Aims. The aim of this study was to evaluate the epidemiology and treatment of Perthes’ disease of the hip. Methods. This was an anonymized comprehensive cohort study of Perthes’ disease, with a nested consented cohort. A total of 143 of 144 hospitals treating children’s hip disease in the UK participated over an 18-month period. Cases were cross-checked using a secondary independent reporting network of trainee surgeons to minimize those missing. Clinician-reported outcomes were collected until two years. Patient-reported outcome measures (PROMs) were collected for a subset of participants. Results. Overall, 371 children (396 hips) were newly affected by Perthes’ disease arising from 63 hospitals, with a median of two patients (interquartile range 1.0 to 5.5) per hospital. The annual incidence was 2.48 patients (95% confidence interval (CI) 2.20 to 2.76) per 100,000 zero- to 14-year-olds. Of these, 117 hips (36.4%) were treated surgically. There was considerable variation in the treatment strategy, and an optimized decision tree identified joint stiffness and age above eight years as the key determinants for containment surgery. A total of 348 hips (88.5%) had outcomes to two years, of which 227 were in the late reossification stage for which a hip shape outcome (Stulberg grade) was assigned. The independent predictors of a poorer radiological outcome were female sex (odds ratio (OR) 2.27 (95% CI 1.19 to 4.35)), age above six years (OR 2.62 (95% CI (1.30 to 5.28)), and over 50% radiological collapse at inclusion (OR 2.19 (95% CI 0.99 to 4.83)). Surgery had no effect on radiological outcomes (OR 1.03 (95% CI 0.55 to 1.96)). PROMs indicated the marked effect of the disease on the child, which persisted at two years. Conclusion. Despite the frequency of containment surgery, we found no evidence of improved outcomes. There appears to be a sufficient case volume and community equipoise among surgeons to embark on a randomized clinical trial to definitively investigate the effectiveness of containment surgery. Cite this article: Bone Joint J 2022;104-B(4):510–518

Aims. Perthes’ disease is an uncommon hip disorder with limited data on the long-term outcomes in adulthood. We partnered with community-based foundations and utilized web-based survey methodology to develop the Adult Perthes Survey, which includes demographics, childhood and adult Perthes’ disease history, the University of California Los Angeles (UCLA) Activity Scale item, Short Form-36, the Hip disability and Osteoarthritis Outcome Score, and a body pain diagram. Here we investigate the following questions: 1) what is the feasibility of obtaining > 1,000 survey responses from adults who had Perthes’ disease using a web-based platform?; and 2) what are the baseline characteristics and demographic composition of our sample?. Methods. The survey link was available publicly for 15 months and advertised among support groups. Of 1,505 participants who attempted the Adult Perthes survey, 1,182 completed it with a median timeframe of 11 minutes (IQR 8.633 to 14.72). Participants who dropped out were similar to those who completed the survey on several fixed variables. Participants represented 45 countries including the USA (n = 570; 48%), UK (n = 295; 25%), Australia (n = 133; 11%), and Canada (n = 46; 4%). Of the 1,182 respondents, 58% were female and the mean age was 39 years (SD 12.6). Results. Ages at onset of Perthes’ disease were < six years (n = 512; 43%), six to seven years (n = 321; 27%), eight to 11 years (n = 261; 22%), and > 11 years (n = 76; 6%), similar to the known age distribution of Perthes’ disease. During childhood, 40% (n = 476) of respondents had at least one surgery. Bracing, weightbearing restriction, and absence of any treatment varied significantly between USA and non-USA respondents (p < 0.001, p = 0.002, and p < 0.001, respectively). As adults, 22% (n = 261) had at least one total hip arthroplasty, and 30% (n = 347) had any type of surgery; both more commonly reported among women (p = 0.002). Conclusion. While there are limitations due to self-sampling, our study shows the feasibility of obtaining a large set of patient-reported data from adults who had childhood Perthes’ from multiple countries. Cite this article: Bone Jt Open 2022;3(5):404–414

Aims. Avascular femoral head necrosis in the context of gymnastics is a rare but serious complication, appearing similar to Perthes’ disease but occurring later during adolescence. Based on 3D CT animations, we propose repetitive impact between the main supplying vessels on the posterolateral femoral neck and the posterior acetabular wall in hyperextension and external rotation as a possible cause of direct vascular damage, and subsequent femoral head necrosis in three adolescent female gymnasts we are reporting on. Methods. Outcome of hip-preserving head reduction osteotomy combined with periacetabular osteotomy was good in one and moderate in the other up to three years after surgery; based on the pronounced hip destruction, the third received initially a total hip arthroplasty. Results. The described pathology is quite devastating, and extensive joint preserving surgery (which has been shown successful in Perthes’ cases) was less successful in this patient cohort. Conclusion. Supraselective angiography may be helpful to improve pathomechanical understanding and surgical decision making. Cite this article: Bone Jt Open 2022;3(9):666–673

Aims. The aim of this study was to produce clinical consensus recommendations about the non-surgical treatment of children with Perthes’ disease. The recommendations are intended to support clinical practice in a condition for which there is no robust evidence to guide optimal care. Methods. A two-round, modified Delphi study was conducted online. An advisory group of children’s orthopaedic specialists consisting of physiotherapists, surgeons, and clinical nurse specialists designed a survey. In the first round, participants also had the opportunity to suggest new statements. The survey included statements related to ‘Exercises’, ‘Physical activity’, ‘Education/information sharing’, ‘Input from other services’, and ‘Monitoring assessments’. The survey was shared with clinicians who regularly treat children with Perthes’ disease in the UK using clinically relevant specialist groups and social media. A predetermined threshold of ≥ 75% for consensus was used for recommendation, with a threshold of between 70% and 75% being considered as ‘points to consider’. Results. A total of 40 participants took part in the first round, of whom 31 completed the second round. A total of 87 statements were generated by the advisory group and included in the first round, at the end of which 31 achieved consensus and were removed from the survey, and an additional four statements were generated. A total of 60 statements were included in the second round and 45 achieved the threshold for consensus from both rounds, with three achieving the threshold for ‘points to consider’. The recommendations predominantly included self-management, particularly relating to advice about exercise and education for children with Perthes’ disease and their families. Conclusion. Children’s orthopaedic specialists have reached consensus on recommendations for non-surgical treatment in Perthes’ disease. These statements will support decisions made in clinical practice and act as a foundation to support clinicians in the absence of robust evidence. The dissemination of these findings and the best way of delivering this care needs careful consideration, which we will continue to explore. Cite this article: Bone Joint J 2024;106-B(5):501–507

Aims. Perthes’ disease is an idiopathic avascular necrosis of the developing femoral head, often causing deformity that impairs physical function. Current treatments aim to optimize the joint reaction force across the hip by enhancing congruency between the acetabulum and femoral head. Despite a century of research, there is no consensus regarding the optimal treatment. The aim of this study was to describe the experiences of children, their families, and clinicians when considering the treatment of Perthes’ disease. Methods. A qualitative study gathered information from children and their families affected by Perthes’ disease, along with treating clinicians. Interviews followed a coding framework, with the interview schedule informed by behavioural theory and patient and public involvement. Transcripts were analyzed using the framework method. Results. A total of 24 interviews took place, with 12 child/family dyads and 12 clinicians from UK NHS centres. Interviews identified widespread variation of routine care. Children/their families recounted positive experiences when included in the decision-making process for treatment. There is a strong desire from clinicians and children/families for consistent guidance from everyone involved in care, which should be based on clinical consensus. Conclusion. This is the first study to describe how children/families and clinicians experienced receiving or providing treatment in Perthes’ disease. The results indicate the need for robust evidence to support treatment decisions. Children and families valued feeling involved in the clinical decision-making process. Clinicians acknowledged the central importance of providing patient-centred care, particularly in the absence of robust evidence to guide the optimal treatment decisions. This study will inform a future Delphi project to develop clinical consensus guidelines for the treatment of Perthes’ disease. Cite this article: Bone Jt Open 2023;4(10):735–741

Aims. This study aimed to evaluate the relationship between hip shape and mid-term function in Perthes’ disease. It also explored whether the modified three-group Stulberg classification can offer similar prognostic information to the five-group system. Methods. A total of 136 individuals aged 12 years or older who had Perthes’ disease in childhood completed the Patient-Reported Outcomes Measurement Information System (PROMIS) Mobility score (function), Nonarthritic Hip Score (NAHS) (function), EuroQol five-dimension five-level questionnaire (EQ-5D-5L) score (quality of life), and the numeric rating scale for pain (NRS). The Stulberg class of the participants’ hip radiographs were evaluated by three fellowship-trained paediatric orthopaedic surgeons. Hip shape and Stulberg class were compared to PROM scores. Results. A spherical hip was associated with the highest function and quality of life, and lowest pain. Conversely, aspherical hips exhibited the lowest functional scores and highest pain. The association between worsening Stulberg class (i.e. greater deviation from sphericity) and worse outcome persisted after adjustment for age and sex in relation to PROMIS (predicted mean difference -1.77 (95% confidence interval (CI) -2.70 to -0.83)), NAHS (-5.68 (95% CI -8.45 to -2.90)), and NRS (0.61 (95% CI 0.14 to 1.08)), but not EQ-5D-5L (-0.03 (95% CI -0.72 to 0.11)). Conclusion. Patient-reported outcomes identify lower function, quality of life, and higher pain in aspherical hips. The magnitude of symptoms deteriorated with time. Hip sphericity (i.e. the modified three-group classification of spherical, oval, and aspherical) appeared to offer similar levels of detail to the five-group Stulberg classification. Cite this article: Bone Joint J 2023;105-B(6):711–716

Aims. Perthes’ disease is a condition which leads to necrosis of the femoral head. It is most commonly reported in children aged four to nine years, with recent statistics suggesting it affects around five per 100,000 children in the UK. Current treatment for the condition aims to maintain the best possible environment for the disease process to run its natural course. Management typically includes physiotherapy with or without surgical intervention. Physiotherapy intervention often will include strengthening/stretching programmes, exercise/activity advice, and, in some centres, will include intervention, such as hydrotherapy. There is significant variation in care with no consensus on which treatment option is best. The importance of work in this area has been demonstrated by the British Society for Children’s Orthopaedic Surgery through the James Lind Alliance’s prioritization of work to determine/identify surgical versus non-surgical management of Perthes’ disease. It was identified as the fourth-highest priority for paediatric lower limb surgery research in 2018. Methods. Five UK NHS centres, including those from the NEWS (North, East, West and South Yorkshire) orthopaedic group, contributed to this case review, with each entre providing clinical data from a minimum of five children. Information regarding both orthopaedic and physiotherapeutic management over a two-year post-diagnosis period was reviewed. Results. Data were extracted from the clinical records of 32 children diagnosed with Perthes’ disease; seven boys and 25 girls. The mean age of the children at diagnosis was 6.16 years (standard deviation (SD) 3.001). In all, 26 children were referred for physiotherapy. In the two-year period following diagnosis, children were seen a median of 7.5 times (interquartile range (IQR) 4.25 to 11) by an orthopaedic surgeon, and a median of 9.5 times (IQR 8 to 18.25) by a physiotherapist. One centre had operated on all of their children, while another had operated on none. Overall, 17 (53%) of the children were managed conservatively in the two-year follow-up period, and 15 (47%) of the children underwent surgery in the two-year follow-up period. Conclusion. The results of this case review demonstrate a variation of care provided to children in the UK with Perthes’ disease. Further national and international understanding of current care is required to underpin the rationale for different treatment options in children with Perthes’ disease. Cite this article: Bone Joint Open 2020;1-11:691–695

Aims. Perthes’ disease is a condition leading to necrosis of the femoral head. It is most common in children aged four to nine years, affecting around one per 1,200 children in the UK. Management typically includes non-surgical treatment options, such as physiotherapy with/without surgical intervention. However, there is significant variation in care with no consensus on the most effective treatment option. Methods. This systematic review aims to evaluate the effectiveness of non-surgical interventions for the treatment of Perthes’ disease. Comparative studies (experimental or observational) of any non-surgical intervention compared directly with any alternative intervention (surgical, non-surgical or no intervention) were identified from: Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, the Cumulative Index to Nursing and Allied Health Literature (CINAHL), EMcare, Allied and Complementary Medicine Database (AMED), and the Physiotherapy Evidence Database (PEDro). Data were extracted on interventions compared and methodological quality. For post-intervention primary outcome of radiological scores (Stulberg and/or Mose), event rates for poor scores were calculated with significance values. Secondary outcomes included functional measures, such as range of movement, and patient-reported outcomes such as health-related quality of life. Results. In all, 15 studies (1,745 participants) were eligible for inclusion: eight prospective cohort studies, seven retrospective cohort studies, and no randomized controlled trials were identified. Non-surgical interventions largely focused on orthotic management (14/15 studies) and physical interventions such as muscle strengthening or stretching (5/15 studies). Most studies were of high/unknown risk of bias, and the range of patient outcomes was very limited, as was reporting of treatment protocols. Similar proportions of children achieving poor radiological outcomes were found for orthotic management and physical interventions, such as physiotherapy or weightbearing alteration, compared with surgical interventions or no intervention. Conclusion. Evidence from non-randomized studies found no robust evidence regarding the most effective non-surgical interventions for the treatment of children with Perthes’ disease. Future research, employing randomized trial designs, and reporting a wider range of patient outcomes is urgently needed to inform clinical practice. Cite this article: Bone Jt Open 2020;1-12:720–730

Introduction. Legg-Calve-Perthes (Perthes Disease) was first recognised by three physicians, Arthur Legg (1874–1939), Jacqui Calve (1875–1954) and George Perthes (1869 – 1927) in 1910. Perthes disease is a rare childhood condition that affects the hip. It occurs when the blood supply to the femoral head is disrupted. Without this blood supply, the bone cells die and avascular necrosis can occur. The Herring classification is used to diagnose the stages of Perthes Disease. It is an important prognostic factor. There are three classifications, Herring A, B and C. Herring A has no involvement of the lateral pillar with no density changes noted on x-ray. Herring B has at least 50% of the lateral pillar height maintained on x-ray. Herring C has less than 50% of the lateral pillar height on x-ray (Herring et al, 1992). Children with Perthes disease require specialist Limb Reconstruction team throughout their treatment journey, this includes Orthopaedic surgery and therapy (Physiotherapy and Occupational Therapy). The National Limb Reconstruction Therapy Team is based at the National Orthopaedic Hospital, Cappagh. The therapy team consists of 1 Clinical Specialist Physiotherapist, 1 Senior Physiotherapist and 1 Senior Occupational Therapist who provide input to this cohort. This study aims to analyse the importance of a comprehensive pre-operative assessment by the therapy team (Physiotherapist and Occupational Therapist) to maximise patient outcomes post operatively. Methodology. This is a quantitative research study conducted by the National Limb Reconstructive Therapy Service of the National Orthopaedic Hospital in Cappagh, Dublin. The inclusion criteria for this study consisted of:. Age – Patient must be part of the Paediatric Service, i.e., under the age of 16. Diagnosis – Patient must have a diagnosis of Perthes Disease with a Herring Classification documented. Application of a Hip Distractor Frame formed part of the patient's surgical management. Surgery was completed by Mr Connor Green. Surgery was completed between January 2021 and December 2022. Patient were required to have their external hip distractor frame removed by December 2022. Exclusion Criteria: Those not meeting the above inclusion criteria. Following the inclusion criteria, a number of cases were identified of which 10 cases were selected at random. A retrospective analysis of these samples was completed. The medical charts were reviewed as well as patient electronic healthcare records. Microsoft Excel was utilised to analyse the data and capture results. Results. From analysing the data, the following results were identified:. 80% of the sample cohort had a length of stay of 5 days following surgery. There were two outliers due to infection who had a length of stay of 14 days. 90% of the sample received a pre-operative Physiotherapy and Occupational Therapy assessment. This assessment included information gathering regarding the child's home and social environment; their functional baseline and anticipated post-operative needs. Standardised and non-standardised assessments were used. 88.89% of those who completed a pre-operative assessment required referral to community Occupational Therapy teams for equipment provision (wheelchair, transfer aids) to allow for timely discharge. On average, each patient in the sample required 17 physiotherapy outpatient sessions prior to handover to the community teams. 100% of our sample required post operation onward referral for MDT input in the community (Occupational Therapy and Physiotherapy). Conclusions. The importance of a multi-disciplinary approach towards family and children was highlighted in this study. A comprehensive pre-operative therapy assessment optimizes care for this cohort by preparing them in terms of equipment provisions, local team input and expectations for therapy. The data suggests future Limb Reconstruction team should include Physiotherapy and Occupational Therapy as part of the multi-disciplinary team, in the treatment of children with Perthe's Disease. We suggest an MDT pre-assessment is completed to optimize patient care, reduce length of stay and improve patient satisfaction in the acute hospital setting

Introduction. 2010 marked a century since Perthes' disease was first described, but the aetiology and mechanism remain unknown. Worldwide, the incidence of Perthes' disease varies widely, yet this may be through differences in study design, population denominators or case ascertainment. It is suggested that differential exposure to adverse socioeconomic circumstances may be a key precipitant, although this remains the subject of debate. This work draws on several epidemiological studies that have sought to develop the understanding of Perthes' disease by examining a case register from Merseyside, discharge data from Scotland and the world's largest community disease register. A systematic review was performed to ensure a robustness and homogeneity between published studies in order to allow meaningful comparisons. Methods. Studies were based on data from the Merseyside Perthes' Disease Register (1976–2008), the UK General Practice Research Database (GPRD, 1990–2008) and hospital discharge data for Scotland (2000–2009). Temporal trends and geographic patterns were analysed and the relationship to deprivation investigated. A systematic review of the published literature was used to explore international variations in incidence up to December 2010 focusing upon the influence of race and latitude. Analyses were conducted using Poisson regression. Results. Systematic Review: 21 studies were included which described 27 populations in 16 countries, observing 124 million person years. The annual incidence ranged from 0.2 – 19.1 per 100,000 0–14 year-olds. Race was a key determinant with East Asians least affected and Whites most affected (East Asian IRR 1.0 (Ref), South Asian IRR 2.9 (2.4, 3.5), White IRR 8.8 (8.2, 9.6)). Latitude was a strong predictor of disease, even after adjusting for race. Each 10 degree increase in latitude resulted in the incidence rate increasing by a factor of 1.44 (1.30, 1.58). GPRD Study. There was a dramatic decline in Perthes' disease incidence within the UK, with annual incidence rates falling from 12.2 to 5.7 cases/100,000 0–14 year-olds over the 19 year study period (p<0.001). There was marked geographic variation in incidence with incidence rates in Scotland more than twice those in London (10–39 (95%CI 8.05 – 13.2) vs 4.6 (95% CI 3.4 – 6.1) per 100.000 0–14 year-olds). A more rapid decline in incidence was apparent in the Northern regions compared to Southern regions. The most deprived quintile had the highest disease incidence (rate ratio 1.49 (95% CI 1.10 – 2.04)) and, with the exception of London, regional incidence showed a strong linear relationship to regional deprivation score (p<0.01). Merseyside. There was a dramatic decline in Perthes' disease incidence within Liverpool with rates falling from 14.2 to 7.7 cases/100,000 0–14 year-olds over the 34-year study period (p<0.001). Incidence rates similarly halved within the nearby region of Knowsley (p=0.01) but remained largely static in the more affluent regions of Sefton where the incidence has remained around 7.2 cases/100,000 0–14 year-olds (p=0.73). The association with socioeconomic deprivation is striking with over three times the incidence in the most deprived quintile of multiple deprivation compared to the most affluent quintile of IMD (11.5 vs 3.8 cases/100.000 0–14 year-olds (p<0.001). The incidence, by ward region, was strongly correlated to the ward index of deprivation (p<0.001) (IRR 1.014 (1.007 – 1.021)). Scotland. Hospital discharge rates due to Perthes' disease fell annually by 5.6% (95% CI 2.4% – 8.8% p<0.001) between 2000–2010. Given that there has been no significant change in treatment practice during this period and the results of the above studies, this is likely to represent a real change in disease incidence. There was a strong association between socioeconomic deprivation and disease with rates amongst the most deprived quintile more than twice those of the most affluent (RR 2.1 (1.5 – 2.9)). Similar incidence gradients for deprivation were seen in both urban and rural environments. Conclusions. These studies provide strong evidence to suggest a declining incidence of Perthes' disease within the UK and a strong relationship to socioeconomic deprivation. Although Perthes' disease incidence is falling it remains an important cause of child morbidity and exemplifies socioeconomic inequalities in health. The striking UK North–South divide is similar to that seen in adult diseases such as cardiovascular disease and osteoporosis. Latitude has an independent association with disease which may be through the action of sunlight and Vitamin D. A deprivation-related exposure (probably acting prenatally) appears critical but the aetiological determinants remain elusive

Purpose of the study is to investigate the outcome of the patients with Perthes disease who have had a surgical dislocation of their hip for the treatment of resultant symptoms from the disease process. Retrospective review of consecutive patients treated with surgical dislocation of the hip for Perthes disease. Review of clinical case notes and radiological imaging. Patient outcome was assessed at follow-up. Between 2010 and 2015, 31 cases of surgical hip dislocation were performed for Perthes disease at our institution by 2 senior surgeons. Age range at time of surgery was 12–33. Male:female ratio was 13:18; right:left ratio was 15:17. Age at the time of Perthes diagnosis was between 3 and 13 years, with 3 diagnosed retrospectively. Mean follow-up was 18months. All patients had an EUA and arthrogram while 61.3%(19/31) had previous surgery for Perthes. 71%(22/31) required a labral repair, 6.5%(2/31) had a peri-acetabular osteotomy at the time of surgery and 3.2%(1/31) required a proximal femoral valgus osteotomy. 22.5%(7/31) required microfracture (femoral head or acetabulum): all of whom had evidence of contained area of degenerative changes on preoperative MRI. 64.5%(20/31) had the trochanteric screws removed. Complications included 1 greater trochanter non-union, 1 pain secondary to suture anchor impinging on psoas tendon, 1 AVN leading to early THR 12 months post-op. Another 2 had further deterioration of degenerative changes and pain leading to THR 18 and 24 months post-op. All 3(9.7%) had microfracture at the time of the dislocation for established degenerative change and also required custom made prostheses. Surgical hip dislocation is an option in treating Perthes patient with resultant symptoms such as impingement. Improved outcome is seen in patients who are younger with a congruent hip joint in contrast to those with established degenerative change evident on MRI / intraoperatively and have an arrow shaped femoral head

Northern Ireland has previously demonstrated high incidence of Perthes' disease (11.6 per 100,000). The aim of this study is to confirm a declining incidence in this diagnosis in Northern Ireland. Methods. A reduction in new Perthes' patients had been noted by the senior author. A retrospective study was designed to identify patients with a new diagnosis of this disease over a 7 year period (2004–10). Clinical notes were interrogated using word searches. Further cross referencing with x-ray system reports were utilised to capture all new patients presenting with subsequently proven Perthes'. Patients not resident in Northern Ireland at the time of diagnosis were excluded. Patients with epiphyseal dysplasia or avascular necrosis secondary to treatment of developmental dysplasia of the hip were also excluded. A previous study from this unit had demonstrated 313 new cases over the 7 year period 1992–1998, with average incidence of approximately 45/year for the population. Results. All years in our study (2004–10) have had less than 30 for the entire population (<8.3 per 100,000). Significant variation between years with some years much less detected, so further searches are been undertaken to ensure completeness if data capture. Conclusions. Perthes' disease incidence appears to have reduced significantly. Factors such as a lower threshold for investigation, better radiological diagnostics and better understanding of Perthes' should artificially increase the incidence. However, these study findings suggest a decrease. Passive smoking has long been debated as a possible cause in Perthes' disease. The prevalence of smoking is falling in N. Ireland. The 2007 introduction of a workplace smoking ban coincided with a statistically significant fall in smoking behaviour from 25% in 2006/07 to 23% in 2007/08. The cause of Perthes' is undoubtedly multifactorial, but these findings do coincide with decreasing smoking habits in this captive population

This study sought to determine the genetic contribution of Perthes' disease, using the world's largest twin-registry. We extracted all twin pairs from the Danish Twin Registry (DTR) in which at least one individual had Perthes' Disease. The DTR captures every twin pair born alive in Denmark. Those with Perthes' disease were identified using health record linkage to the Danish Morbidity Record. Probandwise concordance was calculated to describe the likelihood that any given individual had LCPD if their co-twin was also diagnosed. There were 81 twin pairs; 10 monozygotic (MZ), 51 dizygotic (DZ), and 20 unclassified (UZ). There was no association between birth weight and being the affected co-twin. Four pairs (two dizygotic and two unclassified) were concordant for LCPD, which is greater than would be expected assuming no familial aggregation. There were no concordant MZ twin pairs. The overall probandwise concordance was 0.09 (95% CI 0.01–0.18): 0.00 for the MZ, 0.08 (95% CI 0.00–0.18) for the DZ, and 0.18 (95% 0.00–0.40) for the UZ twin pairs. This study found evidence of familial clustering in LCPD but did not demonstrate a genetic component. The absolute risk that a co-twin of an affected individual will develop LCPD is low, even in the case of MZ twin pairs

Perthes disease is a childhood disorder often resulting in femoral head deformity. Categorical/dichotomous outcomes of deformity are typical clinically, however quantitative, continuous measures, such as Sphericity Deviation Score (SDS), are critical for studying interventions. SDS uses radiographs in two planes to quantify femoral head deformity. Limitations of SDS may include non-orthogonal planes and lost details due to projections. We applied this method in 3D, with specific objectives to: 1. Develop SDS-like sphericity measures from 3D data 2. Obtain 2D and 3D sphericity for normal and Perthes hips 3. Compare slice-based (3D) and projection-based (2D) sphericity CT images of 16 normal (8 subjects) and 5 Perthes hips (4 subjects) were segmented to create 3D hip models. Ethics board approval was obtained for this study. SDS consists of roundness error (RE) in two planes and ellipsoid deformation (ED) between planes. We implemented a modified SDS which was applied to (a) orthogonal projections simulating radiographs (sagittal/coronal; 2D-mSDS), and (b) largest radii slices (sagittal/coronal; 3D-mSDS). Mean 2D-mSDS was higher for Perthes (27.2 (SD 11.4)) than normal (11.9 (SD 4.1)). Mean 3D-mSDS showed similar trends, but was higher than 2D (Perthes 33.6 (SD 5.3), normals 17.0 (SD 3.1)). Unlike 2D-mSDS, 3D-mSDS showed no overlap between groups. For Perthes hips, 2D-mSDS was consistent with SDS. For normal hips, 2D-mSDS was higher than expected (similar to Stulberg II). Projection-based (2D) measures may produce lower mSDS due to spatial averaging. Slice-based (3D) measures may better distinguish between normal and Perthes shapes, which may better differentiate effectiveness of treatments

Objective. To compare the effectiveness of arthrodiastasis with shelf acetabuloplasty for Perthes' disease in older children, by assessing the radiological outcome in matched pairs of children at skeletal maturity. Design. Retrospective observational study case series. Patients and Methods. Children were selected who had Perthes' disease, which was in the initial or fragmentation stage at presentation, with a chronological age > 7 years at diagnosis and Grade B or B/C borderline (using the modified Herring's lateral pillar classification). Exclusion criteria were other surgery for Perthes', and if they were skeletally immature at time of latest follow up. The matching criteria were age at diagnosis, sex, ethnicity, modified lateral pillar classification. The children who had a shelf were drawn from one institution and arthrodiastasis from the other. In both centres, the prevalence of Perthes' is similar. 11 matched pairs were identified (22 children). Main outcome measures. Stulberg class at skeletal maturity and complications Results - There was no statistical difference between the Stulberg class at maturity in the two groups. 6 pairs had the same class. One shelf patient developed a temporary peroneal nerve palsy. Nine of the eleven arthrodiastasis patients developed pin track infections and one had a pin breakage. Conclusion. The study is limited by small numbers, but the radiographic results appear similar. The complications were different. Both appear valid options for B or B/C border hips before onset of head deformity, but ultimately the choice of treatment depends on patient and surgeon preference

Objective. There is mounting evidence to suggest a vascular insult is responsible for Perthes' disease, and it is suggested that this may have long-term implications for the vascular health of affected individuals. This study sought to use ultrasound measures to investigate vascular structure and function in children affected by Perthes' disease. Material and Methods. This case control study encompassed 149 cases and 146 controls, frequency matched for age and sex. Endothelial function was measured using the technique of flow-mediated dilatation of the brachial artery, and alterations in arterial flow were recorded in response to an ischaemic stimulus. Results. There was a significant structural alteration in the vasculature amongst individuals with Perthes' disease (resting brachial artery diameter (cases 2.97mm vs. controls 3.11mm; p=0.01)), which remained even after adjusting for height. Additionally, there was a notable reduction in blood velocity (cases 33.84cm/sec vs controls 37.83cm/sec; p=0.01) and blood flow (cases 149.82ml/min vs. controls 184.67 ml/min; p=0.001), which was independent of baseline arterial size. There was no evidence to suggest that flow mediated dilatation of the brachial artery was impaired amongst affected individuals (p=0.71). Conclusion. Children with Perthes' disease exhibit small artery calibre and reduced function, which is independent of body composition. This data implies that that Perthes' disease may reflect a wider vascular phenomenon that could have long-term implications for the vascular health of affected individuals

Femoral head deformity can be a devastating outcome in a small percentage of patients with Perthes' disease. Deformities usually start during the fragmentation stage. In this study, we aimed to determine the effects of Vitamin D deficiency on the natural history of Perthes' disease. Patients with Perthes' disease and Vitamin D deficiency presenting to our unit in the last 3 years were identified. All X-rays were reviewed retrospectively to determine the duration of the fragmentation and ossification stages. Treatment methods were obtained from the notes. Late presenters (i.e. after fragmentation stage) were excluded. In our unit, Vitamin D deficiency is diagnosed if levels <72 nmol/L. Fifteen patients (17 hips) with Perthes' disease were found to be Vitamin D deficient. Levels ranged from (18–71 nmol/L). The mean length of the fragmentation stage was 15.7 months which is significantly higher than quoted literature figures (8 months). Ossification stage duration was 18.8 months which was comparable to quoted figures. However, patients with severe Vitamin D deficiency (< 52 nmol/L) were found to have longer ossification stage (20.6 months) compared with patients with mild deficiency (52–72 nmol/L) (16.4 months). Seven out of 16 patients (44%) required surgical containment which is significantly higher than the usually low rates of surgical intervention. The critical fragmentation stage in Vitamin D deficiency is significantly longer putting the femoral head at higher risk of deformity and extrusion. This leads to higher rates of surgical containment. Also the severity of Vitamin D deficiency might be an important determinant of the period of time required for ossification and healing. Vitamin D level is an important prognostic factor and must be measured in all patients with Perthes' disease. Prescribing Vitamin D supplements is advisable in this group of patients. However, the effects of these supplements on the course of the disease requires further research. Level of evidence: III

Aims: To assess the value of hip arthrography in planning definitive treatment for children with DDH and Perthes’ disease. Background: It is sometimes unclear whether children with DDH and Perthes’ disease need an operation. Surgeons are guided by the clinical symptoms and signs together with the radiographic appearances of the affected hip. In our Unit children on whom an operation is considered undergo an examination under anaesthetic (EUA) and a hip arthrogram. This prospective study was developed to assess whether treatment was modified by the arthrogram. Methods: All patients who underwent an EUA and hip arthrogram for DDH or Perthes’ disease over a 10 month period were entered into this prospective study. Prior to arthrography a Consultant Children’s Orthopaedic Surgeon formulated a treatment plan. A standard arthrogram was performed which included anteroposterior, frog lateral, Von Rosen and oblique views. Traction axial loading views were taken to assess stability. Following EUA and arthrogram the Consultant Surgeon formulated a definitive treatment plan. Three Children’s Consultants were then invited subsequently to review the preoperative and arthrographic appearances blindly to monitor reproducibility. Twenty-one patients with DDH and 19 with Perthes’ disease were entered into the study. Results: In the DDH group of patients the treatment plan was modified in 12 of the 21 patients as a consequence of the arthrogram. Six of the 19 patients (31.6 %) of the Perthes’ affected patients had their treatment modified. When it was felt likely prior to EUA a conservative treatment was indicated. The correlation of intra-observer Consultant agreement was high but inter-observer Consultant agreement was moderate only. In both groups of patients the range of hip motion was significantly greater under anaesthesia. Conclusion: Static and dynamic arthrography helps decision making in patients with DDH and Perthes’ disease

Over the last several years, triple pelvic osteotomy has become our preferred method for surgical containment in Perthes disease. Since 1995, seventeen patients with Perthes disease have been treated with triple pelvic oste-otomy at our institution. Fourteen of seventeen patients (82%) had a good or excellent clinical result at latest follow-up. According to Sundt’s criteria, radiographic outcome was rated as good in fourteen patients (82%), fair in one patient and poor in two patients. Experience with the technical aspects of the procedure is necessary to avoid pseudarthrosis and iatrogenic external rotation of the acetabular fragment. To evaluate the efficacy of triple pelvic osteotomy as a method of surgical containment in Perthes disease. Recent trends point to surgery as the method of choice for containment in older children with Perthes disease. Over the last several years, triple pelvic osteotomy has become our preferred method for surgical containment in Perthes disease. Since 1995, seventeen patients (seventeen hips) with Perthes disease classified as either lateral pillar B or C have been treated with triple pelvic osteotomy at our institution. The average age at surgery was 8.5 years with an average follow-up of 4.3 years. Outcome was assessed using clinical as well as multiple radiographic criteria. Fourteen of seventeen patients (82%) had a good or excellent clinical result at latest follow-up. No patients had a residual limp or limb length inequality. Two patients had a minor postoperative complication (transient peroneal nerve palsy, meralgia paresthetica). According to Sundt’s criteria, radiographic outcome was rated as good in fourteen patients (82%), fair in one patient and poor in two patients. Triple pelvic osteotomy minimizes potential complications associated with other surgical methods such as Trendelenberg gait and shortening with proximal femoral osteotomy or hinge abduction following a Salter innominate osteotomy. Experience with the technical aspects of the procedure is necessary to avoid pseudarthrosis and iatrogenic external rotation of the acetabular fragment. Triple pelvic osteotomy is now our procedure of choice for containment in the older child with Perthes disease

Introduction: Physical challenges and a long term treatment for Perthes disease can affect patients’ behaviour in their adolescence. We carried out a study to assess the long term psychosocial development of children treated non-operatively using abduction cast and brace. Methodology: 104 patients treated for Perthes disease between 1992 and 2001 were recruited for a retrospective study. Besides clinical review, patients and their main carers were asked to fill in Strengths and Difficulties Questionnaires (SDQ). SDQ included scores for total difficulties, emotional symptoms, conduct problems, hyperactivity, peer problems, social behaviour and total impact. 70 controls, matched for age and sex, attending the clinics for other unrelated pathology were requested to fill in the same questionnaires along with their main carers. Results: Data from 91 patients was available for assessment. Age at diagnosis was 1.9 to 7.3 years (median 3.9 years). Follow-up duration was 5.6 to 15.1 years (median 8.7 years). The age of subjects at final follow up was 8.9 to 20.4 years (median 15.3 years). The mean duration of treatment in abduction cast or brace was 2.4 years. 57 controls and 69 subjects were found to be low risk for emotional disorder (p> 0.05). Similarly 58 control and 74 subjects were predicted to be low risk for behavioural disorder. 16 controls and 18 subjects had medium or high risk for hyperactivity or concentration disorder (p> 0.05; student t test). There was no significant difference between the self report and parent questionnaires for difficulties or their impact. Conclusion: Perthes disease and the resultant restricted physical activities in early childhood does not affect the emotional and mental well being of patients in a long term