procedure to correct patellar instability in trochlear dysplasia patients, further study is recommended to assess longer-term outcomes using well-designed studies

Purpose of study: Glenoid dysplasia occurs early in the shoulders of some children affected by obstetric brachial plexus palsy (OBPP). Prompt treatment can reverse the deformity. A program has been devised to examine these children’s shoulders by ultrasound and the early results are described. Method: Since March 2006, all neonates born in New-castle upon Tyne with a diagnosis of OBPP have been referred to the hip ultrasound clinic. The shoulders were examined clinically for range of movement and signs of instability. A static and dynamic ultrasound examination was then performed. Treatment of subluxed shoulders involved splinting the shoulder in adduction and external rotation for six weeks after injection of the internal rotator muscles with botulinum toxin, as recommended by Ezaki and co-workers. Results: To date, six infants have been screened. Two had significant instability with ultrasonographic evidence of early glenoid dysplasia and have been treated. One had mild glenoid dysplasia with restricted external rotation which improved with physiotherapy alone. Three were clinically and ultrasonographically normal. Conclusions: The early experience of this program confirms the high incidence of shoulder dysplasia in the neonatal period in these children, as reported by others. The examination is safe and relatively easy. In the early stages of the condition the treatment to date has been simple and effective. We plan to continue with ultrasound screening for shoulder dysplasia in neonates with OBPP

The aim was to study the use of limb reconstruction techniques in the management of Ollier’s Dysplasia over a period of 25 years. This was a retrospective review of case records and radiographs of patients who had lower limb reconstruction for deformity and limb length discrepancy. There were a total of 9 patients of whom 7 had reached maturity and four of these were still under follow up. The major aims of surgery were to correct lower limb length discrepancy and deformity. A total of twenty segments were operated upon. These were 11 femurs and 9 tibiae. In some segments repeated surgery was required. 41 index and 54 secondary procedures were necessary giving an average of 10.5 procedures per patient. The most common problems were difficulty in fixation in abnormal bone, premature consolidation reflecting the rich osteogenic potential and growth related recurrence of deformities and discrepancy. The mean length gained was 13.8 cms per patient. Healing of regenerate occurred with radiologically normal appearance even in chondro-dysplastic areas. All patients who had completed treatment had a satisfactory mechanical axis and the mean length discrepancy was 1.7 cms. Patients with Ollier’s dysplasia appear to respond well to limb reconstructive surgery. It is possible to correct severe limb length discrepancies and angular deformities. Surgeons should be aware of the possibility of premature healing and should consider faster lengthening rates of up to 1.5 mms per day. Distraction should begin early by day 5 or less. Immature patients should be warned about the possibility of recurrence of deformity and possible need for repeated surgery

For the treatment of the acetabular dysplasia in the early childhood the spherical periacetabular osteotomy of the ilium is a well-recognized procedure. 52 hips of 35 patients treated with a periacetabular osteotomy between 1969 and 1985 we followed-up after 14 to 31 years. At the time of operation the average age was 2,8 years. The measurement of the anterio-posterior radiographs showed a normalization of the acetabular roof obliquity in 79% of the cases. However, there were only 46% of the cases with a normal center-edge angle. 42% had a medium pathologic angle of 20 to 30 °. In 12 % we saw severe pathology with angles smaller than 20. Fife cases (10%) presented radiologic signs of osteoarthritis and three a slight incongruence between head and acetabulum. Rare cases of postoperative complications are presented. The low incidence of radiographic osteoarthritis supports the view that the spherical periacetabular osteotomy is an appropriate surgical procedure to treat acetabular dysplasia in early childhood

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