Recommendations for physiotherapy and physical activity for children with Legg-Calvé-Perthes disease: a survey of pediatric orthopedic surgeons and physiotherapists in Sweden.

BACKGROUND AND PURPOSE: Physiotherapy, restrictions of physical activity, and weightbearing are part of the treatment of children with Legg-Calvé-Perthes disease (LCPD). Prescription practices are widely discussed and vary between pediatric orthopedic surgeons (POSs) and physiotherapists (PTs). The purpose of this study was to identify recommendations for treatment methods in clinical practice to find some consensus and elaborate guidelines. PATIENTS AND METHODS: A web-based questionnaire including 3 cases of LCPD (initial, fragmentation, and reossification stages) was answered by 25 POSs and 19 PTs. They were asked to describe their preferred recommendations for physiotherapy, including stretching, strengthening, weightbearing, and physical activities in relation to, e.g., range of motion (ROM) pain, sex, and disease stage. RESULTS: ROM was considered to be important when recommending physiotherapy; PTs also recognized pain and disease stage. Sex was reported as a factor with low importance. Stretching exercises were recommended for all disease stages. Recommendations for strengthening exercises varied for the initial and fragmentation stages. None of the participants recommended total non-weightbearing. Most restricted trampolining, running, ball sports, and gymnastics in the first 2 stages of the disease and allowed swimming, short walks, cycling, and horse riding without restrictions for all stages. CONCLUSION: We found high agreement on recommending stretching exercises for all disease stages, but controversies regarding recommendations for strengthening exercises in the initial and fragmentation stages. No non-weightbearing treatment for the affected hip was recommended by any participants at any stage of the disease. There was no clear consensus regarding the appropriate timeline for resuming full activities.

Epidemiology and treatment of pediatric tibial fractures in Sweden: a nationwide population-based study on 5828 fractures from the Swedish Fracture Register.

PURPOSE: Pediatric tibial fractures have been described internationally as mainly caused by fall during leisure activities and organized sports and showing a higher incidence in boys. Still, most studies are single center studies or have a small sample size. This study aimed to analyze sex and age distribution, seasonal variation, injury mechanisms and treatment of pediatric tibial fractures based on the nationwide Swedish Fracture Register (SFR). METHODS: All tibial fractures in patients < 16 years at injury and registered in 2015-2019 were extracted from the SFR. We analyzed patient characteristics such as sex and age, injury mechanism, fracture location and treatment. RESULTS: The study cohort consisted of 5828 pediatric tibial fractures in 5719 patients. Median age of the patients was 7 years and 58% were boys. Shaft fractures were most common, followed by the distal and proximal tibia. The lowest incidence was observed during autumn. The most common cause of injury was fall mostly involving winter sports, stumbles and fall from play equipment. Play/free time and sports were the most common activities, common places of injury were sports facility and home. 1% were open fractures. 78% were treated non-surgically. Screw fixation was performed in 52% of surgically treated fractures, predominantly in the distal segment. CONCLUSION: Injury mechanism differs between age groups; play/free time injuries are common in younger children compared with sport activities in older children. Most patients are treated non-surgically. Open fractures are rare. Information on injury patterns is useful working preventively, for example safety work in playgrounds.

Do Weightbearing Restrictions Cause Excess Weight Gain in Children With LEGG-Calvé-Perthes Disease?

BACKGROUND: Restricted weight bearing is commonly prescribed in Legg-Calvé-Perthes Disease (LCPD), raising concerns of causing overweight or obesity. This study utilizes prospectively collected data to address the following questions: (1) does body mass index (BMI) Z-score increase over the course of LCPD follow-up; (2) is having a BMI category of normal, overweight, or obese at baseline associated with BMI Z-score changes over the course of follow-up; and (3) is the duration of weight bearing restrictions (no restrictions, <3, 3 to <6, 6 to 9, or >9 mo) associated with BMI Z-score changes. METHODS: Data of 130 children aged 5 to 12 years with unilateral early-stage LCPD were extracted from an international database. Nation-specific BMI Z-scores and percentile-based weight categories were determined, and the duration of follow-up and weight bearing restrictions were calculated. Longitudinal changes in BMI Z-scores were evaluated for the 3 study questions using mixed effects linear regression models with surgery as a covariate. Sensitivity analyses were used to determine the influence of socio-cultural background (USA vs. India) for each study question. RESULTS: During the 35.5±15.9 months of follow-up, no statistically significant increase in BMI Z-scores was observed across the entire cohort, or following stratification by baseline weight categories or the duration of the weight bearing restriction. Sensitivity analyses indicated that patients in the USA had no change in their BMI Z-score. When stratified by weight categories, the normal weight of US children had a small increase in their BMI Z-score (0.005 per mo, 95% confidence interval: 0.0002, 0.009), but this was not seen in other BMI categories. The cohort of Indian children had a small but significant decrease in their BMI Z-score (-0.005/mo, 95% CI: -0.009, -0.0002). After stratification by weight categories, a small decrease of the BMI Z-score was observed only in the Indian overweight children (-0.016 per mo, 95% CI: -0.027, -0.005) and no other BMI category. CONCLUSIONS: Weightbearing restrictions over the course of follow-up for our cohort of children with early-stage LCPD were not associated with clinically meaningful increases of BMI Z-scores. Weight gain is multi-factorial and probably not caused by weight bearing restrictions alone. LEVEL OF EVIDENCE: III Diagnostic Study.

Pediatric Hip Disease Increases the Risk for Opioid Use in Adulthood: Long-term Burden of Pain and Depression.

BACKGROUND: Legg-Calvé-Perthes disease (LCPD) and slipped capital femoral epiphysis (SCFE) can result in painful deformation of the hip joint with impaired range of motion and early development of secondary osteoarthritis. It has not been investigated whether having LCPD or SCFE is associated with increased use of pain or antidepressant drug prescriptions later in life. OBJECTIVE: With this study, we aimed to investigate if patients with a history of LCPD or SCFE have an increased risk of prescription analgesic or antidepressant drugs in adulthood compared with matched controls. STUDY DESIGN: The included patients were identified by the Swedish Patient Register and matched for age, gender, and residency with 10 control individuals not exposed to any of the mentioned pediatric hip diseases, by the Swedish National Population Register. SETTING: This was a nationwide, registry-based cohort study which included 1,292 patients diagnosed with LCPD at age 2-15 years and 1,613 patients diagnosed with SCFE at age 5-16 years and > 17 years from 2005 through 2011. METHODS: Prescription data of first-line analgesic drugs (acetaminophen, nonsteroidal anti-inflammatory drugs, and opioids), or first-line antidepressant drugs (selective serotonin reuptake inhibitors, serotonin-norepinephrine reuptake inhibitors, and tricyclic antidepressants) were derived from the Swedish Prescribed Drugs Register. Conditional logistic regression models were fitted to estimate the relative risk for the prescription in exposed compared with unexposed individuals. Adjustment was performed for gender and birth year. RESULTS: In the group with an LCPD diagnosis, the adjusted odds ratio for analgesic prescriptions overall was 1.3 (95% CI, 1.2-1.5). For patients with an SCFE diagnosis, the adjusted odds ratio for analgesic prescriptions overall was 1.4 (95% CI, 1.3-1.6). Among patients with an LCPD diagnosis, the adjusted odds ratio for antidepressant prescriptions overall was 1.0 (95% CI, 0.8-1.2). For patients with an SCFE diagnosis, the adjusted odds ratio was 1.2 (95% CI, 1.1-1.4). LIMITATIONS: As with all register studies, there are known associated biases such as selection, detection, and observational bias as well as the uncertain quality of input data. Further, the Swedish Prescribed Drugs Register only includes drugs that were prescribed by a physician and dispensed at a pharmacy. This is also a factor that may lead to underestimating the use of acetaminophen and nonsteroidal anti-inflammatory drugs, as these drugs can be acquired "over the counter." CONCLUSION: During childhood, patients with LCPD or SCFE seem to suffer long-term pain and have an increased risk of requiring analgesic medication in adulthood, including opioids. It is important to assess the causes, type, and severity of pain to optimize pain management to counteract possible overuse in these patients. Seemingly, patients with LCPD do not have an increased risk for antidepressant drug therapy in adulthood whereas we did see an increased risk for that in patients with previous SCFE compared with the general population.

Similar risk of cancer in patients younger than 55 years with or without a total hip arthroplasty (THA): a population- based cohort study on 18,771 exposed to THA and 87,683 controls.

BACKGROUND AND PURPOSE: Concerns related to a potentially increased risk of cancer after total hip arthroplasty (THA) have frequently surfaced, especially since the novel EU medical device regulation classified cobalt as carcinogenic. We assessed the risk of cancer after THA in a nationwide cohort of patients younger than 55 years at surgery. PATIENTS AND METHODS: In this population-based longitudinal cohort study, 18,771 individuals exposed to THA were identified in the Swedish Hip Arthroplasty Registry (SHAR) and compared with 87,683 unexposed individuals who were matched by age, sex, and residence. Diagnoses, socioeconomic background, and dates of death were obtained from the Swedish Cancer Register, the National Patient Register, and Statistics Sweden. Primary outcome was the adjusted risk of any cancer after the first THA; secondary outcomes were specific cancer forms. RESULTS: We found no enhanced adjusted risk of developing any cancer, either in exposed females compared with unexposed females (hazard ratio [HR] 1.1, 95% confidence interval [CI] 0.95-1.2), or in exposed males (HR 1.1, CI 0.99-1.2). When analysing specific cancers, increased adjusted risks were found for thyroid and pancreas cancer in exposed females, and for cancer of the stomach, skin melanoma, and prostate cancer in exposed males. INTERPRETATION: This study indicates that there is no statistically significant increased overall risk of cancer in young THA-exposed patients. The potentially slightly enhanced risk for specific cancers may be due to residual confounding resulting from risk factors not accounted for and merits further investigation.

Epidemiology of proximal and diaphyseal humeral fractures in children: an observational study from the Swedish Fracture Register.

BACKGROUND: Most fractures in children are fractures of the upper extremity. Proximal and diaphyseal humeral fractures account for a minority of these fractures. To our knowledge, few previous reports address these fractures. This study aimed to describe the epidemiology and current treatment of proximal and diaphyseal humeral fractures by using the Swedish Fracture Register (SFR). METHODS: In this nationwide observational study from the SFR we analysed data on patient characteristics, injury mechanism, fracture classification and treatment. We included patients aged < 16 years at time of injury with proximal or diaphyseal humeral fracture registered in 2015-2019. RESULTS: 1996 (1696 proximal and 300 diaphyseal) fractures were registered. Proximal fractures were more frequent in girls whereas diaphyseal fractures were more frequent in boys. The median age at fracture was 10 years in both fracture types but patient's age was more widespread in diaphyseal fracture (IQR 5-13 compared to IQR 7-12 in proximal). In both sexes, the most registered injury mechanism was fall. Horse-riding was a common mechanism of injury in girls, whereas ice-skating and skiing were common mechanisms in boys. Most proximal fractures were metaphyseal fractures. Most diaphyseal fractures were simple transverse or oblique/spiral fractures. The majority of fractures were treated non-surgically (92% of proximal and 80% of diaphyseal fractures). The treatment method was not associated with the patient's sex. Surgery was more often performed in adolescents. The most common surgical methods were K-wire and cerclage fixation in proximal fracture and intramedullary nailing in diaphyseal fracture. CONCLUSION: Following falls, we found sex-specific sport activities to cause most proximal and diaphyseal paediatric fractures. Further studies on prophylactic efforts in these activities are needed to investigate whether these fractures are preventable. The majority of the fractures were treated non-surgically, although surgical treatment increased with increasing age in both sexes. TRIAL REGISTRATION: Not applicable. The present study is a register-based cohort study. No health care intervention had been undertaken.

No increased mortality after total hip arthroplasty in patients with a history of pediatric hip disease: a matched, population-based cohort study on 4,043 patients.

Background and purpose - Patients with pediatric hip diseases are more comorbid than the general population and at risk of premature, secondary osteoarthritis, often leading to total hip arthroplasty (THA). We investigated whether THA confers an increased mortality in this cohort.Patients and methods - We identified 4,043 patients with a history of Legg-Calvé-Perthes disease (LCPD), slipped capital femoral epiphysis (SCFE), or developmental dysplasia of the hip (DDH) in the Swedish Hip Arthroplasty Register (SHAR) between 1992 and 2012. For each patient, we matched 5 controls from the general population for age, sex, and place of residence, and acquired information on all participants' socioeconomic background and comorbidities. Mortality after THA was estimated according to Kaplan-Meier, and Cox proportional hazard models were fitted to estimate adjusted hazard ratios (HRs) for the risk of death.Results - Compared with unexposed individuals, patients exposed to a THA due to pediatric hip disease had lower incomes, lower educational levels, and a higher degree of comorbidity but a statistically non-significant attenuation of 90-day mortality (HR 0.9; 95% CI 0.4-2.0) and a lower risk of overall mortality (HR 0.8; CI 0.7-0.9).Interpretation - Patients exposed to THA due to a history of pediatric hip disease have a slightly lower mortality than unexposed individuals. THA seems not to confer increased mortality risks, even in these specific patients with numerous risk factors.

High blood pressure and overweight in children with Legg-Calvé-Perthes disease: a nationwide population-based cohort study.

PURPOSE: Legg-Calvé-Perthes disease (LCPD) and its association with cardiovascular diseases, obesity and hypertension has been consistently observed but remains cloudy. This study aimed to investigate the presence of hypertension and overweight/obesity at diagnosis of LCPD and at a 2-year follow-up and its association with age, sex and lateral pillar classification. METHOD: We compared blood pressure (BP) (n = 93) and body mass index (BMI) (n = 125) in patients registered in the Perthes' register - a part of the Swedish pediatric orthopedic quality register (SPOQ) - with normative data for children with the same age and sex. RESULTS: In children with LCPD 19% had high BP. At the 2-year follow-up, 13% had high BP. For children with LCPD, 30% were either overweight or obese. At the 2-year follow-up, 32% were either overweight or obese. Paired analysis showed stable BMI z-score between these 2 measurements. The sample size of this study was too small to analyze possible associations of high BP or BMI with age, sex and lateral pillar classification. CONCLUSIONS: The prevalence of hypertension was higher in children with LCPD compared to general pediatric normative data. The same pattern was seen for overweight/obesity. Further studies are needed to investigate whether BP and obesity are catalyzing factors in the etiology of LCPD.

A novel heterozygous variant in FGF9 associated with previously unreported features of multiple synostosis syndrome 3.

Human multiple synostoses syndrome 3 is an autosomal dominant disorder caused by pathogenic variants in FGF9. Only two variants have been described in FGF9 in humans so far, and one in mice. Here we report a novel missense variant c.566C > G, p.(Pro189Arg) in FGF9. Functional studies showed this variant impairs FGF9 homodimerization, but not FGFR3c binding. We also review the findings of cases reported previously and report on additional features not described previously.

Epidemiology of pediatric femur fractures in children: the Swedish Fracture Register.

BACKGROUND: Although femur fractures in children are rare, they are the most common fractures in need of hospitalization. We sought to describe the epidemiology and treatment of pediatric femur fractures recorded in the Swedish Fracture Register (SFR). We also studied the relationship between femur fractures, age, sex, fracture pattern, injury mechanism, seasonal variation and treatment. METHODS: This nationwide observational register study was based on the pediatric part of the SFR. We included all patients < 16 years of age who were registered in the SFR from 2015 to 2018. RESULTS: Of the 709 femur fractures, 454 (64%) occurred in boys. Sixty-two of these fractures were proximal (9%), 453 shaft (64%) and 194 distal (27%). A bimodal age distribution peak was observed in boys aged 2-3 and 16-19 years. In contrast, the age distribution among girls was evenly distributed. Younger children were mainly injured by a fall, whereas older children sustained their fracture because of traffic accidents. Non-surgical treatment prevailed among younger children; however, prevalence of surgical treatment increased with age. CONCLUSIONS: We found a lower ratio between boys and girls (1.8:1) compared to earlier studies. The bimodal age distribution was seen only in boys. Falls were the most common injury in younger children, whereas traffic-related accidents were the most common in adolescents. With age, there was a corresponding increase in surgical treatment.

Range of abduction in patients with Legg-Calvé-Perthes disease - a nationwide register-based cohort study.

BACKGROUND: Range of abduction often decreases during Legg-Calvé-Perthes Disease (LCPD) disease. However, a good range of abduction is required during the course of LCPD, especially when containment surgery should be performed. This study aimed to investigate how many patients registered in the Swedish Pediatric Orthopedic Quality register (SPOQ) with LCPD had reduced range of abduction at diagnosis in relation to sex or age at diagnosis or severity of disease (lateral pillar class at the time at diagnosis), if physiotherapy (PT) was prescribed and has a beneficial impact in maintaining (or increasing) abduction and if the range of abduction at diagnosis before fragmentation stage is predictive for the lateral pillar classification at fragmentation stage. METHODS: The national Swedish Pediatric Orthopedic Quality Register (SPOQ), established in 2015, is used to identify patients with LCPD. The patients are registered at three time points: at diagnosis, at potential surgery and 2 years after diagnosis. Range of abduction and information on PT are required to register at all registration sessions. One hundred ninety-nine hips from 192 children were registered in the SPOQ. RESULTS: Of all hips, the mean range of abduction at diagnosis was 39 degrees (range 0 to 90). One hundred twenty-six patients (63%) either received instructions for PT or were referred to a physiotherapist; two patients were treated additionally with an abduction brace. There was a trend that patients who received PT, compared to patients without PT, either maintained or increased their range of abduction at the 2-year follow-up. Older age at diagnosis correlated with decreased range of abduction at the 2-year follow-up (Estimate [Est]: - 3.1, 95% confidence interval [CI]: - 4.4 to - 1.7). The degree of abduction at diagnosis before fragmentation stage correlated with the lateral pillar group at the fragmentation stage (Est: -5.3, 95% CI: - 10.0 to - 1.1). CONCLUSION: In all, 63% of the children with LCPD in SPOQ received either written instructions or were referred to PT or both. PT seems to have a favorable impact for maintaining the range of abduction in children with LCPD. Children with a lower range of abduction at diagnosis (before the fragmentation stage) developed a higher degree of lateral pillar involvement as measured by the lateral pillar classification.

Fate of patients with slipped capital femoral epiphysis (SCFE) in later life: risk of obesity, hypothyroidism, and death in 2,564 patients with SCFE compared with 25,638 controls.

Background and purpose - Associations between obesity and slipped capital femoral epiphysis (SCFE) during adolescence are described; however, few studies report on the lifetime risk of obesity in patients with SCFE. In addition, with the obesity epidemic in children and adolescents, an increasing incidence of SCFE might be expected. An association of SCFE with hypothyroidism seems ambiguous, and the association between SCFE and depression and all-cause mortality has not yet been evaluated. This study investigates the associations of SCFE with obesity, hypothyroidism, depression, and mortality, and putative changes in the yearly incidence of SCFE.Patients and methods - 2,564 patients diagnosed with SCFE at age 5-16 diagnosed between 1964 and 2011 were identified in the Swedish Patient Register. These were matched for age, sex, and residency with unexposed control individuals. Cox regression models were fitted to estimate the risk of obesity, hypothyroidism, depression, and death, in exposed compared with unexposed individuals.Results - The risk of obesity (HR 9, 95% CI 7-11) and hypothyroidism (HR 3, CI 2-4) was higher in SCFE patients compared with controls. There was no increase in the risk of developing depression (HR 1, CI 1-1.3) in SCFE patients. In contrast, all-cause mortality was higher in SCFE patients than in controls (HR 2, CI 1-2). The incidence of SCFE did not increase over the past decades.Interpretation - Patients with SCFE have a higher lifetime risk of obesity and hypothyroidism and a higher risk of all-cause mortality compared with individuals without SCFE. These findings highlight the lifetime comorbidity burden of patients who develop SCFE in childhood, and increased surveillance of patients with a history of SCFE may be warranted. The incidence of SCFE did not increase over the last decades despite increasing obesity rates.

Expanding the phenotypic spectrum of osteogenesis imperfecta type V including heterotopic ossification of muscle origins and attachments.

BACKGROUND: Osteogenesis imperfecta (OI) is a clinical and genetic heterogeneous group of connective tissue disorders, characterized by bone fragility and a propensity to fracture. METHODS: In this report we describe the clinical phenotype of two patients, a 28-year-old woman and her mother (54 years old), both with a history of short stature and multiple fractures. RESULTS: Exome sequencing revealed the recurring IFITM5:c.-14 C>T variant causing OI type V. Both patients had several fractures during childhood. CT-scan and scintigraphy showed ossification of the origin and attachment of muscles and hypertrophic callus formation. CONCLUSION: Ossification of the origin and attachment of muscles seems to be part of the phenotype in patients with OI type V.

Agreement of radiographic measurements and patient-reported outcome in 61 patients with Legg-Calvé-Perthes disease at mean follow-up of 28 years.

It is unclear how patient-reported outcome in patients with Legg-Calvé-Perthes disease (LCPD) is correlated with radiographic outcome. It was therefore the aim of our long-term follow-up to evaluate the agreement of patient-reported outcome measures (PROM) with radiographic outcome in patients with a history of unilateral LCPD and a femoral head involvement of more than 50%. We further investigated to what extent the functional outcome (range of motion and Trendelenburg sign) correlates with PROM and radiographic outcome. At a mean follow-up of 28 years (15-42), 61 patients were investigated clinically and by plain radiography to evaluate the sphericity deviation score, femoral head enlargement and femoral neck growth inhibition. The patients also completed questionnaires for generic measures of health-related quality-of-life (ED-5D, EQ-visual analogue scale), the joint-specific Harris hip score and the nonarthritic hip score questionnaire. The radiographic measures sphericity deviation score, femoral head enlargement and femoral neck growth inhibition were strongly correlated with the joint-specific PROMs (Harris hip score and nonarthritic hip score) but not with EQ-5D and EQ-visual analogue scale. Inferior range of flexion and abduction and a positive Trendelenburg sign were associated with an inferior patient-reported outcome. Our findings highlight the importance of supporting femoral head re-modelling and containment and balancing trochanteric and femoral neck growth in patients with LCPD. To capture the whole picture of the outcome after LCPD, future studies should include a combination of radiographic measurements and joint-specific and generic outcome scores. Level of Evidence: Level III.

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.

Legg-Calvé-Perthes disease and the risk of ADHD, depression, and mortality.

BACKGROUND AND PURPOSE: Hyperactive behavior pattern (such as attention deficit hyperactivity disorder (ADHD)) is proposed to be present in individuals with Legg-Calvé-Perthes disease (LCPD). We investigated whether individuals with LCPD have a higher risk of ADHD, depression, and mortality. SUBJECTS AND METHODS: We identified 4,057 individuals with LCPD in Sweden during the period 1964-2011. 40,570 individuals without LCPD were randomly selected from the Swedish general population and matched by year of birth, sex, and region (control group). We used Cox proportional hazard regression to estimate the relative risks. RESULTS: Compared to the control group, individuals with LCPD had a raised hazard ratio (HR) of 1.5 (95% CI: 1.2-1.9) for ADHD. The risks were higher for female individuals (HR = 2.1, CI: 1.3-3.5) than for male individuals (HR = 1.4, CI: 1.1-1.8). Individuals with LCPD had a modestly higher hazard ratio for depression (HR = 1.3, CI: 1.1-1.5) than the control group. Furthermore, individuals with LCPD had a slightly higher mortality risk than the control group (HR = 1.2, CI: 1.0-1.4) INTERPRETATION: Individuals with LCPD have a higher risk of ADHD. Hyperactivity could expose the femoral head to higher mechanical stress and contribute to the etiology of LCPD. The higher risk of depression may be due to the burden of LCPD itself or could reflect neurobehavioral aspects of ADHD changing into depression later in life. Individuals with LCPD have a higher mortality risk, with higher risk of suicide and cardiovascular diseases.

Legg-Calvé-perthes disease: quality of life, physical activity, and behavior pattern.

BACKGROUND: Legg-Calvé-Perthes disease (LCPD) is a disease in children leading to deformation of the femoral head and can be a promoter for early dysfunction of the hip and early osteoarthritis of the hip. The study of health-related quality of life, physical activity, and behavior patterns in patients with LCPD can reveal its consequences later in life and also contribute to a better understanding of the etiology of the disease. PATIENTS AND METHODS: We identified 145 patients with LCPD diagnosed and treated at Uppsala University Hospital between 1978 and 1995. A total of 116 patients answered questionnaires regarding health-related quality of life (EQ-5D-3L), physical activity [International Physical Activity Questionnaire (IPAQ)], and hyperactive/inattentive behavior pattern [ADHD self-reporting symptom checklist (ASRS v1.1)] by interview. Patients were asked to report on fractures or soft-tissue injuries that required medical care. Medical charts were reviewed to determine age at onset of LCPD and treatment received. RESULTS: Patients with LCPD had significantly lower EQ-5D-3L and EQ VAS scores than the Swedish general population in all age groups. A total of 28% of our patient group had ASRS scores indicating they are likely or highly likely to have an ADHD diagnosis. A lower EQ-5D-3L score was significantly correlated with a higher total ASRS v1.1 score (ρ=-0.309**). Over 90% of our patient group was physically active on a moderate or high level, despite 52% reporting either some or severe problems with pain according to the EQ-5D-3L questionnaire. Patients with high ASRS v1.1 scores (>16) had a significantly higher incidence of soft-tissue injuries than those with lower ASRS v1.1 scores. CONCLUSION: The consequence of LCPD in adulthood was expressed in a lower quality of life compared with the Swedish general population. Despite this, the patients in our study reported a higher level of physical activity than the general population. A tendency toward hyperactive behavior pattern and high physical activity level may be present even in childhood and could contribute to the etiology of LCPD. LEVEL OF EVIDENCE: A retrospective study, level II.

Legg-Calvé-Perthes disease and the risk of injuries requiring hospitalization: a register study involving 2579 patients.

BACKGROUND AND PURPOSE: Previous studies have suggested that Legg-Calvé-Perthes disease (LCPD) is associated with repetitive trauma, coagulation problems and anatomical abnormalities of the blood supply to the femoral head. The hypothesis that repetitive trauma can affect the blood supply of the femoral head, leading to LCPD, is supported by an animal model. For evidence of an increased risk of repetitive trauma, we investigated whether patients with LCPD have a higher risk for severe injuries requiring hospitalization. PATIENTS AND METHODS: We identified 2579 patients with LCPD in Sweden during the period 1964-2005. 13,748 individuals without LCPD were randomly selected from the Swedish general population, matched by year of birth, sex and region (control group). Cox proportional hazard regression estimated the risks. RESULTS: Compared to the control group, patients with LCPD had a modestly raised hazard ratio (HR) of 1.2 (95% CI 1.1-1.3) for injury requiring hospitalization. The risks were slightly higher for soft tissue injuries (HR = 1.3, 95% CI:1.1-1.4) than for fractures (HR = 1.1, 95% CI: 1.0-1.3) and more pronounced among females. Compared to the control group, the higher risk for injury only applied to the lower extremities (HR = 1.2, 95% CI: 1.0-1.4) in patients with LCPD. INTERPRETATION: Patients with LCPD are vulnerable to injuries which could be interpreted as a marker of hyperactive behavior. It could also implicate that anatomical changes in the bone formation or blood supply of the femoral head - increasing its sensibility for trauma - contribute to the etiology of LCPD.

Legg-Calve-Perthes disease and risks for cardiovascular diseases and blood diseases.

OBJECTIVE: We hypothesized that patients with Legg-Calvé-Perthes disease (LCPD) might have higher risks of cardiovascular and blood diseases. METHODS: A total of 3141 patients, 2 to 15 years of age, with LCPD diagnosed between 1965 and 2005 were identified with the Swedish Inpatient Register. A total of 15 595 individuals without LCPD were selected randomly from among the Swedish general population, with matching according to year of birth, age, gender, and region of residence. Cox proportional-hazard regression analyses, with adjustment for socioeconomic index, were used to estimate relative risks. The patients also were compared with their same-gender siblings. RESULTS: Patients with LCPD had a hazard ratio (HR) of 1.70 (95% confidence interval [CI]: 1.39-2.09) for cardiovascular diseases, compared with individuals without LCPD. The point estimate was slightly higher among subjects >30 years of age at the follow-up (HR: 2.10 [95% CI: 1.52-2.91]). There were statistically significantly higher risks for blood diseases, including anemias and coagulation defects (HR: 1.41 [95% CI: 1.07-1.86]), which were more pronounced among subjects >30 years of age at the follow-up (HR: 2.70 [95% CI: 1.50-4.84]). Patients also had statistically significantly higher risks of hypertensive disease (HR: 2.97 [95% CI: 1.87-4.72]) and nutritional anemia (HR: 2.92 [95% CI: 1.58-5.40]). Analyses using siblings as the comparison group showed consistent results for cardiovascular diseases. CONCLUSION: The results are consistent with the hypothesis that an insufficient blood supply to the femoral head, attributable to vascular pathologic conditions, is involved in the pathogenesis of LCPD.

Management of a nonunion of the distal femur in osteoporotic bone with the internal fixation system LISS (less invasive stabilization system).

We present a case illustrating the successful use of the internal fixation LISS in an osteoporotic nonunion of the distal femur, where classic osteosynthesis has failed. The LISS plate with its angular stability offered the possibility to achieve excellent purchase in the severely porotic and partially destroyed bone. In combination with the use of an autologeous bone graft laterally and a strut cortical autograft medially, a mechanical support and an osteoinductive stimulus was provided and the extremity could be saved by this procedure.