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. Ganz’s studies made it possible to address joint deformities on both the femoral and acetabular side brought about by Perthes’ disease. Femoral head reduction osteotomy (FHRO) was developed to improve joint congruency, along with periacetabular osteotomy (PAO), which may enhance coverage and containment. The purpose of this study is to show the clinical and morphological outcomes of the technique and the use of an implemented planning approach. Methods. From September 2015 to December 2021, 13 FHROs were performed on 11 patients for Perthes’ disease in two centres. Of these, 11 hips had an associated PAO. A specific CT- and MRI-based protocol for virtual simulation of the corrections was developed. Outcomes were assessed with radiological parameters (sphericity index, extrusion index, integrity of the Shenton’s line, lateral centre-edge angle (LCEA), Tönnis angle), and clinical parameters (range of motion, visual analogue scale (VAS) for pain, Merle d'Aubigné-Postel score, modified Harris Hip Score (mHHS), and EuroQol five-dimension five-level health questionnaire (EQ-5D-5L)). Early and late complications were reported. Results. The mean follow-up was 39.7 months (standard deviation (SD) 26.4). The mean age at surgery was 11.4 years (SD 1.6). No major complications were recorded. One patient required a total hip arthroplasty. Mean femoral head sphericity increased from 46.8% (SD 9.34%) to 70.2% (SD 15.44; p < 0.001); mean LCEA from 19.2° (SD 9.03°) to 44° (SD 10.27°; p < 0.001); mean extrusion index from 37.8 (SD 8.70) to 7.5 (SD 9.28; p < 0.001); and mean Tönnis angle from 16.5° (SD 12.35°) to 4.8° (SD 4.05°; p = 0.100). The mean VAS improved from 3.55 (SD 3.05) to 1.22 (1.72; p = 0.06); mean Merle d’Aubigné-Postel score from 14.55 (SD 1.74) to 16 (SD 1.6; p = 0.01); and mean mHHS from 60.6 (SD 18.06) to 81 (SD 6.63; p = 0.021). The EQ-5D-5L also showed significant improvements. Conclusion. FHRO associated with periacetabular procedures is a safe technique that showed improved functional, clinical, and morphological outcomes in Perthes’ disease. The newly introduced simulation and planning algorithm may help to further refine the technique. Cite this article: Bone Joint J 2024;106-B(5 Supple B):40–46

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. To identify a suite of the key physical, emotional, and social outcomes to be employed in clinical practice and research concerning Perthes' disease in children. Methods. The study follows the guidelines of the COMET-Initiative (Core Outcome Measures in Effectiveness Trials). A systematic review of the literature was performed to identify a list of outcomes reported in previous studies, which was supplemented by a qualitative study exploring the experiences of families affected by Perthes’ disease. Collectively, these outcomes formed the basis of a Delphi survey (two rounds), where 18 patients with Perthes’ disease, 46 parents, and 36 orthopaedic surgeons rated each outcome for importance. The International Perthes Study Group (IPSG) (Dallas, Texas, USA (October 2018)) discussed outcomes that failed to reach any consensus (either ‘in’ or ‘out’) before a final consensus meeting with representatives of surgeons, patients, and parents. Results. In total, 23 different outcome domains were identified from the systematic review, and a further ten from qualitative interviews. After round one of the Delphi survey, participants suggested five further outcome domains. A total of 38 outcomes were scored in round two of the Delphi. Among these, 16 outcomes were scored over the prespecified 70% threshold for importance (divided into six main categories: adverse events; life impact; resource use; pathophysiological manifestations; death; and technical considerations). Following the final consensus meeting, 14 outcomes were included in the final Core Outcome Set (COS). Conclusion. Core Outcome Sets (COSs) are important to improve standardization of outcomes in clinical research and to aid communication between patients, clinicians, and funding bodies. The results of this study should be a catalyst to develop high-quality clinical research in order to determine the optimal treatments for children with Perthes’ disease. Cite this article: Bone Joint J 2020;102-B(5):611–617

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

Aims. The aim of this study was to assess the prognostic value of the modified three-group Stulberg classification, which is based on the sphericity of the femoral head, in patients with Perthes’ disease. Methods. A total of 88 patients were followed from the time of diagnosis until a mean follow-up of 21 years. Anteroposterior pelvic and frog-leg lateral radiographs were obtained at diagnosis and at follow-up of one, five, and 21 years. At the five- and 21-year follow-up, the femoral heads were classified using a modified three-group Stulberg classification (round, ovoid, or flat femoral head). Further radiological endpoints at long-term follow-up were osteoarthritis (OA) of the hip and the requirement for total hip arthroplasty (THA). Results. There were 71 males (81%) and 17 females. A total of 13 patients had bilateral Perthes’ disease; thus 101 hips were analyzed. At five-year follow-up, 37 hips were round, 38 ovoid, and 26 flat. At that time, 66 hips (65%) were healed and 91 (90%) were skeletally immature. At long-term follow-up, when the mean age of the patients was 28 years (24 to 34), 20 hips had an unsatisfactory outcome (seven had OA and 13 had required THA). There was a strongly significant association between the modified Stulberg classification applied atfive-year follow-up and an unsatisfactory outcome at long-term follow-up (p < 0.001). Between the five- and 21-year follow-up, 67 hips (76%) stayed in their respective modified Stulberg group, indicating a strongly significant association between the Stulberg classifications at these follow-ups (p < 0.001). Conclusion. The modified Stulberg classification is a strong predictor of long-term radiological outcome in patients with Perthes’ disease. It can be applied at the healing stage, which is usually reached five years after the diagnosis is made and before skeletal maturity. Cite this article: Bone Joint J 2021;103-B(12):1815–1820

Aims. Perthes’ disease (PD) is a childhood hip disorder that can affect the quality of life in adulthood due to femoral head deformity and osteoarthritis. There is very little data on how PD patients function as adults, especially from the patients’ perspective. The purpose of this study was to collect treatment history, demographic details, the University of California, Los Angeles activity score (UCLA), the 36-Item Short Form survey (SF-36) score, and the Hip disability and Osteoarthritis Outcome score (HOOS) of adults who had PD using a web-based survey method and to compare their outcomes to the outcomes from an age- and sex-matched normative population. Methods. The English REDCap-based survey was made available on a PD study group website. The survey included childhood and adult PD history, UCLA, SF-36, and HOOS. Of the 1,182 participants who completed the survey, the 921 participants who did not have a total hip arthroplasty are the focus of this study. The mean age at survey was 38 years (SD 12) and the mean duration from age at PD onset to survey participation was 30.8 years (SD 12.6). Results. In comparison to a normative population, the PD participants had significantly lower HOOS scores across all five scales (p < 0.001) for all age groups. Similarly, SF-36 scores of the participants were significantly lower (p < 0.001) for all scales except for age groups > 55 years. Overall, females, obese participants, those who reported no treatment in childhood, and those with age of onset > 11 years had significantly worse SF-36 and HOOS scores. Pairwise correlations showed a strong positive correlation within HOOS scales and between HOOS scales and SF-36 scales, indicating construct validity. Conclusion. Adult PD participants had significantly worse pain, physical, mental, and social health than an age- and sex-matched normative cohort. The study reveals a significant burden of disease on the adult participants of the survey, especially females. Cite this article: Bone Joint J 2022;104-B(12):1304–1312

Perthes’ disease is an osteonecrosis of the juvenile hip, the aetiology of which is unknown. A number of comorbid associations have been suggested that may offer insights into aetiology, yet the strength and validity of these are unclear. This study explored such associations through a case control study using the United Kingdom General Practice Research database. Associations investigated were those previously suggested within the literature. A total of 619 cases of Perthes’ disease were included, as were 2544 controls. The risk of Perthes’ disease was significantly increased with the presence of congenital anomalies of the genitourinary and inguinal region, such as hypospadias (odds ratio (OR) 4.04 (95% confidence interval (CI) 1.41 to 11.58)), undescended testis (OR 1.83 (95% CI 1.12 to 3.00)) and inguinal herniae (OR 1.79 (95% CI 1.02 to 3.16)). Attention deficit hyperactivity disorder was not associated with Perthes’ disease (OR 1.01 (95% CI 0.48 to 2.12)), although a generalised behavioural disorder was (OR 1.55 (95% CI 1.10 to 2.17)). Asthma significantly increased the risk of Perthes’ disease (OR 1.44 (95% CI 1.17 to 1.76)), which remained after adjusting for oral/parenteral steroid use. Perthes’ disease has a significant association with congenital genitourinary and inguinal anomalies, suggesting that intra-uterine factors may be critical to causation. Other comorbid associations may offer insight to support or refute theories of pathogenesis

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

The role of heritable thrombophilic risk factors in the pathogenesis of the Perthes’ disease is controversial. The clinical and radiological findings of Perthes’ disease may be indistinguishable from those of Gaucher’s disease, and the most common Jewish N370S Gaucher mutation is threefold greater in patients with Perthes’ disease. Familial osteonecrosis of the femoral head is associated with variant mutations of collagen type II (COL2A1 mutations). We therefore studied the potential role of genetic thrombophilia and the Gaucher and COL2A1 mutations in children with Perthes’ disease. Genomic DNA of 119 children with radiologically-confirmed Perthes’ disease diagnosed between 1986 and 2005 was analysed for the thrombophilic polymorphisms Factor V Leiden, 677T-MTHFR and FIIG20210A. The results were compared with those of a group of 276 children without Perthes’ disease. DNA was also analysed for the Gaucher mutations N370S, G insertion (84GG), L444P, Intron 2 (IVS2+1G> A) and R496H. Enzymic assays confirmed the Gaucher disease status. Collagen (COL2A1) mutations of the 12q13 gene were also analysed. The prevalence of thrombophilic markers was similar among the 119 patients with Perthes’ disease and the 276 control subjects. The prevalence of the Gaucher mutation was consistent with Israeli population carriership data and did not confirm an earlier-claimed association with Perthes’ disease. All 199 patients were negative for the studied COL2A1 mutations. We found no genetic association between Perthes’ disease and either Gaucher’s disease or COL2A1 mutations or increased genetic thrombophilia among our patients compared with the control group. A systematic review of case-control studies suggested that there was a positive association between Perthes’ disease and Factor V Leiden. The impact of this association upon the disease, although not consistent across the studies, remains unclear

Aims. Perthes’ disease (PD) often results in femoral head deformity and leg length discrepancy (LLD). Our objective was to analyze femoral morphology in PD patients at skeletal maturity to assess where the LLD originates, and evaluate the effect of contralateral epiphysiodesis for length equalization on proximal and subtrochanteric femoral lengths. Methods. All patients treated for PD in our institution between January 2013 and June 2020 were reviewed retrospectively. Patients with unilateral PD, LLD of ≥ 5 mm, and long-leg standing radiographs at skeletal maturity were included. Total leg length, femoral and tibial length, articulotrochanteric distance (ATD), and subtrochanteric femoral length were compared between PD side and the unaffected side. Furthermore, we compared leg length measurements between patients who did and who did not have a contralateral epiphysiodesis. Results. Overall, 79 patients were included, of whom 21 underwent contralateral epiphysiodesis for leg length correction. In the complete cohort, the mean LLD was 1.8 cm (95% confidence interval (CI) 1.5 to 2.0), mean ATD difference was 1.8 cm (95% CI -2.1 to -1.9), and mean subtrochanteric difference was -0.2 cm (95% CI -0.4 to 0.1). In the epiphysiodesis group, the mean LLD before epiphysiodesis was 2.7 cm (95% CI 1.3 to 3.4) and 1.3 cm (95% CI -0.5 to 3.8) at skeletal maturity. In the nonepiphysiodesis group the mean LLD was 2.0 cm (95% CI 0.5 to 5.1; p = 0.016). The subtrochanteric region on the PD side was significantly longer at skeletal maturity in the epiphysiodesis group compared to the nonepiphysiodesis group (-1.0 cm (95% CI -2.4 to 0.6) vs 0.1 cm (95% CI -1.0 to 2.1); p < 0.001). Conclusion. This study demonstrates that LLD after PD originates from the proximal segment only. In patients who had contralateral epiphysiodesis to balance leg length, this is achieved by creating a difference in subtrochanteric length. Arthroplasty surgeons need to be aware that shortening of the proximal femur segment in PD patients may be misleading, as the ipsilateral subtrochanteric length in these patients can be longer. Therefore, we strongly advise long-leg standing films for THA planning in PD patients in order to avoid inadvertently lengthening the limb. Cite this article: Bone Joint J 2021;103-B(11):1736–1741

Aims. To explore the of age of onset distribution for Perthes’ disease of the hip, with particular reference to gender, laterality and conformity to the lognormal distribution. Patients and Methods. A total of 1082 patients were identified from the Liverpool Perthes’ Disease Register between 1976 and 2010, of which 992 had the date of diagnosis recorded. In total, 682 patients came from the geographical area exclusively served by Alder Hey Hospital, of which 673 had a date of diagnosis. Age of onset curves were analysed, with respect to the predefined subgroups. Results. The age of onset demonstrated a positive skew with a median of 5.8 years (interquartile range 4.6 to 7.5). Disease onset was a mean five months earlier in girls (p = 0.01) and one year earlier in those who went on to develop bilateral disease (p < 0.001). There was no difference in the age of onset between geographical districts with differing incidence rates. The entire dataset (n = 992) conformed to a lognormal distribution graphically and with the chi-squared test of normality (p = 0.10), but not using the Shapiro-Wilk test (p = 0.01). The distribution for the predefined geographical subgroup (n = 673) conformed well to a lognormal distribution (chi-squared p = 0.16, Shapiro-Wilk p = 0.08). Given the observed lognormal distribution it was assumed that Perthes’ disease followed on incubation period consistent with a point-source disease exposure. The incubation period was further examined using Hirayama’s method, which suggested that the disease exposure may act in the prenatal period. Conclusion. The age of onset in Perthes’ disease conforms to a lognormal distribution, which allows comparisons with infectious disease epidemiology. Earlier onset in girls and those who develop bilateral disease may offer clues to understanding the aetiological determinants of the disease. The analysis suggests that an antenatal aetiological determinant may be responsible for disease. Take home message: Perthes’ disease age of onset conforms to a lognormal model, which is most typical of infectious diseases. The shape of the distribution suggests that an aetiological trigger in the pre-natal period may be an important determinant of disease. Cite this article: Bone Joint J 2016;98-B:710–14

Aims. The aims of this study were to describe the course of non-operatively managed, bilateral Perthes’ disease, and to determine specific prognostic factors for the radiographic and clinical outcome. . Patients and Methods. We identified 40 children with a mean age of 5.9 years (1.8 to 13.5), who were managed non-operatively for bilateral Perthes’ disease from our prospective, multicentre study of this condition, which included all children in Norway who were diagnosed with Perthes’ disease in the five-year period between 1996 and 2000. All children were followed up for five years. . The hips were classified according to the Catterall classification. A modified three-group Stulberg classification was used as an outcome measure, with a spherical femoral head being defined as a good outcome, an oval head as fair, and a flat femoral head as a poor outcome. . Results. Concurrent, simultaneous bilateral Perthes’ disease was seen in 23 children and 17 had the sequential onset of bilateral disease. The mean delay in onset for the second hip in the latter group was 1.9 years (0.3 to 5.5). . The five-year radiographic outcome was good in 30 (39%), fair in 25 (33%) and poor in 21 (28%) of the hips. The strongest predictors of poor outcome were > 50% necrosis of the femoral head, with odds ratio (OR) 19.6, and age at diagnosis > 6 years (OR 3.3). Other risk factors for poor outcome were the timing of the onset of disease, where children with the sequential onset of bilateral disease had a higher risk than those with the concurrent onset of bilateral disease (p = 0.021, chi-squared test). . Following a diagnosis of Perthes’ disease in one hip, there was a 5% chance of developing it in the contralateral hip. . Conclusion. These results imply that we need to distinguish between children with concurrent onset and those with sequential onset of bilateral Perthes’ disease, as the outcomes may be different. This has not been previously described. Children with concurrent onset of bilateral disease had a similar outcome to our previous series of those with unilateral disease, whereas children with sequential onset of bilateral disease had a worse prognosis. The increased risk of developing Perthes’ disease in the contralateral hip in those with unilateral disease is important information for the child and parents. . Cite this article: Bone Joint J 2016;98-B:569–75

This paper reports a high incidence of minor congenital anomalies in boys and girls with Perthes' disease compared with that in a control population. There is a similarity of the incidence of minor anomalies in the children with Perthes' disease to that in babies with a single major congenital defect. Multiple major defects were more numerous and more severe than in the control children. It is speculated that there may be a congenital abnormality affecting skeletal development which in some way makes the hip susceptible to Perthes' disease at a later date

It has been reported that there is an association between Perthes’ disease and poverty. We examined the demographic data of a group of 240 children (263 hips) who presented with Perthes’ disease in Greater Glasgow, where the mean deprivation scores are substantially greater than in the rest of Scotland, to see if this association applied and whether other clues to the aetiology of Perthes’ disease could be found. There were 197 boys and 43 girls; 39 (16.25%) had a family history of Perthes’ disease. Bone age in this series was heavily skewed towards the lower percentiles. The mean number of siblings was 1.9, with 31 (12.9%) being an only child. Maternal age at the birth of the first child showed no preponderance of older mothers. Maternal smoking during and after pregnancy was noted in 132 (55%), which compared with the 52% reported in the population of Greater Glasgow in general. Of the children in our series, 60 (25%) were in social class IV and V. However, this applies to more than half of the population of Greater Glasgow. There was no significant evidence of a preponderance of Perthes’ disease in the most deprived groups. The aetiology of Perthes’ disease is likely to be multifactorial and may include a genetic or deprivation influence resulting in delayed bone age

We have investigated the annual incidence of Perthes’ disease in Dumfries and Galloway (Southwest Scotland), in relation to the population density and socio-economic status. The incidence of Perthes’ disease in rural Scotland is comparable with that in urban areas (15.4 per 100 000). There was a direct association between the incidence of Perthes’ disease and deprivation scores, with the highest incidence in the most deprived areas. A higher incidence of Perthes’ disease was noted in areas with a lower population density compared with those with a higher density. We found no correlation between population density and deprivation scores for individual electoral wards within the region

Our aim was to investigate the relationship between urinary excretion of deoxypyridinoline (DPD) as a marker of bone resorption, and Perthes’ disease. There were 39 children with Perthes’ disease in the florid stage who collected first-morning urine samples at regular intervals of at least three months. The level of urinary DPD was analysed by chemiluminescence immunoassay and was correlated with the radiological stage of the disease as classified by Waldenström, and the severity of epiphyseal involvement according to the classification systems of Catterall and Herring. The urinary DPD levels of a group of 44 healthy children were used as a control. The median urinary DPD/creatinine (CREA) ratio was significantly reduced (p < 0.0001) in the condensation stage and increased to slightly elevated values at the final stage (p = 0.05) when compared with that of the control group. Herring-C patients showed significantly lower median DPD/CREA ratios than Herring-B patients (p = 0.03). The significantly decreased median DPD/CREA ratio in early Perthes’ disease indicated a reduced bone turnover and supports the theory of a systemic aetiology. Urinary levels of DPD may therefore be used to monitor the course of Perthes’ disease

A nationwide study of Perthes’ disease in Norway was undertaken over a five-year period from January 1996. There were 425 patients registered, which represents a mean annual incidence of 9.2 per 100 000 in subjects under 15 years of age, and an occurrence rate of 1:714 for the country as a whole. There were marked regional variations. The lowest incidence was found in the northern region (5.4 per 100 000 per year) and the highest in the central and western regions (10.8 and 11.3 per 100 000 per year, respectively). There was a trend towards a higher incidence in urban (9.5 per 100 000 per year) compared with rural areas (8.9 per 100 000 per year). The mean age at onset was 5.8 years (1.3 to 15.2) and the male:female ratio was 3.3:1. We compared 402 patients with a matched control group of non-affected children (n = 1 025 952) from the Norwegian Medical Birth Registry and analysed maternal data (age at delivery, parity, duration of pregnancy), birth length and weight, birth presentation, head circumference, ponderal index and the presence of congenital anomalies. Children with Perthes’ disease were significantly shorter at birth and had an increased frequency of congenital anomalies. Applying Sartwell’s log-normal model of incubation periods to the distribution of age at onset of Perthes’ disease showed a good fit to the log-normal curve. Our findings point toward a single cause, either genetic or environmental, acting prenatally in the aetiology of Perthes’ disease

Aims. Modular or custom-made femoral components have been preferred for total hip arthroplasty (THA) in patients with a history of Perthes’ disease because of the distortion in the anatomy of the proximal femur. However, it has not been established whether a monobloc cementless stem will fit the distorted proximal femur or whether the results of the procedure are satisfactory in this group of patients. Patients and Methods. We reviewed 68 consecutive patients who had undergone THA for childhood Perthes’ disease between June 2003 and December 2008. There were 35 men and 33 women with a mean age of 48 years (16 to 73) at the time of index arthroplasty. Their mean body mass index was 24.4 (18.3 to 32.9). Of the 68 hips, 32 were classified as Stulberg class III and 36 as class IV. The mean pre-operative shortening of the affected leg was 17.2 mm (5 to 34). The minimum follow-up was five years (mean 8.5 years; 5.2 to 10). Results. An intra-operative calcar fracture occurred in eight hips (11.8%) and was successfully treated by cerclage wiring. The mean stem version was 14.6° (-2.3 to 30; standard deviation (. sd. ) 7.3). The mean acetabular component abduction was 40.2° (23.7 to 56.0; . sd. 6.5) and the mean anteversion 28.3° (6.4 to 43.0; . sd. 7.6), respectively. The mean follow-up was 8.5 years (5.2 to 10). No dislocations occurred and no hips were revised during the course of the study. At final follow-up, the mean Harris Hip Score was 91 points (59 to 100) and the mean University of California, Los Angeles activity score was 3.2 (2 to 8). Conclusion. Monobloc cementless stems reliably restore the anatomy in Perthes’ disease at THA without the need for custom-made or modular implants. Cite this article: Bone Joint J 2017;99-B:440–444

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

The purpose of this study was to determine the annual incidence of Perthes’ disease in Korea and compare this with other populations. A survey identified all newly diagnosed children with Perthes’ disease aged 14 years or younger in South Honam, Korea, between January 1999 and December 2001. A total of 84 children were included: 29 in 1999, 28 in 2000 and 27 in 2001. The mean annual incidence was 3.8 per 100 000. This is similar to that reported in other Asian countries, but higher than in black populations and lower than in Caucasians

Children presenting with Perthes’ disease before their sixth birthday are considered to have a good prognosis. We describe 166 hips in children in this age group. The mean age at onset of the disease was 44 months (22 to 72). Mild forms (Catterall I and II) were treated conservatively and severe forms (Catterall III and IV) either conservatively or operatively. The aim of the former treatment was to restrict weight-bearing. Operative treatment consisted of innominate osteotomy and was indicated by a Conway type-B appearance on the bone scan. All the patients were followed to skeletal maturity with a mean follow-up of 11 years (8 to 15). The end results were evaluated radiologically using the classifications of Stulberg and Mose. A total of 50 hips were Catterall grade-I or grade-II, 65 Catterall grade-III and 51 Catterall grade-IV. All hips with mild disease had a good result at skeletal maturity. Of the hips with severe disease 78 (67.3%) had good (Stulberg I and II), 26 (22.4%) fair (Stulberg III) and 12 (10.3%) poor results (Stulberg IV and V). Of the Catterall grade-III hips 38 were treated conservatively of which 31 (81.6%) had a good result, six (15.8%) a fair and one (2.6%) a poor result. Operative treatment was carried out on 27 Catterall grade-III hips, of which 21 (77.8%) had a good, four (14.8%) a fair and two (7.4%) a poor result. By comparison conservative treatment of 19 Catterall grade-IV hips led to ten (52.7%) good, seven (36.8%) fair and two (10.5%) poor results. Operative treatment was carried out on 32 Catterall grade-IV hips, of which 16 (50.0%) had a good, nine (28.1%) a fair and seven (21.9%) a poor result. We confirm that the prognosis in Perthes’ disease is generally good when the age at onset is less than six years. In severe disease there is no significant difference in outcome after conservative or operative treatment (p > 0.05). Catterall grade-III hips had a better outcome according to the Stulberg and Mose criteria than Catterall grade-IV hips, regardless of the method of treatment

The deformity index is a new radiological measurement of the degree of deformity of the femoral head in unilateral Perthes’ disease. Its values represent a continuous outcome measure of deformity incorporating changes in femoral epiphyseal height and width compared with the unaffected side. The sphericity of the femoral head in 30 radiographs (ten normal and 20 from patients with Perthes’ disease) were rated blindly as normal, mild, moderate or severe by three observers. Further blinded measurements of the deformity index were made on two further occasions with intervals of one month. There was good agreement between the deformity index score and the subjective grading of deformity. Intra- and interobserver agreement for the deformity index was high. The intraobserver intraclass correlation coefficient for each observer was 0.98, 0.99 and 0.97, respectively, while the interobserver intraclass correlation coefficient was 0.98 for the first and 0.97 for the second set of calculations. We also reviewed retrospectively 96 radiographs of children with Perthes’ disease, who were part of a multicentre trial which followed them to skeletal maturity. We found that the deformity index at two years correlated well with the Stulberg grading at skeletal maturity. A deformity index value above 0.3 was associated with the development of an aspherical femoral head. Using a deformity index value of 0.3 to divide groups for risk gives a sensitivity of 80% and specificity of 81% for predicting a Stulberg grade of III or IV. We conclude that the deformity index at two years is a valid and reliable radiological outcome measure in unilateral Perthes’ disease

This nationwide prospective study was designed to determine prognostic factors and evaluate the outcome of different treatments of Perthes’ disease. A total of 28 hospitals in Norway were instructed to report all new cases of Perthes’ disease over a period of five years and 425 patients were reported and followed for five years. Of these, 368 with unilateral disease were included in the present study. The hips were classified radiologically according to a modified two-group Catterall classification and the lateral pillar classification. A total of 358 patients (97%) attended the five-year follow-up, when a modified three-group Stulberg classification was used as a radiological outcome measure. For patients over six years of age at diagnosis and with more than 50% necrosis of the femoral head (152 patients), the surgeons at the different hospitals had chosen one of three methods of treatment: physiotherapy (55 patients), the Scottish Rite abduction orthosis (26), and proximal femoral varus osteotomy (71). Of these hips, 146 (96%) were available for the five-year follow-up. The strongest predictor of outcome was femoral head involvement of more or less than 50% (odds ratio (OR) = 7.76, 95% confidence interval (CI) 2.82 to 21.37), followed by age at diagnosis (OR = 0.98, 95% CI 0.92 to 0.99) and the lateral pillar classification (OR = 0.62, 95% CI 0.40 to 0.98). In children over six years at diagnosis with more than 50% of femoral head necrosis, proximal femoral varus osteotomy gave a significantly better outcome than orthosis (p = 0.001) or physiotherapy (p = 0.001). There was no significant difference between the physiotherapy and orthosis groups (p = 0.36), and we found no difference in outcome after any of the treatments in children under six years (p = 0.73). We recommend proximal femoral varus osteotomy in children aged six years and over at the time of diagnosis with hips having more than 50% femoral head necrosis. The abduction orthosis should be abandoned in Perthes’ disease

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

We studied 16 patients suffering from osteoarthritis of the hip who had had Perthes’ disease during childhood. They were compared clinically and radiologically with a control group who had not had Perthes’ disease, in order to assess whether a generalised, pre-existing constitutional disorder was present. Nine patients with a previous history of Perthes’ disease had some other skeletal abnormality, but only three presented with clinical symptoms. Only one patient in the control group was found to have an abnormality but was symptom-free. Our findings provide further evidence that patients with Perthes’ disease may have a generalised abnormality related to chondrogenesis which can produce other skeletal anomalies that persist into adult life

Aims. It is well established that there is a strong association between Perthes’ disease and worsening socioeconomic deprivation. It has been suggested that the primary determinant driving this association is exposure to tobacco smoke. This study aimed to examine this hypothesis. Patients and Methods. A hospital case-control study (n = 149/146) examined the association between tobacco smoke exposure and Perthes’ disease, adjusting for area-level socioeconomic deprivation. Tobacco smoke exposure was assessed by parental questionnaire of smoking habits during pregnancy, and by quantitative assay of current exposure using the urinary cotinine-creatinine ratio, which is a widely used and validated measure of tobacco smoke exposure. Results. The odds of Perthes’ disease significantly increased with reported in utero exposure after adjustment for socioeconomic deprivation (maternal smoking odds ratio (OR) 2.06, 95% confidence interval (CI) 1.17 to 3.63; paternal smoking OR 2.09, 95% CI 1.26 to 3.46). The cotinine-creatinine ratio was significantly greater in cases, OR 1.63 (95% CI 1.09 to 2.43), suggesting a greater ‘dose’ of current tobacco exposure. Conclusion. An association exists between tobacco smoke exposure and Perthes’ disease but we remain unable to disentangle the association with socioeconomic deprivation. Cite this article: Bone Joint J 2017;99-B:1102–8

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

It has been shown that in the puppy, two infarcts separated by an interval of four weeks produce a disorder of long duration which results in flattening and broadening of the femoral head and which reproduces the radiological changes seen in Perthes' disease in man. The histological appearances produced by two infarcts are characteristic. In this study the histological appearance of fifty-seven femoral head biopsy specimens in Perthes' disease in man have been studied. In 51 per cent of hips histopathological changes characteristic of double infarction were present, and there were grounds for postulating that double infarction might eventually occur in all cases. The findings support the concept that the deformation of the femoral head and the chronicity of Perthes' disease in man may be due at least as much or even more to repeated episodes of infarction and the ensuing abnormalities of growth as to mechanical factors related to weight-bearing

Recent reports have suggested an association between Perthes’ disease and an underlying thrombophilic or hypofibrinolytic tendency. In Northern Ireland there is a high incidence of Perthes’ disease (11.7 per 100 000 or 1 in 607 children) in a stable paediatric population. We reviewed 139 children with Perthes’ disease and compared them with a control group of 220 aged- and gender-matched healthy primary schoolchildren with similar racial and ethnic backgrounds. There were no significant deficiencies of antithrombotic factors protein C, protein S, antithrombin III or resistance to activated protein C. A total of 53 (38.1%) of the children with Perthes’ disease had a prolonged activated partial thromboplastin time (> 38) compared with 13 (5.9%) of the control group (p < 0.001). Our findings have shown that using standard assays, thrombophilia secondary to antithrombotic factor deficiency or resistance to activated protein does not appear to be an aetiological factor for Perthes’ disease. The cause of the prolonged activated partial thromboplastin time, usually associated with a clotting factor deficiency, is under further investigation

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

Radiographs of 155 Indian children were examined to identify the acetabular changes which occur in Perthes' disease. These changes included osteoporosis of the acetabular roof, irregularity of contour, premature fusion of the triradiate cartilage, hypertrophy of articular cartilage and changes in dimensions. These changes tended to be more marked in older children and when more than half of the femoral epiphysis was involved. Comparison with 25 cases of Perthes' disease from Liverpool showed the same picture. Several of the acetabular changes noted during the active stages were also seen in a series of 24 adult hips after Perthes' disease. Radio-isotope scans of the hips of 27 children with Perthes' disease showed a consistently increased uptake in the acetabulum on the affected side, indicative of a local increase in vascularity and metabolic activity. It was possible to postulate a working model for the pathogenesis of all the acetabular changes. A number of statistical correlations suggest that most of the changes have a bearing on the final outcome

1. Perthes' disease is an ischaemic lesion of the ossific nucleus of the head of the femur which may vary both in extent and degree. It is probably never quite complete. 2. When part of the ossific nucleus only is affected, as is usually the case, it is almost invariably the antero-lateral part. 3. The process of absorption of the damaged bone is complete radiologically before there is radiological evidence of reossification. 4. Reossification always occurs in Perthes' disease. 5. The aim of treatment must be to see that the mould in which the head is shaped is the right shape when ossification occurs. 6. The deformity of the head of the femur does not occur from pressure alone, but from pressure combined with subluxation. Full unrestricted weight-bearing can be allowed with safety on a femoral head in which there are ischaemic changes provided the femoral head is well contained. 7. The time of treatment can be very greatly reduced by using operation to correct the subluxation instead of relying on external splintage. This can be achieved by subtrochanteric osteotomy with rotation, or rotation combined with varus angulation. 8. Perthes' disease and avascular necrosis of the head of the femur are different conditions with different characteristics. 9. Suggestions are made as to the nature of the disease in relation to absorption, continued growth and reossification

We used ultrasonography to examine 36 children suffering from transient synovitis and 12 children with early Perthes' disease. Widening of the joint space was revealed by ultrasonography in all affected hips with either disease. In the patients with transient synovitis, capsular distension was attributed to synovial effusion, while in the patients with Perthes' disease it was produced by thickening of the synovial membrane. Neither capsular distension nor thickening of the joint cartilage was seen in the contralateral normal hip in the patients with transient synovitis, but they were common in early Perthes' disease. Ultrasonography may provide significant diagnostic clues to differentiate early Perthes' from transient synovitis

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

Objective. The relationship between the index (2D) to ring finger (4D) is one of the most commonly studied anthropometric measures, which is believed to offer insight into early growth and the foetal environment. This study aimed to determine the relationship between the 2D:4D ratio and the risk of Perthes' disease in children. Methods. The 2D:4D ratio was measured in 144 cases of Perthes' disease, and 144 controls. Cases and controls were frequency matched for age and sex. Measurements were recorded using a digital venier calliper on the palmar surface of the hand. Logistic regression was undertaken adjusting for age, with stratification for sex. Results. There was a significant negative association between Perthe's disease and digit ration in the right hand in affected females OR −0.78 (95% CI 0.65 – 0.93). There was no such association in males 0.97 (0.90 – 1.05). Conclusions. There is a significant association between degree of ‘masculinisation’ and Perthes' disease. This adds evidence to suggest that a significant aetiological component in disease acts prenatally, and may begin to explain the preponderance of disease amongst males

The radiographs of 153 children suffering from Perthes' disease of one hip were studied to examine the bony outline of the femoral capital epiphysis in the unaffected hip. In 48.4 per cent of patients irregularity of the surface, flattening or dimpling, were noted; in the majority of instances (37.2 per cent) these changes were present in the initial anteroposterior radiograph. By contrast, these changes were present in only 10.4 per cent of a control series of 153 children in whom intravenous urography was being performed, these children being matched for age and sex with the children with Perthes' disease. A second unmatched control series of 49 children whose pelves were being radiographed after injury showed a 6.1 per cent incidence of contour irregularities in 98 femoral capital epiphyses. In the patients with Perthes' disease and in the control series obtained at urography the incidence of changes was inversely related to age. The possible cause and significance of contour irregularities in normal children and in those with Perthes' disease is disscussed

There is a high incidence of Perthes' disease among the children of unskilled manual workers in underprivileged urban areas in Britain. The skeletal measurements of 38 Liverpool children with Perthes' disease were compared with those of their siblings and of normal children from the inner and outer city. Children in families where Perthes' disease occurs have retarded growth of the trunk, with reduced sitting height and bi-acromial diameter. Among those who develop the disease there is also retarded limb growth, most evident as unusually small feet

Perthes' disease is common in certain urban areas within Britain. It is one manifestation of a generalised growth disorder and nutritional causes are suspected. Orthopaedic surgeons throughout the Yorkshire region recorded all new patients with Perthes' disease over two years. There were large geographical differences in incidence which could not be explained by urban-rural or social class differences. No cases were recorded in a large area within the eastern part of the region, which is in high-grade farming land and has had a relatively low infant mortality throughout this century

A prospective study was made of 119 children with transient synovitis or any other cause for synovial effusion and elevated intra-articular pressure. During a follow-up of one year not one case of Perthes' disease was diagnosed and the late clinical and radiographic changes were minimal with moderate overgrowth of the femoral head in 33% and widening of the joint space in 14.2%. Our results do not support the widely accepted concept that Perthes' disease develops as a result of the period of elevated intra-articular pressure found in transient synovitis. Further research into this and Perthes' disease should follow the premise that they are two different diseases without any aetiological connection

It has been suggested that Perthes’ disease is more prevalent in urban areas, and that the risk increases with deprivation. We present the findings of a preliminary analysis of Perthes’ disease in Northern Ireland, which is shown to have one of the highest national annual rates of incidence in the world (11.6 per 100 000). Of the 313 children diagnosed over a seven-year period, 311 were allocated to the enumeration districts of the 1991 census, thus allowing the incidence to be calculated using both spatial and non-spatial aggregation. The cases were grouped according to the size of the settlement from highly urbanised to open countryside and by level of area deprivation. While the incidence of Perthes’ disease was found to be associated with indicators of the level of deprivation for areas, there was no evidence to suggest that there was an increased risk in urban areas; the highest rate was found in the most deprived rural category

The bicompartmental acetabulum is one of the morphological changes which may be seen in children with Legg-Calvé-Perthes’ disease. Three-dimensional CT and MRI were used to analyse the detailed morphology of the acetabulum with special reference to its inner surface, in 16 patients with Perthes’ disease and a bicompartmental acetabulum. The bicompartmental appearance was seen on the coronal plane image through the acetabular fossa. The lunate surface was seen to grow laterally resulting in an increased mediolateral thickness of the triradiate cartilage. On the horizontal plane images, the acetabular fossa had deepened and had a distinct prominence at its posterior border. The combination of these morphological changes resulted in a bicompartmental appearance on plain radiography. Acetabular bicompartmentalisation appears to be the result of an imbalance of growth between the cartilage-covered lunate surface and the cartilage-devoid acetabular fossa

Serum immunoglobulin concentrations in 41 children with Perthes' disease and 82 age and sex matched controls were measured by radial immunodiffusion. Significant increases in IgG and IgM were seen in children with Perthes' disease

Recent work has suggested that thrombophilia may be an aetiological factor in up to 50% of children with Perthes’ disease, and that up to 75% may have a coagulopathy. Our aim was to test these findings in the local population of children with Perthes’ disease and attempt to correlate them with the severity of the condition. In 64 children there were only eight (12%) with low levels of clotting proteins, as defined by normal paediatric ranges. Of these eight, only five could be said to show any thrombophilic tendency

Skeletal age was estimated by examination of radiographs of the carpus in 182 children suffering from Perthes' disease after the reliability of the Greulich and Pyle Atlas had been checked for a control group of British children. A striking tendency to delayed skeletal maturation was shown in the children with Perthes' disease. This trait was also found in ninety-three unaffected siblings of the patients. The velocity of skeletal ageing as the disease progressed was estimated. In some patients the carpal skeleton failed to mature at all for periods of up to three years and the term "skeletal standstill" is applied to this phenomenon. The significance of these findings is discussed and it is suggested that the maturation defect may have aetiological significance

We performed superselective angiography in 28 hips in 25 patients with Perthes’ disease in order to study the blood supply of the lateral epiphyseal arteries (LEAs). Interruption of the LEAs at their origin was observed in 19 hips (68%). Revascularisation in the form of numerous small arteries was seen in ten out of 11 hips in the initial stage of Perthes’ disease, in seven of eight in the fragmentation stage and in five of nine in the healing stage. Penetration of mature arteries into the depths of the epiphysis was seen in four of nine hips in the healing stage. Vascular penetration was absent in the weight-bearing portion of the femoral head below the acetabular roof. Interruption of the posterior column artery was seen where it passed through the capsule in seven hips when they lay either in internal rotation or in abduction with internal rotation. We suggest that in Perthes’ disease the blood supply of the LEAs is impaired at their origin and that revascularisation occurs from this site by ingrowth of small vessels into the femoral epiphysis. This process may be the result of recurrent ischaemic episodes

Purpose: The current literature lacks a truly comprehensive examination of the use of a valgus osteotomy to treat osteonecrosis of the femoral head and Perthes disease in the pediatric and adolescent population. Owing to the severity of the pathology, a retrospective examination of the success of valgus osteotomies in treating avascular necrosis and Perthes disease in children and adolescents is warranted. Method: Twenty-four patients with diagnosis of osteo-necrosis of the femoral head or Perthes disease treated between 1995 and 2007 with a proximal femoral valgus osteotomy were identified. The causes of avascular necrosis were Perthes (N=14, 60.9%), slipped capital femoral epiphyses, (N=4, 17.4%), femoral neck fracture (N=2, 8.7%), hip septic arthritis (N=2, 8.7%) and developmental delays and idiopathic osteonecrosis (N=1, 4.3%). The average age of receiving a femoral valgus osteotomy was 10± 3.6 years. The average age at follow-up was 12.2± 4 years, mean time of follow-up from the initial valgus osteotomy to last assessment was 23± 15.3 months. Results: Postoperative pain improved significantly compared to preoperative pain (p=.002). Seventy five percent of patients (n=9) who had limitations in their daily activities preoperatively reported that they no longer experienced them after the treatment. External rotation (p=.005) and abduction (p=.003) improved significantly at follow up. Postoperative Ficat & Arlet classification was significantly better when compared to the classification before valgus osteotomy (p=0.007). Univariate analysis showed that sixty percent of patients (n=6) who had Perthes had improved in their Ficat & Arlet stage whereas one (14.3%) patient with a different diagnosis improved (p=.082). Complications from the surgery were reported in 5 of the 23 (21.7%) cases. Conclusion: The results of this study suggest that proximal femoral valgus osteotomy is a safe and effective treatment method for osteonecrosis of the femoral head in the pediatric population. Perthes patients improved their femoral head shape more than all other etiologies; there was a trend toward statistical significance in this change. Proximal femoral valgus osteotomy may improve pain and activity limitations in children with osteonecrosis of the femoral head. It is also effective at improving range of motion and femoral head x-ray appearance of the femoral head for these children

Purpose: Coxa magna is well known in Perthes’ disease but a quantitative evaluation of the early, in particular cartilaginous, enlargement of the femoral head and the necessary adaptive changes of the acetabulum (widening and/or growth) does not exist. We would like to present MR-based volumetric data. Methods: We measured the volume of the femoral head and the acetabulum in MRI by means of a software established for the sizing of tumours before therapy and the determination of liver lobe volume prior to transplantation. We evaluated MRI exams in 47 children with Perthes’ disease and 72 normal children from 4 to 9 years and present data of the affected hip in comparison to the unaffected hip and to normal hips. Results:. Femoral head:. On the average the affected head had a volume that was 47% (range 42 – 57%) larger than on the unaffected side and 44 % (range 13 – 59%) larger than in hips of healthy children. Cases with serial exams showed that the volume of the affected head increased in the course of time. Acetabulum:. On the average the acetabular volume was 21% (range 13 to 30%) larger on the affected side than on the unaffected side and 20% (range 10 to 29%) larger than in healthy children. In patients who underwent surgery (pelvic osteotomy, alone or together with intertrochanteric varus osteotomy) the acetabular volume was 24% larger (range 9 – 33%) on the affected side than on the unaffected side. In patients without surgery the acetabular volume was 16% larger (range 10 to 33%) on the affected side. Conclusions:. We found that Perthes’ disease is associated with an average increase of femoral head volume of 47% in comparison to the unaffected side and of 44% in comparison to healthy children. There was an average increase of the acetabular volume of 21% in comparison to the unaffected side and of 20% in comparison to healthy children. These data may allow a better understanding of the disease and a reappraisal of current forms of treatment. Significance: Given a chronic disproportion between the size of the femoral head and the acetabulum therapy should aim at:. Retardation of the (cartilaginous) enlargement of the femoral head. Promotion of widening or growth of the acetabulum. We believe that current conservative modes of treatment are effective through rationale A and B. Operative modalities, in particular pelvic osteotomies and/or intertrochanteric varus osteotomy, seem to be mainly effective through rationale B. By reorientation of the acetabulum and/or the proximal femur they should favour a better distribution of forces through the hip joint allowing for a gradual widening of the acetabulum. In addition, the operative trauma in the vicinity of the triradiate cartilage may have a stimulating effect on acetabular growth

We treated 98 consecutive patients with Perthes’ disease by a unilateral brace in external rotation, flexion and abduction and a further consecutive 110 by a bilateral cast with the hips in internal rotation and abduction. During treatment in the unilateral brace, six (6.1%) hips on the opposite side developed evidence of Perthes’ disease and one developed this after the brace had been removed. In children managed in bilateral casts, no contralateral Perthes’ disease was seen. Adequate containment of the femoral head may prevent subsequent changes in the opposite hip

1. The findings in a femoral head obtained at necropsy on a boy aged nine suffering from Perthes' disease are described. 2. The findings revealed that there had been avascular necrosis of the epiphysis followed by revascularisation and healing, and there was evidence to suggest a second episode of infarction. 3. The findings provide strong support for the suggestion that Perthes' disease is the result not of one but of more than one episode of major infarction

Introduction: We describe the successful treatment of advanced Perthes’ disease in 5 patients using a combined pelvic and femoral osteotomy. To our knowledge, there are no reports in orthopaedic literature describing simultaneous pelvic and femoral osteotomy as treatment for healed Perthes disease. Method: There were 4 males and 1 female, age range 10 years to 18 years (mean 13 years). All five patients were rated as Stulberg IV. We used a Tonnis pelvic osteotomy and a 20° valgus femoral osteotomy. Clinical parameters, measured pre and post operatively, included range of movement, Harris hip and pain scores (patient and parent perception of pain on an analogue scoring system). Results: The mean improvement in Harris hip score was 30 points and the mean reduction in pain score was 6. Range of movement was not affected. Complications included one case of non-union of the femoral osteotomy, successfully treated by open reduction and internal fixation with bone graft. Conclusions: Simultaneous pelvic and femoral osteotomy may improve symptoms and function in symptomatic patients with healed Stulberg IV Perthes disease

Thirty-four patients were studied through the whole of the Perthes’ disease in Alder Hey Children’s Hospital, Liverpool. The acetabular changes included osteopaenia of the roof, irregularity of its contour, and decrease in the depth. These changes were proportional to the femoral head involvement. The purpose of the study was to know the effect of the morphological changes of the femoral head on the acetabulum and its outcome. Radioisotope scans of the hip were examined in fourteen children with unilateral Perthes’ disease and comparison was made with the contralateral hip. These scans showed increased uptake on the lateral part of the acetabulum and no uptake over the avascular part of the femoral head. Average follow-up was ten years and on an average children were followed up from six years to fifteen years of age. Six readings of the measurements of various dimensions of the acetabulum and the femoral head were done. CT scan also showed irregularity in the acetabulum. Statistical tests lead to the conclusion that the decrease in the depth of the acetabulum was secondary to the femoral head involvement and the extent of its dimensional changes affected the final congruity between the femoral head and the acetabulum. Also the remodelling potential of acetabulum decreases as the child grows older. Therefore containment procedures could be done by femoral osteotomy in younger children and acetabular osteotomy may benefit older children

Introduction. Perthes' disease is associated with coxa breva, plana and magna, and a high riding prominent greater trochanter causing abductor shortening and weakness, leg shortening and extra-articular impingement. A trochanteric advancement with an infero-lateralising oblique sliding osteotomy of the proximal femur would lengthen femoral neck, improve abductor length and strength, relieve impingement and improve leg length. We assessed the mid-term outcomes for this procedure. Method. We included patients who underwent the operation by the senior author (JNOH) with more than 2 years follow-up. The osteotomies were performed minimally invasively under image intensifier guidance and fixed with blade plate or locking plates. We assessed functional scores, radiological changes in neck length, Tonnis grading for arthritis and evidence of femoral head avascular necrosis, time interval for conversion to hip arthroplasty and associated complications. Results. Twenty four patients (25 hips) underwent the procedure at mean age of 18.7 years (range:9.3–38.8) with a mean follow-up of 5 years (range:2–13.8). At the last assessment, the mean Oxford Hip Score was 41.6 (range:58–27), Non-Arthritic Hip Score was 53.4 (range:25–77) and UCLA activity score was 4.2 (range:2–6). For changes in neck length, the mean “Head-centre-to-Greater-trochanteric-tip-distance” was 60 mm (range:43–78) compared to 39 mm (range:30–48) pre-operatively and the mean “Head-centre-to-Lesser-trochanteric-tip-distance” was 54 mm (range:47–64) compared to 37 mm (range:31–41) pre-operatively. The mean Tonnis grade was 1.5 (range:1–3) compared to 1.3 (range:1–2) pre-operatively. Two patients underwent arthroplasty conversion at 2 and 13.8 years later. One patient needed head-neck debridement for impingement and 2 patients underwent trochanteric refixation for non-union. There were no cases of avascular necrosis. Discussion. Symptomatic Perthes' hip deformity in adolescents and young adults is difficult to treat with joint preserving surgery. The mid-term clinical, functional and radiological results for double proximal femur osteotomy are encouraging

Introduction. Perthes disease is associated with coxa breva, plana and magna, and a high riding prominent greater trochanter causing abductor shortening and weakness, leg shortening and extra-articular impingement. A trochanteric advancement with an infero-lateralizing sliding osteotomy of the proximal femur would lengthen femoral neck, improve abductor length and strength, relieve impingement and improve leg length. We assessed the mid-term outcomes for this procedure. Method. We included patients who underwent the operation by the senior author (JNOH) with more than 2 years follow-up. The osteotomies were performed under image intensifier guidance and fixed with blade plate or locking plates. We assessed functional scores, radiological changes in neck length, Tonnis grading for arthritis and evidence of progression in femoral head avascular necrosis, time interval for conversion to hip arthroplasty and associated complications. Results. Twenty four patients (25 hips) underwent the procedure at mean age of 18.7 years (range:9.3–38.8) with a mean follow-up of 5 years (range:2–13.8). At the last assessment, the mean Oxford Hip Score was 41.6 (range:58–27), Non-Arthritic Hip Score was 53.4 (range:25–77) and UCLA activity score was 4.2 (range:2–6). For changes in neck length, the mean “Head-centre-to-Greater-trochanteric-tip-distance” was 60 mm (range:43–78) compared to 39 mm (range:30–48) pre-operatively and the mean “Head-center-to-Lesser-trochanteric-tip-distance” was 54 mm (range:47–64) compared to 37 mm (range:31–41) pre-operatively. The mean Tonnis grade was 1.5 (range:1–3) compared to 1.3 (range:1–2) pre-operatively. Two patients underwent arthroplasty conversion at 2 and 13.8 years later. One patient needed head-neck debridement for impingement and 2 patients underwent trochanteric refixation for non-union. There was no progression in avascular necrosis of femoral head. Discussion. Symptomatic Perthes hip deformity in adolescents and young adults is difficult to treat with joint preserving surgery. The mid-term clinical, functional and radiological results for double proximal femur osteotomy are encouraging

We evaluated radiological hip remodelling after shelf acetabuloplasty and sought to identify prognostic factors in 25 patients with a mean age of 8.9 years (7.0 to 12.3) who had unilateral Perthes’ disease with reducible subluxation of the hip in the fragmentation stage. At a mean follow-up of 6.7 years (3.2 to 9.0), satisfactory remodelling was observed in 18 hips (72%). The type of labrum in hip abduction, as determined by intra-operative dynamic arthrography, was found to be a statistically significant prognostic factor (p = 0.012). Shelf acetabuloplasty as containment surgery seems to be best indicated for hips in which there is not marked collapse of the epiphysis and in which the extruded epiphyseal segment slips easily underneath the labrum on abduction, without imposing undue pressure on the lateral edge of the acetabulum

The nuclide bone-scan will reliably diagnose Perthes' disease with a sensitivity of 0.98 and a specificity of 0.95. The comparable figures for radiographic sensitivity and specificity are respectively 0.92 and 0.78. In addition, it is possible on the scan to recognise the onset of revascularisation of the femoral capital epiphysis some months before there are radiographic signs of new bone formation. Scintigraphy also suggests that in some cases of transient synovitis there may bae a period of reversible ischaemia of the capital epiphysis, which may have relevance to the pathogenesis of Perthes' disease

We studied, clinically and radiologically, the growth and remodelling of 21 hips after valgus femoral osteotomy with both rotational and sagittal correction for hinge abduction in 21 patients (mean age, 9.7 years) with Perthes’ disease. The exact type of osteotomy performed was based on the pre-operative clinical and radiological assessment and the results of intra-operative dynamic arthrography. The mean IOWA hip score was 66 (34 to 76) before surgery and 92 (80 to 100) at a mean follow-up of 7.1 years (3.0 to 15.0). Radiological measurements revealed favourable remodelling of the femoral head and improved hip joint mechanics. Valgus osteotomy, with both rotational and sagittal correction, can improve symptoms, function and remodelling of the hip in patients with Perthes’ disease

Aim: To assess the outcome for Perthes disease in children over eight treated by observation, varus osteotomy, abduction plasters and acetabular augmentation. Methods and results: A retrospective case note review of prospectively collected data for 44 children (48) hips with Catterall grades 2, 3 or 4 Perthes’ disease with onset age eight or older and followed to maturity was performed. The groups were demographically similar. For all groups combined 60% had a satisfactory Stulberg grade I to III outcome. Poorer outcomes (as assessed by Stulberg, centre edge angle and Reimer’s migration index) were associated with increasing age, greater initial head deformity and greater head involvement. Initial head deformity did not remodel for any treatment group. Indeed, progressive head deformity occurred despite plaster treatment or varus osteotomy but not after acetabular augmentation. Hips managed by acetabular augmentation also had better outcomes than the other groups for Stulberg, Reimer’s index and centre-edge angle. Conclusions: Whatever the treatment the outcome for Perthes’ disease in children over eight is poorer with increasing age. No treatment offers the prospect of a good result in the older child with significant head involvement or significant initial deformity but acetabular augmentation seems to improve Stulberg, Reimer’s migration and centre-edge angle outcomes and prevents progressive femoral head deformity compared with observation, varus osteotomy and plaster treatment

Seventeen patients, with an average age of nine years 11 months, underwent 18 Chiari osteotomies for the treatment of painful subluxation of the hips following Perthes' disease. The average follow-up period was four years three months. The 13 patients reviewed clinically all did well and none complained of pain or instability. The radiographs were examined in all 17 cases. The average centre-edge angle and percentage femoral head cover were definitely improved by the operation. No significant medical displacement of the femoral head was achieved. The clinical success may result from improved femoral head coverage and diminished eccentricity. Chiari's osteotomy is recommended for adolescent patients with painful subluxation of the hip as a consequence of Perthes' disease

The aims of this survey were to establish the familial incidence of Perthes' disease, to note any associated developmental anomalies and to collect information on preceding trauma or synovitis, on the pregnancy and birth, and on various sociological factors. Height and weight measurements were obtained for 217 patients, and comparisons made with those of their parents, unaffected sibs and (local) controls. Results showed an extremely low frequency of Perthes' disease among relatives, with no obvious pattern of inheritance. As genetic factors were not apparent, environmental and sociological causes were sought. The disease occurred particularly in children who were third-born or later in the family, and had older than average parents. Many came from low-income families and one in ten had been a breech birth, shown other malposition or had had a version late in pregnancy. Many children were already undersized at the time of developing Perthes' disease and remained short than average throughout life. Neither their parents nor sibs were shorter than normal, indicating that the patients' short stature was not familial. The child who is going to develop Perthes' disease is already constitutionally and socially at a disadvantage, and during the perinatal period and the first few years of life is perhaps more susceptible to trauma than is a normal child

The authors added a classification of posterior pillars to Herring’s classification of lateral pillars in a retrospective study of 33 patients with Perthes disease. Anteroposterior and frog position lateral radiographs taken approximately two months later from first visit, were evaluated. All patients were treated by a non-weight-bearing method (continuous traction and wheel chair activity). The outcome was evaluated by the Stulberg method. The mean age at follow-up was 18 years (range,14.2 to 26.9 years). Thirteen hips were in Catterall group II, 18 were in group III, and two were in group IV. Twenty-five of the 33 hips were in Stulberg class I or II (with good results), and eight hips were class III. Nine hips were in Herring group A, 20 hips were in group B, and four were in group C. Eleven hips were in our group A with the posterior pillar classification, 16 were in group B, and six were in group C. Group A, with a lateral or posterior pillar had good results without exception. Concerning Herring’s classification, all four heads had become aspherical in group C, however, 16 hips were good results and four hips were poor in group B. The outcome was poorly related to the classification in 20 patients with 20 affected hips in Herring group B when only lateral pillars were used in classification. When both lateral and posterior pillars are considered in classification, results can be predicted more accurately than when only lateral pillars are considered. This more accurate prognosis at an early stage of Perthes disease can facilitate effective treatment selection

The purpose of this study is to determine how the lateral shape of the acetabulum changes during Perthes disease and if there is any correlation between the lateral acetabulum shape and final result and type of treatment. The study population consisted of 243 patients with unilateral involvement who had reached skeletal maturity at last follow up. There were 35 (14.4%) female and 208 (85.6%) male patients. The mean age at the onset of symptoms was 7 years and 1 month. AP X-ray films were estimated during fragmentation, reossification and last follow up. Group A consisted of 56 hips, 126 hips were classified as group B and 61 hips as group C according to the Herring classification. For the lateral acetabular shape we proposed a classification: group A – a normal concave acetabular roof, group B – a horizontal flat roof and group C – a roof convexly rounded and up going. All hips were treated by containment methods (bed rest and traction in abduction-78 hips, Petri cast-31 hips, brace-94 hips, varus osteotomy-20 hips, Salter oste-otomy-12 hips and shelf arthroplasty-8 hips). The outcomes of treatment were evaluated according to the Stulberg classification. During fragmentation stage we found 78 (32.1%) hips with normal lateral acetabular shape-type A. Horizontal roof-type B was noted in 136 (56%) hips and in 29 (11.9%) type C was observed. We observed improvement in the shape of lateral acetabulum after treatment. At the last follow up there were 124 (51%) hips with type A, 81 (33.3%) with type B and 38 (15.7%) with type C. Statistical analysis revealed significant correlation between lateral acetabular shape and Stulberg classification. A normal concave acetabular roof at the fragmentation stage leaded mainly to Stulberg group 1and 2 whereas a roof convexly rounded and up going leaded to Stulberg group 3, 4 or 5 (p< 0.0001). Analysis showed no statistical significant correlation between treatment by using bed rest and traction in abduction, Petri cast, braces and development the lateral acetab-ular shape (p=0.09). Only treatment by using surgical methods improved the lateral acetabular shape at the last follow up (p=0.0015). The acetabulum is a mould for remodeling of the deformed femoral head in Perthes disease and the lateral acetabulum plays the most important role. We can expect that normal shape of the acetabulum gives good result at final follow up whereas a roof convexly rounded usually follows to Stulberg group 3, 4 or 5. Only surgical treatment improves the shape of the acetabulum

1. Intra-osseous venographs have been obtained in twenty-eight hips affected by Perthes disease and in twenty normal hips after the injection of opaque medium into the femoral neck. 2. In the normal hips the contrast medium drained rapidly into the local veins; none flowed distally into the diaphysis. 3. In the initial and in the fragmentation stages of Perthes' disease some contrast medium always flowed into the diaphysis and the flow into the local veins was greatly reduced. 4. In the restitution stage the venographs approached normal. 5. The implications of these findings are discussed

1. Cancellous bone grafting of the head of the femur in Perthes' disease accelerates reossification and thus shortens the period of treatment. 2. There is no evidence from this series of cases that the final shape of the head is affected for better or for worse by the operation of cancellous chip grafting, by the rate of regeneration of the head after operation or by the stage in the disease process at which the operation is done. 3. No relationship has been found between the shape of the head at the time of operation and its final shape. 4. A larger series of cases is needed to determine the value of bone grafting in Perthes' disease of the hip

The purpose of the research: demonstration of a hip caput matrix modeling opportunities, restoration of a joint stability and joint anatomy improvement in three planes after triple osteotomy of the pelvic by A.M.Sakalouski with Perthes disease patients. Patients and methods: From 1998 to 2007 years were performed 37 triple pelvic osteotomy in 36 patients at 4 to 18 (average age 11 years) with unfavorable course of Perthes disease. In 28 cases patients were male and in 8 cases patients were female. We have observed patients clinically and with radiological methods, including computer tomography with 3D-reconstruction. Indications for pelvic osteotomy include signs of poor prognosis at stages II–III. Radiological signs of risk. The indication to triple pelvic osteotomy on residual stages - this is a secondary acetabular dysplasia and decentration. If the head of a hip is hardly deformed (flat) by the first stage us carried out back or forward rotation hips osteotomy with turn on 70°–90°, and then triple pelvic osteotomy. Results: The triple osteotomy of the pelvic prevented deformation of the head of a hip at e at 2–3 stages as a result remodeling of femoral caput by spherical acetabulum. At late stages operation has provide to avoid hip arthrosis. Medialization of the hip joint which led to reducing the joint reactive force. In all cases the head centration and stability of a joint are restored. The angle of Wiberg has increased from 15° to 35°, index Eyre-Brook has increased on 20°, an index acetabular coverage – from 72% to 100%. In all cases clinically the lengthening of sick finiteness has been received with 0,5–1 cm and a gait of patients has improved. The volume of movements in a joint was on the average: flexion 110°, extension 10°, abduction 23°, adduction 30°, external rotation 37° degrees, internal rotation 13°. The stop of trochanter growth at a level of the center head rotation hip or slightly above. Conclusion: So, triple pelvis osteotomy is the way modern surgical treatment of Perthes disease, it provides preventive maintenance of deformation of the head of a hip – if operative intervention is carried out at early stages of illness when the head has potential to remolding or preventive arthrosis – at residual stages

Perthes' disease involving the whole of the femoral head in 36 children was treated by innominate osteotomy. Radiographs of all cases were reviewed to see the effect of the osteotomy on the shape, the degree of acetabular cover and any subluxation of the diseased femoral head. All femoral heads which were circular before operation remained so, and over half of the previously deformed heads became circular after the osteotomy. The improved acetabular cover provided by the osteotomy resulted in a CE angle of 25 degrees or more in 92% of hips. Possible subluxation of the femoral head was studied by inspecting Shenton's line. If this was intact before operation it remained so; of the 14 which were broken before operation, 11 were restored to normal after osteotomy. We conclude that innominate osteotomy is a worthwhile procedure for Perthes' disease involving the whole of the femoral head

The aim of the study is to evaluate the prognostic value of Herring’s classification for treatment planning of Perthes Disease and predicting the final outcome of the disease. We analysed 45 patients treated in our department in the period of 1992 – 2005. The analysis consisted of determining the lateral pillar collapse in fragmentation stage and the containment of the femoral head in the residual stage. We did retrospective analysis of the x-rays in fragmentation stage and classified the patients according to Herring’s classification. Group A included 10 patients, group B – 20 patients and group C included 15 patients. We used Hayman – Herndon Acetabulum – Head Index (AHI) to analyse the x- rays in the residual stage. The statistical analysis of the results included the analysis of the differences between the lateral pillar collapse among the groups and the differences between affected hip and the contralateral control hip. Correlation between the degree of lateral pillar collapse and the AHI in the residual stage was also analyzed. Correlation between the age of onset of the disease and AHI was determined for each group, too. The results show significant differences between the lateral pillar collapse in Herring groups, and among the affected and control hips. Differences of AHI values were also significant between the groups. Correlation between the AHI and lateral pillar collapse for each group shows moderate connection just as the one between AHI and age. Herring’s classification is reliable in classifying patients with Perthes disease. It enables good prediction of the natural course of the disease and thus influences the treatment decision-making

Perthes disease often leaves young adults with hip joint incongruency due to femoral head asphericity, (extra-articular extrusion and superior flattening). This causes femoro-acetabular impingement, a reduced range of movement and early degenerative change. We report a novel method for restoration of femoral head sphericity and femoro-acetabular congruency. Two males (aged 21 and 22 years) presented with groin pain and severe hip stiffness after childhood Perthes disease. Imaging confirmed characteristic saddle shaped deformities of the femoral head, with cartilage loss overlying a central depression in the superior section of the head. A new method of treatment was proposed. Both cases were treated in the same manner. A surgical dislocation was performed with a trochanteric flip osteotomy. The extra-articular bump was removed with osteotomes and a burr to reduce femoro- acetabular impingement. The sphericity of the femoral head was restored using a HemiCap partial re-surfacing (Arthrosurface, MA, USA). The radius of the implant was selected to match that of the acetabulum. Restoration of the height of the flattened portion of the weight-bearing surface of the femoral head reduces abnormal loading of the acetabular articular cartilage by improving congruency of the joint. Both patients recovered without incident and were mobilised with crutches, restricted to touch weight-bearing for six weeks to protect union of the trochanteric osteotomy. At a minimum of three year follow-up both patients had sustained improved range of movement, pain and Oxford hip score. Repeated imaging shows no evidence of joint space narrowing or loosening at this stage. We conclude that this novel treatment functions well in the short term. Further surveillance is on-going to confirm that this treatment results in improved long term durability of the natural hip joint after Perthes disease

Valgus extension osteotomy (VGEO) is a salvage procedure for ‘hinge abduction’ in Perthes’ disease. The indications for its use are pain and fixed deformity. Our study shows the clinical results at maturity of VGEO carried out in 48 children (51 hips) and the factors which influence subsequent remodelling of the hip. After a mean follow-up of ten years, total hip replacement has been carried out in four patients and arthrodesis in one. The average Iowa Hip Score in the remainder was 86 (54 to 100). Favourable remodelling of the femoral head was seen in 12 hips. This was associated with three factors at surgery; younger age (p = 0.009), the phase of reossification (p = 0.05) and an open triradiate cartilage (p = 0.0007). Our study has shown that, in the short term, VGEO relieves pain and corrects deformity; as growth proceeds it may produce useful remodelling in this worst affected subgroup of children with Perthes’ disease

It has recently been postulated that thrombophilia may have a role in the aetiology of Perthes’ disease. The published reports, however, remain conflicting. In this study a retrospective analysis of the coagulation parameters was made in 47 patients with Perthes’ disease and the results compared with the clinical data. Five patients with Factor V Leiden mutation were found (10.6%) and surprisingly four of them had a homozygous pattern. These four patients showed the most severe form of the disease, Catterall group IV, with flattening of the entire epiphysis, involvement of the metaphysis, shortening and broadening of the femoral neck, trochanteric overgrowth and developed mushroom-shaped aspherical laterally displaced femoral heads in dysplastic acetabula. We would like to suggest that the homozygous form of Factor V Leiden mutation has some role in the clinical course of Perthes’ disease and particularly its most severe form

We performed a paired study of mature patients with Perthes’ disease to compare the radiological results after treatment between conservatively- and surgically-treated groups. One patient was selected from each group to create the pairs for this study. Each pair was strictly matched for gender, body mass index, age at onset, stage at the first visit, necrotic area and radiological at-risk signs and each was assessed by comparing the values of six radiological measurements. Eighteen pairs (36 hips) fitted the criteria. The radiological measurements which showed a statistically better result in the surgical groups were Mose’s method, the acetabular-head index and leg-length discrepancy. There were no statistical differences in the slope of the acetabular roof and the articulotrochanteric distance. Four hips in the conservative group were in Stulberg class II, five in class III and nine in class IV. In the surgical group, 13 were in Stulberg class II, four in class III and one in class IV. We conclude that surgical treatment improved the sphericity of the femoral head and provided greater acetabular cover, but did not reform the acetabular roof. It was noteworthy that a greater leg-length discrepancy and a smaller articulotrochanteric distance were not seen in the surgical group. Our study suggests that surgical treatment is preferable in patients with severe Perthes’ disease

Perthes disease in children above 8 years old, generally has a worst prognosis. On this age group it is common that hinge abduction appears in a descentered and uncontained hip, situation that has a difficult solution with the standard surgical procedures. On those cases arthrodiastasis as described, can be a valuable treatment option. The rationale of arthrodiastasis on Perthes is that it permits to reduce the hip, protect it during the fragmentation stage, and creating a vacuum phenomenon inside the acetabulum it “insufflate” the collapsed plastic head, permitting the reconstruction of a spherical head. Our actual protocol to treat Perthes disease in a more than 5 years old child, include a transphyseal tunneling made as soon as possible on the necrotic stage and protection of the hip in a abduction-flexion brace. If at any time a hinge hip develops then arthrodiastasis is applied. The procedure is simple, fast and low traumatic, including the positioning in a traction table, application of a Ilizarov frame with hinges centered on the center of rotation of the head, with the limb in a position of abduction and slight flexion, which permits the reduction and containment of the hip, and then a progressive arthrodiastasis to 1 1.5 cm. The frame was used for 3–5 months and during this period one could assist to the progressive growth of the collapsed femoral head. After arthrodiastasis the hip is protected with an abductionflexion brace for a mean of 8 months. The Authors present the 5 first cases where this methodology was applied, standing out the good results obtained, without complications, mentioning the faster evolution to reconstruction stage in the cases where transphyseal tunneling was done, permitting a shorter period of arthrodiastasis

Background: Current treatment for Perthes disease aims at preventing deformation of the femoral head during the active stage of the disease by obtaining containment of the femoral head. To effectively pre-empt femoral head deformation, one needs to know, when during the disease irreparable femoral head deformation occurs. This study was undertaken to attempt to clarify this. Methods: Records and 2634 pairs of radiographs (AP and lateral) of 610 patients with Perthes’ disease were reviewed. The evolution of the disease was divided into seven stages (Stages Ia, Ib, IIa, IIb, IIIa, IIIb & IV) based on plain radiographic appearances. Intra-observer and inter-observer reproducibility of this new classification system was assessed. The duration of each stage of the disease was noted. The stage at which epiphyseal extrusion and widening of the metaphysis occurred and the stage at which metaphyseal and acetabular changes appeared were identified. The shape and the size of the femoral head, the extent of trochanteric overgrowth and the radius of the acetabulum were assessed in hips that had healed. Results: The reproducibility of the new classification system of the evolution Of Perthes’ disease was good. The median duration of each stage varied between 95 and 326 days. Epiphyseal extrusion and metaphyseal widening was modest in Stages Ia, Ib and IIa but increased dramatically after Stage IIb. > 20% extrusion occurred in 70% of the hips by Stage IIIa. Metaphyseal changes were most frequently encountered in Stage IIb, while acetabular changes were most prevalent in Stage IIIa. At healing, only 24% of untreated patients had spherical femoral heads, while 52% had irregular femoral heads. Conclusions: The new classification of the stages of evolution of Perthes’ disease helps to identify when crucial events occur during the course of the disease. The timing of epiphyseal extrusion, metaphyseal widening and the appearance of adverse metaphyseal and acetabular changes suggest that femoral head deformation occurs by Stage IIIa in untreated hips. Hence, if containment were to succeed, it should be achieved before this stage

1 . Thirty-four cases of Perthes' disease followed into adult life were reviewed twenty-five to forty years (average thirty years) after diagnosis. 2. Rather more than one-third of the patients developed hips which were good, an equal number were considered fair and about one-quarter were poor. 3. Four out of five patients were fully active and free from pain but only two out of five had hips which were radiologically good. 4. Clinical and/or radiological deterioration had seldom occurred in the last twelve years. 5. A good result in childhood is likely to be maintained with no pain and good function up to the age of forty years and perhaps longer

Background: Femoral varus osteotomy for Perthes’ disease aims at achieving Containment to prevent femoral head deformation. Theoretically, ontainment is most likely to succeed if it is achieved before the femoral head extrudes and is subjected to deforming stresses. It would follow that the timing of the procedure is an important factor in determining the outcome. This study was undertaken to verify this. Methods: Records and radiographs of 610 patients with Perthes’ disease were analysed. The data of 302 patients who underwent femoral osteotomy were compared with those of non-operated patients. A new modification of the Elizabthtown classification of the stages of evolution of the disease with seven stages (Stages Ia, Ib, IIa, IIb, IIIa, IIIb & IV) was used to identify the timing of surgery and to monitor the progress of the disease following surgery. The results of treatment were assessed at healing by Mose’s criteria. Multivariate analysis was used to identify variables that influenced the shape and size of the femoral head at healing. Results: 22 patients among 86 who were operated in Stage Ia or Ib by-passed the stage of fragmentation. The extent of metaphyseal widening was considerably less in operated children. At healing, spherical femoral heads were seen in 72% of operated hips as compared to 24% of non-operated hips. The variables that influenced the shape of the femoral head at healing were, metaphyseal width, sex, age at onset, epiphyseal extrusion and the stage at surgery. Patients who were operated before Stage IIb had significantly better results than those operated later. Conclusions: The results of the study support the impression that the timing of containment is an important factor that influences the outcome in Perthes’ disease. The best results are obtained if containment is achieved before Stage IIb

The changes of stress distribution in the femoral head with Perthes disease were observed under several condition. Finite element models were constructed referring to X-ray images and magnetic resonance images of the intact hip joint. The model was divided into five parts: cancellous bone, articular cartilage, necrotic bone, cortical bone, physeal cartilage. Material properties were alloted to these components by the past literature. The body weight and abductor muscle force were applied as loading. The model was altered to study the effect of age, the extent of necrosis, and lateralization of the fomoral head. Analysis were performed on a digital computer PC-9821(NEC) using the finite element program. There was no significant difference in stress distribution patterns regardless of age or extent of necrosis. However, compressive stresses were concentrated on the lateral portion of the epiphysis by lateralization of femoral head. The femoral head deformity in Perthes disease was more affected by the lateralization than by the age and the extent of necrosis

Aim: To evaluate the long term effect of Distal transfer of the greater trochanter in Perthes’ disease. Patients and methods: Twelve patients (thirteen hip joints, 10 males and 2 females) who suffered from Perthes’ Disease were treated by distal transfer of the greater trochanter (DTT). The operation was performed because of progressive shortening of the articulo-trochanteric distance accompanied by signs of insufficiency of the hip abductors. Total or near total femoral head involvement was found in all the patients except for one of them. Follow up period was 28 years (21–35). Mean age at diagnosis of Perthes’ was 7 years (4–11). Patient were studied in 1992 and reviewed again 13 years later, using the Harris hip score, short form 36 (SF36), physical examination and A-P X-rays. Results: Not one of the patients underwent a total hip arthroplasty. Mean Harris hip score is 80 (range 54–100, 4 patients under 70 score). Mean SF36 score was 71 (range 30–94) and was correlated to the Harris score. Three of the patients were working in a physically demanding profession. The rest were office workers. Two of them chose non strenuous type of work due to the hip condition. Limb length discrepancy was 1.7 cm short on the operated side (range 0–3) and correlated with Trendelenburg sign (4 patients with positive sign). Femoral head sphericity according to Stulberg classification was good in 5 patients (grades 1–2), fair in 3 patients (grade 3) and poor in 4 patients (grade 4–5). Head sphericity was not correlated to age at diagnosis, Harris score, SF36 score or level of hip pain. Conclusions: Long term outcome are surprisingly good in those patients, considering the degree of head involvement, advanced age at diagnosis and severity of disease which necessitated high degree of varus osteotomy and hence trochanter transfer

In a prospective five-year study, 294 episodes of acute transient synovitis of the hip were diagnosed in 275 children. The average annual incidence was 0.2% and the accumulated risk of suffering at least one episode was 3%. The risk of recurrence was 20 times greater than the risk of having a single episode. Perthes' disease was diagnosed from one to five months after the acute attack of synovitis in 10 cases (3.4%). Review of the initial radiographs revealed signs of avascular necrosis in three of the 10 cases, and an increased joint space in five. Only two cases had had completely normal radiographs. The value of routine radiographs taken after three months was minimal. Factors associated with the incidence of Perthes' disease included prolonged time in traction before the range of hip movement became normal, increase in joint space on the initial radiographs and the recurrence of hip symptoms after initial relief

We studied the natural history of Perthes’ disease in 62 children in whom the onset of symptoms was in adolescence. Three patterns of disease were noted, namely, late-onset pattern, segmental collapse, or destructive with failure of revascularisation. In the late-onset pattern, the disease followed the sequence of healing seen in younger children, but adequate epiphyseal remodelling did not occur. Consequently, the femoral head was never spherical after revascularisation. With segmental collapse, early and irreversible collapse of part of the epiphysis occurred with gross deformation of the femoral head. The destructive pattern was characterised by a failure of revascularisation and repair of the avascular epiphysis. The radiological outcome was poor in all three patterns. The poorest clinical results were found in the destructive type which was frequently associated with incapacitating pain requiring arthrodesis or excision arthroplasty within three years of onset of the disease

Of 193 children with Perthes' disease at the Texas Scottish Rite Hospital for Cripppled Children, 24 were found to have only minimal changes. The cases were grouped relative to the area of the femoral head involved, with 10 involving the anterior portion, seven the posteromedial, three the lateral, and four the central portion. Almost all hips lost some height as measured by the epiphysial index but all had good results by the Mose criteria irrespective of treatment. The anterior lesion is the same as that described by Catterall as Group 1. The three additional groups have a similar benign natural history but distinct radiographic features. The need for early recognition of these patterns is emphasised if unnecessary treatment is to be avoided. Possible correlations of these lesions with the segmental blood supply of the femoral head are proposed and an hypothesis relating the Catterall classifications to the blood supply is put forward

We have examined the effect of arthrodiastasis on the preservation of the femoral head in older children with Perthes’ disease. We carried out a prospective trial in boys over the age of eight years and girls over seven years at the time of the onset of symptoms. The patients had minimal epiphyseal collapse and were compared with a conventionally treated, consecutive, historical control group. Arthrodiastasis was applied for approximately four months. The primary outcome measure was the extent of epiphyseal collapse at the end of the fragmentation phase. One of the 15 treated hips and nine of the 30 control hips showed a loss of height of 50% or more of the lateral epiphyseal column on the anteroposterior radiographs (Herring grade-C classification). On a Lauenstein view, one of the treated hips and 19 of the control hips showed at least a loss of height of 50% of the anterior epiphyseal column. The complications of arthrodiastasis included pin-site infection in most hips, transient joint stiffness in two, and breakage of a pin in two. The final outcome will be known when all the patients and the control group reach skeletal maturity

Severe femoral head deformities due to Perthes' disease are characterized by limitation of ROM, pain, and early degeneration, eventually becoming intolerable already in early adulthood. Morphological adaptation of the acetabulum is substantial and complex intra- and extraarticular impingement sometimes combined with instability are the underlying pathologies. Improvement is difficult to achieve with classic femoral and acetabular osteotomies. Since 15 years we have executed a head size reduction. With an experience of more than 50 cases no AVN of the femoral head was recorded. In two hips fracture of the medial column of the neck has been successfully treated with subsequent screw fixation. The clinical mid-term results are characterized by substantial increase of hip motion and pain reduction. Surgical goal is to obtain a smaller head, well contained in the acetabulum. It should become as spherical as possible and the gliding surface should be covered with best available cartilage. Together, it has to be accomplished under careful consideration of the blood supply to the femoral head. In the majority of cases acetabular reorientation is necessary to optimize joint stability. Femoral head segment resections without guidance is difficult. Therefore, 3D-simulation for cut direction and segment size including the implementation of the resultant osteotomy configuration was developed using individually manufactured cutting jigs. First experience in five such cases have revealed good results. The forthcoming steps are the improvement of computer algorithm and automation. Goal is that with first cut decision the other cuts are automatically determined resulting in optimal head size and sphericity

1. The progressive radiographic changes in twenty-five patients suffering from Perthes' disease of the hip are described. 2. The prognostic value of lateral views of the hip is emphasised, and the present concepts of pathogenesis in the light of recent observations are discussed

We compared the prognostic value of the Catterall grouping, the Salter-Thompson grading, the arthrographic shape of the femoral head, and the Herring lateral pillar grouping during the fragmentation stage of Perthes’ disease in 73 patients with 81 affected hips. Radiographs were available for study from the onset of the disease until skeletal maturity. We used the Stulberg classification to assess outcome. The Herring grade and arthrographic sphericity proved to be the best predictors of final outcome. Combining these two values further increased the predictive value. All but one patient in Herring group A achieved an excellent outcome. In Herring group B, the age of the child and the sphericity of the femoral head influenced the end result. If the child was less than seven years old at the onset of symptoms the prognosis was invariably good and all spherical hips in group B had a good outcome with Stulberg grades 1 or 2. Moderately and severely deformed hips on arthrography resulted in Stulberg 3 and 4 hips. None of the hips in Herring group C had a normal appearance at maturity and the outcome was not significantly influenced by the age at onset or the arthrographic appearance

The pathogenesis of Perthes' disease has been related to increased intra-articular pressure secondary to a joint effusion. The pressure within the hip in different positions was measured in eight children with transient synovitis and four with the synovial stage of Perthes' disease. In the position of comfort for the hip this pressure was always less than the arteriolar blood pressure and in a supine position it did not exceed the systolic blood pressure. However, in extension with medial rotation the intra-articular pressure always became several times the systolic blood pressure. This high pressure was also recorded in the stable lateral position which occurs normally during sleep. It is concluded that in the presence of a synovial effusion in the hip, a position of extension and medial rotation causes an increase in intra-articular pressure which may compromise the blood supply to the capital epiphysis of the femur

Forty-eight images using magnetic resonance imaging (MRI) in 16 hips with Perthes' disease were evaluated over a mean period of two years. MRI depicted exactly the infarcted zone in the femoral head before typical radiological changes were evident. Early determination of the extent of the infarcted bone on MRI benefits those patients who require treatment. Follow-up MRI scans at six-monthly intervals, reflected the chronological stages of the repair process in each group classified according to Catterall

There are differences of opinion about the pathogenesis of Perthes' disease. All are agreed that it is due to ischaemia, but the cause of this and the size and number of infarctions are in dispute. Through the generosity of the contributors six whole femoral heads and core biopsies of five other cases have been studied radiographically and histologically. The findings ranged from an ischaemic arrest of ossification in the capital articular cartilage without infarction to multiple complete infarctions of the epiphysial bone. The ensuing reparative process contributes to the pathology, which is of a range to warrant grading or grouping

1. Eighty-nine cases of Perthes' disease are reviewed. 2. The prognosis varies with the amount of the epiphysis involved. 3. It is possible to assess the amount of epiphysial involvement by a study of the early radiographs. Cases were allocated to four groups on this basis. 4. It is confirmed that both sex and age at the time of diagnosis influence the final prognosis. The reasons for this are discussed. 5. The concept of "the head at risk" is suggested and radiological signs described to diagnose such cases. 6. It is hoped that the classification suggested may in future act as a basis for comparisons of treatment

Introduction: Perthes disease often leaves young adults with hip joint incongruency due to femoral head asphericity, (an extra-articular extrusion and a superior flattening). This causes femoroacetabular impingement, a reduced range of movement and early degenerative change. We report a novel method for restoration of femoral head sphericity and femoroacetabular congruency. Methods: 2 males (21 & 22 years) presented with groin pain and severe hip stiffness after childhood Perthes disease. Imaging confirmed characteristic saddle shaped deformities of the femoral head, with central depression and overlying cartilage loss. A new method of treatment was proposed. Both cases were treated in the same manner. Results: Using a surgical dislocation with the trochanteric flip osteotomy it was possible to remove the extra-articular bump to reduce femoroacetabular impingement. We found that the sphericity of the femoral head could be restored using a HemiCap partial resurfacing (Arthrosurface, MA, USA). The radius of the implant was selected to match that of the acetabulum. Restoration of the height of the flattened portion of the weight-bearing surface of the femoral head reduces abnormal loading of the acetabular articular cartilage by improving congruency of the joint. At a minimum of 3 year follow up both patients had sustained improved range of movement, pain and Oxford hip score. Repeated imaging shows no evidence of joint space narrowing or loosening at this stage. Conclusion: We conclude that this novel treatment functions well in the short term. Further studies are needed to confirm that after Perthe’s disease this treatment results in improved long term durability of the natural hip joint