Can large doses of glucocorticoids lead to Perthes? a case report and review of the literature.

BACKGROUND: Perthes disease (Legg-Calvé-Perthes, LCP) is a self-limited and non-systemic disease occurring in the femoral heads of children, which is mainly manifested as an ischemic necrosis of the femoral head epiphysis, leading to subchondral ossification injury of the femoral head. CASE PRESENTATION: Here we report a case of 11-year-old child with long-term use of high-dose glucocorticoids. With MRI examination finding the epiphyseal necrosis of right humeral head, femur and tibia, and X-ray examination finding bilateral femoral head necrosis, the child was diagnosed as Perthes disease based on his clinical and imaging data. CONCLUSIONS: Long-term and high-dose glucocorticoids may be one of the causes of Perthes disease.

METHODS OF TREATMENT OF LEGG-СALVÉ-PERTHES DISEASE (REVIEW).

This Literature Review presents various treatments, including operative and conservative therapies, of Legg - Calvé - Perthes Disease. The problem is relevant because of the prevalence of the disease. The authors presented a review of literature, which managed to classify the main methods of treatment by the principles of action, practical application, and presented the interpretation of the effectiveness of modern research from the point of view of evidence-based medicine.

Determining Hinge Abduction in Legg-Calvé-Perthes Disease: Can We Reliably Make the Diagnosis?

BACKGROUND: Although hinge abduction is recognized as an important finding in children with Legg-Calvé-Perthes disease, variable diagnostic criteria exist. The purpose of this study was (1) to test the interobserver and intraobserver agreement of the current definition of hinge abduction and (2) to develop consensus regarding key diagnostic features that could be used to improve our diagnostic criteria. METHODS: Four orthopaedic surgeons with subspecialty pediatric hip interest independently assessed 30 randomly ordered cases of Legg-Calvé-Perthes disease. Each case included 2 fluoroscopic images of hip arthrograms (anteroposterior and abduction views). Surgeons graded the cases in a binary manner (hinge/no-hinge) on 2 separate occasions separated by a 4-week interval. Following reliability testing and comprehensive review of the literature, consensus-building sessions were conducted to identify key diagnostic features. Surgeons then regraded a new series of cases. Interobserver and intraobserver agreement between first/second and third/fourth readings were assessed using the Fleiss κ. RESULTS: Interobserver κ for hinge abduction between the first and second surveys was 0.52 (with 0.41 to 0.60 considered moderate agreement), compared with 0.56 for the third and fourth surveys. First and second reading intraobserver agreement ranged from 0.59 to 0.83 compared with 0.75 to 1.00 for third and fourth reading. Consensus sessions identified several key diagnostic factors including: adequate visualization of the labral contour and ability of the lateral epiphysis to slip below the chondrolabral complex in abduction. Medial dye pooling, often due to asphericity of the femoral head, was not found to be a useful diagnostic criterion. CONCLUSIONS: Despite a combined experience of over 70 years among the reviewers, we found just slightly better than 50:50 agreement in what constitutes hinge abduction. Consensus discussions did improve our agreement but these modest changes emphasize how difficult it is to develop reliable diagnostic criteria for hinge abduction. As a result, we caution against using hinge abduction as an inclusion criteria or outcome measure for research purposes, as the diagnostic agreement can be inconsistent. LEVEL OF EVIDENCE: Level III-diagnostic study.

[Perthes disease-news in diagnostics and treatment]. / Morbus Perthes ­ Neuigkeiten in der Diagnostik und Behandlung.

DIAGNOSTICS: Perthes disease remains a challenge for paediatric orthopedic surgeons. X­ray imaging is still the method of choice for diagnostics and follow-up examination. A more detailed differentiation of Waldenström's classification, especially in early and late fragmentation stages, might be relevant to optimize timing of containment surgery. So-called "advanced MRI" imaging might help to detect patients at risk earlier than conventional x­ray imaging, which could lead to earlier surgical intervention. TREATMENT: Currently there is no treatment modality available which would improve the bone changes in Perthes disease. Non-operative treatment like improving hip range of motion as well as unloading is still the basic standard of care, with analgesic and/or anti-inflammatory medication, according to symptoms or findings. In the case of loss of containment, especially in children older than 6 years, surgery is indicated. Currently, there is a trend favoring acetabular reorientation techniques-especially the triple osteotomy, since the biomechanical relations would not be additionally impaired as in case of femoral varus osteotomy.

Intra- and inter-observer reliability of Laredo classification system in Legg-Calvé-Perthes Disease.

The purpose of the current study was to investigate intra- and inter-observer reliability of arthrographic Laredo classification system in Perthes disease. Forty-seven patients were included in this cross-sectional descriptive study. Patients' age, gender, physical findings and hip arthrographs were collected from hospital medical records. Two different sets of power point slides were prepared in which the order of cases was randomized and blinded. Observers were divided into three groups according to their surgical experience (9 residents, 10 seniors, 10 pediatric orthopedists), and they assessed two times 1 month's intervals. Statistical analysis was performed by using the SPSS v21. Inter- and intra-observer reliabilities were calculated using intra-class correlation coefficient, weighted kappa and percentage agreement. Percentage agreement of Laredo classification was about 50% for all groups (residents, seniors and pediatric orthopedists); intra-observer reliabilities were excellent, excellent and fair, respectively. Inter-observer reliability of Laredo classification for each set was found to be excellent for all groups. Length of experience did not correlated significantly with the level of intra-observer agreement. As a conclusion, our results showed that Laredo's arthrographic classification system's intra-observer reliability is at least at a fair level and inter-observer reliability is at an excellent level. We believe that this classification system is valuable for an orthopedic surgeon who deals with the treatment of Perthes disease.

Perthes disease: A new finding in Floating-Harbor syndrome.

Floating-Harbor Syndrome (FHS; OMIM #136140) is an ultra-rare autosomal dominant genetic condition characterized by expressive language delay, short stature with delayed bone mineralization, a triangular face with a prominent nose, and deep-set eyes, and hand anomalies. First reported in 1973, FHS is associated with mutations in the SRCAP gene, which encodes SNF2-related CREBBP activator protein. Mutations in the CREBBP gene cause Rubinstein-Taybi Syndrome (RSTS; OMIM #180849, #613684), another rare disease characterized by broad thumbs and halluces, facial dysmorphisms, short stature, and intellectual disability, which has a phenotypic overlap with FHS. We describe a case of FHS associated with a novel SRCAP mutation and characterized by Perthes disease, a skeletal anomaly described in approximately 3% of patients with RSTS. Thus Perthes disease can be added to the list of clinical features that overlap between FHS and RSTS.

Is Legg-Calvé-Perthes Disease a Local Manifestation of a Systemic Condition?

BACKGROUND: Osteochondrosis includes numerous diseases that occur during rapid growth, characterized by disturbances of endochondral ossification. One example, Legg-Calvé-Perthes disease, is characterized by disruption of the blood supply to the femoral head epiphysis, and a systemic etiology often has been suggested. If this were the case, secondary osteochondroses at locations other than the hip might be expected to be more common among patients with Legg-Calvé-Perthes disease, but to our knowledge, this has not been evaluated in a nationwide sample. QUESTIONS/PURPOSES: (1) Do patients with Legg-Calvé-Perthes disease have an increased prevalence of secondary osteochondroses at locations other than the hip? (2) Is the concept of Legg-Calvé-Perthes disease a systemic etiology supported by a higher prevalence of the metabolic diseases obesity and hypothyroidism? METHODS: We designed a retrospective population-based cohort study with data derived from the Swedish Patient Registry (SPR). The SPR was established in 1964 and collects information on dates of hospital admission and discharge, registered diagnoses (categorized along the International Classification of Diseases [ICD]), and applied treatments during the entire lifetime of all Swedish citizens with high validity. Analyzing the time span from 1964 to 2011, we identified 3183 patients with an ICD code indicative of Legg-Calvé-Perthes disease and additionally sampled 10 control individuals per patient with Legg-Calvé-Perthes disease, matching for sex, age, and residence, resulting in 31,817 control individuals. The prevalence of secondary osteochondroses, obesity, and hypothyroidism was calculated separately for patients with Legg-Calvé-Perthes disease and control individuals based on the presence of ICD codes indicative of these conditions. Using logistic regression analysis, we compared the adjusted relative risk of having either of these conditions develop between patients with Legg-Calvé-Perthes disease and their matched control subjects. The mean followup was 26.1 years (range, 2.8-65 years). RESULTS: The prevalence of secondary osteochondroses was greater among patients with Legg-Calvé-Perthes disease (3.11%) than among control subjects (0.31%), resulting in an increased adjusted risk of an association with such lesions in the patients (relative risk [RR], 10.3; 95% confidence interval [CI], 7.7-13.6; p < 0.001). When stratified by sex, we attained a similarly increased risk ratio for females (RR, 12.5; 95% CI, 6.1-25.8; p < 0.001) as for males (RR, 9.9; 95% CI, 7.3-13.5; p < 0.001). Patients with Legg-Calvé-Perthes disease had an increased adjusted risk of an association with obesity (RR, 2.8; 95% CI, 1.9-4.0; p < 0.001) or hypothyroidism (RR, 2.6; 95% CI, 1.7-3.8; p < 0.001) when compared with control subjects. CONCLUSIONS: To our knowledge, this is the first population-based description of a robust association of Legg-Calvé-Perthes disease with osteochondroses at locations other than the hip, and we also found increased risk estimates for an association with obesity and hypothyroidism in patients with Legg-Calvé-Perthes disease. Our findings strengthen the hypothesis that Legg-Calvé-Perthes disease is the local manifestation of a systemic disease, indicative of an underlying common disease pathway that requires further investigation. Physicians should be aware that patients with Legg-Calvé-Perthes disease may present with secondary osteochondroses and metabolic comorbidities. LEVEL OF EVIDENCE: Level III, prognostic study.

[Perthes disease-diagnosis, classification and treatment based on Aachen-Dortmund treatment algorithm]. / M. Perthes ­ Diagnostik, Klassifikation und Therapie anhand des Aachen-Dortmunder Therapiealgorithmus.

Legg-Calvé-Perthes disease is a multifactorial idiopathic necrosis of the hip that typically occurs in childhood between the ages of 3 and 12. Treatment adapted to the stadium of the disease and to the clinical findings is medical art. The treatment is focused on the preservation or recovery of the arthrogenic containment of the femoral head. Multiple treatment options are available. The indications for treatment can be derived from clinical and radiological pathology. Structuring of the therapy options is the aim of this publication. For this purpose, a clear and concise overview of relevant clinical findings and useful radiographic classifications as well as reasonable therapy is presented.

Pediatric Orthopaedic Consults From Chiropractic Care.

Alternative medicine in pediatrics is expanding, with chiropractic now a common choice for families seeking alternative medical care. Currently, there is sparse information in the literature exploring the role of chiropractic in orthopaedic pathology. The objective of this case series is to present pediatric patients who received treatment from chiropractors and orthopaedic physicians as well as to review the respective existing research. Data collected included chiropractic diagnosis, orthopaedic diagnosis, imaging studies, treatments, and complications. Twenty-three patients were studied. Scoliosis, Legg-Calvé-Perthes disease, developmental dysplasia of the hip, cerebral palsy, skeletal dysplasia, and slipped capital femoral epiphysis were diagnoses included. Children had multiple sessions of chiropractic for management of these conditions. The parents' perception for chiropractic was positive in every case. Delayed referral, misdiagnosis, adverse events from manipulative therapy, and ineffective treatments were observed in the present study. More research is indicated to validate chiropractic in children with orthopaedic pathology. (Journal of Surgical Orthopaedic Advances 27(1):58-63, 2018).

Common paediatric conditions of the lower limb.

Growing children are susceptible to a number of disorders to their lower extremities of varying degrees of severity. The diagnosis and management of these conditions can be challenging. With musculoskeletal symptoms being one of the leading reasons for visits to general practitioners, a working knowledge of the basics of these disorders can help in the appropriate diagnosis, treatment, counselling, and specialist referral. This review covers common disorders affecting the hip, the knee and the foot. The aim is to assist general practitioners in recognising developmental norms and differentiating physiological from pathological conditions and to identify when a specialist referral is necessary.

Radiographic classifications in Perthes disease.

Background and purpose - Different radiographic classifications have been proposed for prediction of outcome in Perthes disease. We assessed whether the modified lateral pillar classification would provide more reliable interobserver agreement and prognostic value compared with the original lateral pillar classification and the Catterall classification. Patients and methods - 42 patients (38 boys) with Perthes disease were included in the interobserver study. Their mean age at diagnosis was 6.5 (3-11) years. 5 observers classified the radiographs in 2 separate sessions according to the Catterall classification, the original and the modified lateral pillar classifications. Interobserver agreement was analysed using weighted kappa statistics. We assessed the associations between the classifications and femoral head sphericity at 5-year follow-up in 37 non-operatively treated patients in a crosstable analysis (Gamma statistics for ordinal variables, γ). Results - The original lateral pillar and Catterall classifications showed moderate interobserver agreement (kappa 0.49 and 0.43, respectively) while the modified lateral pillar classification had fair agreement (kappa 0.40). The original lateral pillar classification was strongly associated with the 5-year radiographic outcome, with a mean γ correlation coefficient of 0.75 (95% CI: 0.61-0.95) among the 5 observers. The modified lateral pillar and Catterall classifications showed moderate associations (mean γ correlation coefficient 0.55 [95% CI: 0.38-0.66] and 0.64 [95% CI: 0.57-0.72], respectively). Interpretation - The Catterall classification and the original lateral pillar classification had sufficient interobserver agreement and association to late radiographic outcome to be suitable for clinical use. Adding the borderline B/C group did not increase the interobserver agreement or prognostic value of the original lateral pillar classification.

Diffusion-weighted magnetic resonance imaging of femoral head osteonecrosis in two groups of patients: Legg-Perthes-Calve and Avascular necrosis.

INTRODUCTION: The aim of this prospective study was to evaluate the value of diffusion-weighted magnetic resonance imaging (DW-MRI) in patients with osteonecrosis. Patients were divided into two subgroups as avascular necrosis (AVN) of femoral head for adult group and Legg-Calvé-Perthes (LCP) patients for children. PATIENTS AND METHODS: Seventeen patients with femoral head AVN (mean age 42.3 years) and 17 patients with LCP (mean age 8.2 years) were included in this study. Diagnosis confirmed with clinical and other imaging procedures among the patients complaining hip pain. DW images were obtained using the single-shot echo planar sequence and had b values of 0, 500, 1000 s/mm(2). The apparent diffusion coefficient (ADC) values were measured from ADC maps in epiphysis of patients with AVN, both from metaphysis and epiphysis in patients with LCP, respectively. Mann-Whitney U test was used to compare ADC values. RESULTS: The mean ADC value of femoral heads (1.285 ± 0.204 × 10(-3) mm(2)/s) was increased in patients with AVN when compared to normal bone tissue (0.209 ± 0.214 × 10(-3) mm(2)/s) (p < 0.01). The mean ADC values (×10(-3) mm(2)/s) of both metaphysis (0.852 ± 0.293) and epiphysis (0.843 ± 0.332) were also increased in patients with LCP and differences were statistically significant (p < 0.01). CONCLUSIONS: As a result, osteonecrosis shows increased ADC values. But it is a controversial concept that DWI offers a valuable data to conventional MRI or not. However, as there are report states, there is a correlation between the stage of the disease with ADC values in the LCP disease. DWI is a fast, without-contrast administration technique and provides quantitative values additional to conventional MR techniques; we believe DWI may play an additional assistance to the diagnosis and treatment for LCP patients. Multicentric larger group studies may provide additional data to this issue.

Evaluating the Limping Child: A Rheumatology Perspective.

Children often present to health care providers for evaluation of limp. Having the knowledge of the different causes of leg pains both in the acute and chronic settings will help in diagnosis, treatment, and referrals to subspecialists in a timely manner. Taking a detailed history and completing a thorough evaluation will help hone in on the underlying cause. This article will review important causes of limp from the rheumatologist's viewpoint.

Trichorhinophalangeal syndrome type I: a novel mutation and Perthes-like changes of the hip in a family with 4 cases over 3 generations.

The trichorhinophalangeal syndrome is a rare genetic syndrome with characteristic craniofacial and skeletal abnormalities including hip pathology in variable manifestation. We describe hip involvement with Perthes-like changes and a novel mutation of the TRPSI gene in a family with 4 affected individuals. This case series underlines the clinical significance of rare genetic disorders such as TRPS that among other differential diagnoses should be kept in mind when children present with Perthes-like changes of the hip joint.

Shelf acetabuloplasty for containment in the early stages of Legg-Calve-Perthes disease.

BACKGROUND: To evaluate the effectiveness of shelf acetabuloplasty in the containment of extruded hips without hinge abduction in early stages of Legg-Calve-Perthes disease, we present a retrospective series of 44 patients (45 hips) treated between August 1999 and February 2010, which included 34 boys and 10 girls with a mean age at diagnosis of 7.4 years (range, 3.9 to 15.3). METHODS: All patients presented with sclerosis or early fragmentation stages. The average time from diagnosis to surgery was 2.1 months (range, 0 to 8.2) and the mean time to heal was 40.4 months (range, 20 to 82.2). The Reimer migration and the deformity indices were measured on initial, preoperative, postoperative, and healed x-rays. The average deformity index at 3 of those 4 timepoints was significantly related to their final Stulberg classification. CE angles increased and Sharp angles decreased significantly as a result of treatment. RESULTS: At the healed stage and consistent with other published series, 84.4% of patients were Stulberg III or less, denying any pain, and with full range of movement, whereas 15.6% were classified as Stulberg IV. CONCLUSIONS: We defend that shelf acetabuloplasty should be performed early in the disease and, uniquely, we propose that the indication for treatment should be guided by the deformity and the Reimer migration indices. LEVEL OF EVIDENCE: IV.

Outcome of patients with Legg-Calvé-Perthes onset before 6 years of age.

BACKGROUND: The prognosis of Legg-Calvé-Perthes disease (LCPD) in young patients has been accepted as favorable. The purpose of this study was to clarify the outcome of LCPD patients with onset before 6 years of age. METHODS: From 1989 to 2007, of 332 LCPD patients, 114 hips (in 100 patients) were diagnosed before 6 years of age (mean age, 4.5 y old) with subsequent repair of the epiphysis in all cases. Waldenström classification at presentation was initial stage in 76 hips and fragmentation stage in 38 hips. Lateral pillar classification was group A in 17 hips, group B in 22 hips, group B/C in 24 hips, and group C in 51 hips. Treatment methods were observation with restriction of activity alone in 42 hips and several containment treatments in 72 hips. RESULTS: At the mean age of 14, Stulberg classification was class I in 26 hips, II in 46 hips, III in 28 hips, and IV in 14 hips. These data show an acceptable outcome in 72 of 114 hips (63%). Logistic regression analysis revealed that lateral pillar classification (odds ratio, 3.6) and good range of abduction without treatment (odds ratio, 4.0) were prognostic factors. CONCLUSIONS: Poor outcome was observed even in patients before 6 years of age with large necrotic area. Lateral pillar classification and good range of abduction were prognostic factors. LEVEL OF EVIDENCE: Level IV. Therapeutic studies-investigating the results of treatment. Case series.

Feasibility and safety of perfusion MRI for Legg-Calvé-Perthes disease.

BACKGROUND: Recent studies have suggested that perfusion magnetic resonance imaging (pMRI) using gadolinium contrast and a subtraction technique can provide useful prognostic information in Legg-Calvé-Perthes disease (LCPD) and allow earlier stratification for outcome. There are, however, sparse data available regarding the feasibility and safety of these studies in children. The purpose of this study was to collect this information across multiple centers using pMRI for LCPD. METHODS: We retrospectively reviewed a consecutive series of patients with confirmed or suspected LCPD who had undergone pMRI at 1 of 5 large tertiary-care children's hospitals in the United States, UK, and Mexico. Demographic information, type of contrast administered, and requirement for sedation or anesthesia were noted. Records were scrutinized for adverse events associated with the pMRI protocol. RESULTS: Over the study period, 165 patients underwent 298 pMRI studies. The median age at the time of imaging was 8.6 years (range, 2.5to 16.9 y). A total of 252 scans (85%) were performed for a known diagnosis of LCPD, whereas 46 were performed for a suspected diagnosis. Ninety-two of the 298 (31%) pMRIs required sedation, 48 (16%) required general anesthesia, and 122 (41%) were facilitated by video goggles only. The remaining 36 patients (12%) had their studies performed without additional measures. The ages of patients requiring sedation (mean, 7.2±2.4 y) and anesthesia (mean, 7.7±2.3 y) were significantly younger than those patients requiring neither (mean, 10.2±2.3 y, P<0.001). Four patients (1.3%) reported nausea or vomiting as a result of sedation. Two patients (0.7%) had complications from intravenous cannulation (pull out, difficult access). One child (0.3%) had nausea/vomiting as a result of contrast administration. There were no serious adverse events as a result of the pMRI protocol; specifically none of nephrogenic systemic fibrosis, anaphylaxis, or death. CONCLUSIONS: pMRI is a safe and feasible imaging technique for LCPD. Almost half of our patients required either sedation or general anesthesia to complete the study. LEVEL OF EVIDENCE: IV (case series).

Pediatric hip: pearls and pitfalls.

Pediatric hip pathology varies depending on patient age. Newborns are often screened for developmental dysplasia of the hip, whereas toddlers and older children can present later with pain, leg length discrepancy, or limp. Young children may have hip pain related to transient synovitis, septic arthritis, or Legg-Calvé-Perthes disease. Older children are more likely to suffer from slipped capital femoral epiphysis or apophyseal avulsion fractures. Knowledge about how the growing skeleton differs from adults as well as the classic imaging findings in many of these diagnoses is paramount when taking care of pediatric patients with suspected hip pathology.

Can residual leg shortening be predicted in patients with Legg-Calvé-Perthes' disease?

BACKGROUND: Although Legg-Calvé-Perthes' disease (LCPD) is frequently associated with varying degrees of femoral head deformity and leg length discrepancy (LLD), no factors that predict residual shortening have been clearly identified. QUESTIONS/PURPOSES: We attempted to determine whether (1) the extent of femoral head involvement; (2) varus osteotomy; and (3) patient demographic characteristics are associated with LLD at skeletal maturity in patients with LCPD. METHODS: We retrospectively reviewed the records of 168 skeletally mature patients with unilateral LCPD. The mean age at diagnosis was 7 years (range, 2-14 years). The extent of femoral head involvement was determined from the initial radiographs using the Herring lateral pillar and Catterall classifications. LLD was defined as shortening by ≥ 1.0 cm as measured from scanograms. The patient's sex and the treatment modalities used were also recorded. RESULTS: LLD ranging from 10 to 38 mm (mean, 19 mm) occurred in 93 (55%) patients and was associated with the extent of femoral head involvement. Varus osteotomy was not associated with residual shortening. The patient's age at diagnosis did not affect the LLD at skeletal maturity. The strongest predictor of LLD was a lateral pillar classification of B/C or C (odds ratio, 3.5; 95% confidence interval, 1.39-8.79). CONCLUSIONS: The extent of femoral head involvement, but not the patient's age at diagnosis or sex or the treatment modality, can predict the LLD at skeletal maturity in patients with unilateral LCPD.