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

The lateral pillar classification (LPC) is a widely used tool in determining prognosis and planning treatment in patients who are in the fragmentation stage of Perthes disease. The original classification has been modified to help increase the accuracy of the classification system by the Herring group. The purpose of our study was to independently assess this modified Herring classification.

35 standardized true antero-posterior radiographs of children in various stages of fragmentation were independently assessed by 6 senior observers on 2 separate occasions (6 weeks apart). Kappa analysis was used to assess the inter and intraobserver agreement between observations made. The degrees of agreement were as follows: poor, fair, moderate, good and very good.

Intraobserver analysis revealed at best only moderate agreement for two observers. 3 observers showed fair consistency, whilst 1 remaining observer showed poor consistency between repeated observations (p<0.01). The highest scores for interobserver agreement varying between moderate to good could only be established between 2 observers. For the remaining observers results were just fair (p<0.01).

This study highlights the lack of agreement between senior clinicians when applying the modified LPC. This has clinical implications when applying the classification to the decision making process in treating patients at risk of developing adverse outcomes from the disease. To our knowledge, this is the first time the modified LPC has been independently tested for its reproducibility by another specialist paediatric orthopaedic unit.

The purpose of our study was to independently assess the modified Herring lateral pillar classification.

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.

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.

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

Residual Legg-Calve-Perthes (LCP) deformities represent one of the most challenging disorders in hip reconstructive surgery. We assessed mid-term PRO. M. s, radiographic correction, complications and survivorship of combined surgical dislocation (SD) and periacetabular osteotomy (PAO) for the treatment of complex LCP deformities. A longitudinal cohort study was performed on 31 hips with complex LCP deformities undergoing combined SD/PAO. Treatment included femoral head reshaping, trochanteric advancement and relative neck lengthening, management of intra-articular lesions and PAO. Twenty-seven (87.1%) had minimum 5-year follow up. Average age was 19.8 years with 56% female and 44% having previous surgery. At a mean 8.4 years, 85% (23/27) of the hips remain preserved (no conversion to THA). The survivorship estimates at 5 and 10 years were 93% and 85%, respectively. The median and interquartile range for mHHS increased from 64 [55–67] to 92 [70–97] (p<0.001), the WOMAC-pain improved from 60 [45–75] to 86 [75–100] (p= 0.001). An additional 19% (n=5) reported symptoms (mHHS <70) at final follow-up. UCLA activity score increased from a median of 8 [6–10] to 9 [7–10] (p=0.207). Structural correction included average improvements of acetabular inclination 15.3. o. ± 7.6, LCEA 20.7° ± 10.8, ACEA 23.4° ± 16.3, and trochanteric height 18 mm ± 10 mm (all, p<0.001). Complications occurred in two (7%) patients including one deep and one superficial wound infection. At intermediate follow-up of combined SD/PAO for complex LCP deformities, 85% of hips are preserved. This procedure provides reliable deformity correction, major pain relief, improved function and acceptable complication/failure rates

Cartilage defects of the hip cause significant pain and may lead to arthritic changes that necessitate hip replacement. We propose the use of fresh osteochondral allografts as an option for the treatment of such defects in young patients. Here we present the results of fresh osteochondral allografts for cartilage defects in 17 patients in a prospective study. The underlying diagnoses for the cartilage defects were osteochondritis dissecans in eight and avascular necrosis in six. Two had Legg-Calve-Perthes and one a femoral head fracture. Pre-operatively, an MRI was used to determine the size of the cartilage defect and the femoral head diameter. All patients underwent surgical hip dislocation with a trochanteric slide osteotomy for placement of the allograft. The mean age at surgery was 25.9 years (17 to 44) and mean follow-up was 41.6 months (3 to 74). The mean Harris hip score was significantly better after surgery (p < 0.01) and 13 patients had fair to good outcomes. One patient required a repeat allograft, one patient underwent hip replacement and two patients are awaiting hip replacement. Fresh osteochondral allograft is a reasonable treatment option for hip cartilage defects in young patients. Cite this article: Bone Joint J 2014;96-B(11 Supple A):11–16

Introduction. A recent report based on the NARA database (Nordic Arthroplasty Register Association) found that the 10-year survivorship of patients under 50 with traditional total hip arthroplasty was only 83% in 14,600 cases. The purpose of this study was to compare our experience using metal-on-metal hip resurfacing arthroplasty (HRA) to treat these patients. Methods. from May 2001 to Feb 2012, a single surgeon performed 1029 metal-on-metal HRA in 855 patients younger than 50 years old. Three different implants were used in consecutive groups of patients, first the Corin hybrid HRA (182); then the Biomet hybrid HRA (306); and finally the Biomet uncemented HRA (541). The primary diagnoses were OA (707); dysplasia (125); osteonecrosis (98); post-trauma (28); Legg-Calve-Perthes (27) and others (44). The average age was 43±6 years; 74% were men; the average BMI was 27±4; mean femoral component size was 50±4 (range 40–62); the average T-score was 0±1. 37% of our patients reported a UCLA Activity level of 9 or 10 (impact sports). Six died with causes unrelated to their HRAs. The rate of follow-up was 94%. Our patients were not selected by any criteria except the surgeon's technical ability to perform an HRA. Results. There were a total 42 failures: acetabular component loosening in 15 cases (8 before two years) femoral component loosening in 9 cases; femoral neck fracture in 5 cases; adverse wear related failure (AWRF) in 4 cases; deep infection in 3 cases; recurrent dislocation in 1 case; other causes in 5 cases. With revision of any component as the end point, the Kaplan-Meier survivorship rate was 94.9% at 8 years and 92.5% at 10 years for the entire group. We compared survivorship between groups at shorter follow-up intervals to determine if results were improving. The survivorship rate at 8 years for Biomet Hybrid group was 95.8%, which was significantly better than 89.1% for the earlier Corin Hybrid group; the survivorship rate at 5 years for the latest Biomet Uncemented HRA group was 98.7%, also better than 96.5% for the prior Biomet Hybrid group. (P=0.0001). Conclusion. Metal-on-metal hip resurfacing has less than half the 10-year failure rate of THR in young patients. As HRA technology and experience improve, our data suggests that the results further improve. HRA currently meets the NICE criteria for 10-year survivorship of implants while THR doesn't in young patients. Our HRA patients are allowed unrestricted activity, while THR patients are typically restricted. AWRF (0.4%) is a minor problem that has been blown far out of proportion to its significance. Patient selection against HRA in certain subgroups (women, small bearings, history of metal allergy) may not be advisable based on our results

Introduction. Patients presenting with osteoarthritis as late sequelae following pediatric hip trauma have few options aside from standard Total Hip Replacement (THR). For younger more active patients, Hip Resurfacing Arthroplasty (HRA) can be offered as an alternative. HRA has been performed in the United States over the past decade and allows increased bone preservation, decreased hip dislocation rates versus THR, and potential to return to full activities. Patients presenting with end-stage hip arthritis as following prior pediatric trauma or disease often have altered hip morphology making HRA more complicated. Often Legg-Calve-Perthes (LCP) patients present with short, wide femoral necks, and femoral head distortion including coxa magna or coxa plana. There often can be acetabular dysplasia in conjunction with the proximal femoral abnormalities. Slipped Capital Femoral Epiphysis (SCFE) patients have an alteration of the femoral neck and head alignment, which can make reshaping the femoral head difficult. In particular, the femoral head is rotated medially and posteriorly, reducing the anterior and lateral offset. We present a cohort of 20 patients, with history of a childhood hip disorder (SCFE or LCP), who underwent HRA to treat end-stage arthritis. Fifty percent had prior pediatric surgical intervention at an average age of 11. Method. After Institutional Review Board approval, data was reviewed retrospectively on patients with pediatric hip diseases of SCFE and LCP who underwent HRA using the Birmingham Hip Resurfacing (BHR) by a single orthopaedic surgeon at a teaching institution. Harris Hip Scores (HHS), plain radiographs and blood metal ion levels were reviewed at routine intervals (12 months and annually thereafter). Those who had not returned for recent follow-up were contacted via telephone survey for a modified HHS. Results. Twenty patients had mean follow up of 2.8 years (range 1–7 years). Twelve had LCP and 8 SCFE. Median implant duration was 2.4 years. One-year metal ion testing revealed median chromium level of 2.3 ppb and median Cobalt level of 1.5 ppb. At one-year follow up, plain radiographs demonstrated all patient implants to be well-fixed, without radiolucent lines or osteolysis. Two patients at three and five-year follow-up exhibited heterotopic ossification. Mean HHS for LCP at 6 weeks post-operative was 88, and 98 at one year. Mean HHS for SCFE at 6 weeks post-operative was 77.5, and 98.6 at one year. LLD was significantly improved with an average pre-operative LLD of 12.6 mm and post op of 2.6 mm (p-value <0.001). At most recent follow-up, all retained their implants with overall average HHS of 98. Conclusion. At minimum of one-year following HRA, an increase in functional outcomes is found in patients who underwent HRA for osteoarthritis associated with LCP and SCFE with a mean HHS of 98. No increase was found in complications including femoral neck fracture or implant loosening despite technical challenges of the procedure related to proximal femoral morphologic abnormalities, or presence of acetabular dysplasia [Fig 1]

Introduction: In severe Legg-Calve-Perthes (LCPD) disease with subluxated femoral head, the acetabulum sometimes takes a bicompartmental appearance. This study analyzed acetabular pathoanatomy using a 3-D CT program. Materials and Methods: A 3-D CT software program that affords the section of 2-D image in any plane was used to analyze the acetabular pathoanatomy, with specific reference to the morphology of the inner surface of the acetabulum. Thirteen children with the bicompartmental acetabulae (12 LCPD and 1 AVN subsequent to septic hip arthritis) were evaluated. Results: The anterior half of the acetabulum was concentric. The contour of the acetabular margin in the posterior half of the acetabulum consisted of two different arcs – an arc of the iliac acetabulum (superior) and the other arc of the acetabular fossa of the ischium (medial). The junction of these two arcs was located at the triradiate cartilage, which was increased in mediolateral thickness at this point. The osteochondral articular margin of the ischium posterior to the non-articular acetabular fossa was thickened forming a ridge. The mediolateral thickness of the non-articular acetabular fossa was thinner than that of the normal contralateral side. The combination of these focal morphologic changes of the acetabular fossa rendered the bicompartmental appearance on plain AP radiograph of the pelvis. Discussion: The authors conclude that bicompartmentalization of the acetabulum apparently reflects the altered biomechanics of the hip joint due to the subluxated femoral head. The abnormal osteocartilaginous hypertrophy appears to be caused by synovial irritation and an eccentric molding effect of the subluxated femoral head

Legg-Calve-Perthes and slipped capital femoral epiphysis may result in alterations of the proximal femoral morphology, leading to the development of secondary hip osteoarthritis as a young adult. Hip surface arthroplasty presents special technical challenges in these patients because of the abnormal anatomy of the head and neck. We reviewed the radiographic and clinical results of patients with a history of either LCP or SCFE, who underwent hip resurfacing between 1996 and 2002. Twenty-three patients (25 hips, 14 with LCP and 11 with SCFE) underwent metal-on-metal surface arthroplasty. The average age at the time of surgery was 38.1 years and 87% of the patients were male. The mean time to follow-up was 26.9 months. UCLA pain, walking, function, and activity scores improved significantly in both groups as well as the SF-12 physical component scores. One patient with LCP required bilateral conversion to THA at 55 months postoperatively secondary to femoral component loosening. There were no dislocations or femoral neck fractures in this series. Of 11 patients who presented leg length discrepancies preoperatively, limb length was postoperatively equalized in six patients, 3 were maintained with the same amount of discrepancy, and 2 decreased their discrepancy by at least 1 cm. In Charnley Class A patients, there was no difference in femoral offset between the operated and the disease-free, contralateral hip after reconstruction. Despite the challenges in performing hip resurfacing in this patient population, the results to date have been encouraging. Even with the limitations of resurfacing in restoring leg length and offset, the clinical results of this group of patients are comparable to those of the other etiologies

Avascular necrosis (AVN) of the immature femoral head is the final common pathway of the Legg-Calve-Perthes (LCP) disease. Since cigarette smoking has been linked to the development of vascular disease, a study was performed to see if there was any association between parental smoking and LCP disease. The biological parents of 97 children with LCP disease were questioned on their smoking habits, which were compared to a control group of parents with unaffected children. Further comparison was made with respondents from the Perthes’ Association website who completed an on-line questionnaire. Parents were classified as being smokers or non-smokers on the basis of their smoking habit pre-pregnancy, during pregnancy and at the time of diagnosis of LCP being made. There was a higher proportion of children in the LCP group who had parents who smoke (N=67/97, 69%) compared to the control group (N=14/87, 16%). Further analysis showed that the highest rate occurred when both parents smoke before pregnancy (N=37/97, 38%) followed by when only the father smoked (N=23/97, 24 %). Maternal smoking alone appeared to have the least association (N=7/97, 7 %). In the control group the non-smoking rate was 58/87 [67%]. The changes in smoking patterns with respect to the pregnancy concerned were also noted. Fisher’s Exact test was used to determine any difference between the study group and the control group. There was a significant difference between the Perthes’ SE group concerned to the controls in all respects except maternal smoking. Comparison made with data obtained from the Perthes’ association website showed no difference between the two Perthes’ groups. Conclusion: These results support an association between parental smoking and the development of disease LCP disease

Introduction: The aim of this paper was to present a profile of Legg-Calve-Perthes’ (LCP) disease and test the hypothesis of an association between LCP disease and poverty. Methods: We examined demographic data on a group of 240 children (263 hips) presenting with LCP disease in Greater Glasgow, where the mean deprivation scores are substantially greater than in the rest of Scotland, to see if this association applies or whether other clues to the aetiology of LCP could be divined. Results: There were 197 males and 43 females. The majority presented in the sclerosis phase with much smaller numbers in the other phases. 70 % (184 cases of LCP) were Catterall grades 3 or 4. 16.25% had a family history of LCP. Bone age in our series is heavily skewed towards the lower centiles. The number of siblings in the family averaged 1.9, with 13 % being an only child. The maternal age at birth of the index child showed no preponderance to older age. Maternal smoking during and after pregnancy was noted in 55 %, which compares with 52% reported in the population of Greater Glasgow in general. Bone age in our series was heavily skewed towards the lower centiles. Birth weight showed a definite shift to the left, height a weaker shift to the left. 25 % of the children in our series are in social class IV and V, although this accounts for more than 50 % of the population of the Greater Glasgow. Discussion: There is no significant evidence of a preponderance of LCP disease in the most deprived groups (p=0.9). The aetiology of LCP disease is likely to be multifactorial and may include a genetic or deprivation influence causing low bone age, hyperactivity and a high pain threshold

Introduction: The aim of this paper was to test the hypothesis that for hips affected with Legg-Calve-Perthes’ (LCP) disease under the age of 8 years, surgery does not affect the outcome. Methods: We performed a retrospective paired study of patients, who were diagnosed with LCP disease before the age of 8 years, to compare the radiological results after treatment between conservatively and surgicallytreated 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, and stage at the first visit, Catterall and Herring grading and radiological at-risk signs. Each pair was assessed by comparing the values of five radiological measurements. Results: From a cohort of 345 hips diagnosed with LCP disease 14 pairs (28 hips) fitted the criteria. The radiological measurements, which showed a statistically better result in the surgical groups, were Mose’s method (p = 0.019), the Acetabular-Head Index (p = 0.034). There were no statistical differences in the Slope of the Acetabular Roof (p = 0.37), Articulotrochanteric distance (p = 0.17) and Stulberg grading (p = 0.2). 5 pairs had a better Stulberg result in the operative group. Three of these 5 pairs were less than 6.5 years at the time of their surgical procedure. Three pairs had a better Stulberg result in the conservative group. Six pairs had no difference between the groups. Discussion: We conclude that surgical treatment can improve the sphericity of the femoral head and provide greater acetabular cover than conservative treatment in hips of patients less than 8 years at the onset of LCP disease. However, the Stulberg grading was not affected. Our study supports the hypothesis that for hips affected with LCP disease under the age of 8 years, surgery does not affect the outcome

Introduction: There are several possibilities for the treatment of Legg-Calve-Perthes (LCP) disease in older age group (more than 10 years of age): varus femoral osteotomies, different pelvic osteotomies (Salter, Chiari, triple), and the combination of pelvic and femoral osteotomies (Salter with femoral shortening). Material and methods: We analyzed 214 hips with LCP disease surgically treated in our Institute in the period 1972–1999. Age of our patients ranged from 10–13 years. All of them were operated in the fragmentation phase of the disease. The distribution according to Catteral classification was: group II – 29 (13,5%), group III – 108 (50,5%), group IV – 77 (36%). Different risk factors were present in 154 (72%) cases. We performed: 69 (32,3%) varus femoral osteotomies, 32 (14,9%) Salter osteotomies, 69 (32,3%) Salter osteotomies with femoral shortening, 23 (10,7%) Chiariosteotomies and 21 (9,8%) triple pelvic osteotomies. There were no statistically significant differences between treatment groups (preoperatively). Postoperativelly hips were assessed clinically and radiologically. Follow-up period was in average 9,2 years (at least 3 years). Results: All procedures showed improvement in hip containment and functional status. The best anatomical results were in the group of patients treated by triple pelvic osteotomy (p=0,02), very good results were found in the groups of patients treated by varus femoral osteotomy and Salter osteotomy with femoral shortening, whereas the results of treatment in the other groups were slightly worse. Triple pelvic osteotomy and Salter osteotomy (with or without femoral shortening) showed the best functional recovery. Positive Trendelenburg sign and waddling gait were present only in the groups of patients treated by varus femoral osteotomy and Chiariosteotomy. Conclusion: Triple pelvic osteotomy is the best procedure for the treatment of LCP disease in the older age group