Bone Remodeling and Bone Structural Genes in Legg-Calvé-Perthes Disease: The OPG rs2073618 and IL-6 rs1800795 Are Associated with High Risk in Mexican Patients.

Legg-Calve-Perthes disease (LCPD) is an idiopathic avascular necrosis of the pediatric femoral head. Bone remodeling and bone structural genes have the potential to contribute to the progression of LCPD when there is disequilibrium between bone resorption and bone formation. A case-control study was performed to search for associations of several common polymorphisms in the genes Receptor Activator for Nuclear Factor κappa B (RANK), Receptor Activator for Nuclear Factor κappa B Ligand (RANKL), osteoprotegerin (OPG), interleukin (IL)-6, and type 1 collagen (COL1A1) with LCPD susceptibility in Mexican children. A total of 23 children with LCPD and 46 healthy controls were genotyped for seven polymorphisms (rs3018362, rs12585014, rs2073618, rs1800795, rs1800796, rs1800012, and rs2586498) in the RANK, RANKL, OPG, IL-6, and COL1A1 genes by real-time polymerase chain reaction with TaqMan probes. The variant allele (C) of IL-6 rs1800795 was associated with increased risk of LCPD (odds ratio [OR]: 3.8, 95% confidence interval [CI]: [1.08-13.54], p = 0.033), adjusting data by body mass index (BMI) and coagulation factor V (FV), the association with increased risk remained (OR: 4.9, 95% CI: [1.14-21.04], p = 0.025). The OPG polymorphism rs2073618, specifically GC-GG carriers, was associated with a more than fourfold increased risk of developing LCPD (OR: 4.34, 95% CI: [1.04-18.12], p = 0.033) when data were adjusted by BMI-FV. There was no significant association between RANK rs3018362, RANKL rs12585014, IL-6 rs1800796, COL1A1 rs1800012, and rs2586498 polymorphisms and LCPD in a sample of Mexican children. The rs1800975 and rs2037618 polymorphisms in the IL-6 and OPG genes, respectively, are informative markers of increased risk of LCPD in Mexican children.

Predictors of Persistent Limp Following Proximal Femoral Varus Osteotomy for Perthes Disease.

INTRODUCTION: One of the most popular containment procedures for Legg-Calvé-Perthes disease (LCPD) is proximal femur varus osteotomy (PFO). While generally successful in achieving containment, PFO can cause limb length discrepancy, abductor weakness, and (of most concern for families) a persistent limp. While many studies have focused on radiographic outcomes following containment surgery, none have analyzed predictors of this persistent limp. The aim of this study was to determine clinical, radiographic, and surgical risk factors for persistent limp 2 years after PFO in children with LCPD. METHODS: A retrospective review of a prospectively collected multicenter database was conducted for patients aged 6 to 11 years at disease onset with unilateral early-stage LCPD (Waldenström I) who underwent PFO. Limp status (no, mild, and severe), age, BMI, and pain scores were obtained at initial presentation, 3-month, and 2-year postoperative visits. Preoperative and follow-up radiographs were used to measure traditional morphologic hip metrics including acetabular index (AI), lateral center-edge angle (LCEA), and femoral neck-shaft angle (NSA). Univariate analysis as well as multivariate logistic regression models were used to analyze factors associated with mild and severe limp at the 2-year visit. RESULTS: A total of 95 patients met the inclusion criteria, and of these 50 patients underwent concomitant greater trochanter apophysiodesis (GTA) at the time of PFO. At the 2-year visit, there were 38 patients (40%) with a mild or severe limp. Multivariate logistic regression revealed no significant radiographic factors associated with a persistent limp. However, lower 2-year BMI and undergoing GTA were associated with decreased rates of persistent limp regardless of age ( P <0.05). When stratifying by age of disease onset, apophysiodesis appeared to be protective against any severity of limp in patients aged 6 to 8 years old ( P = 0.03), but not in patients 8 years or older ( P = 0.49). CONCLUSIONS: Persistent limp following PFO is a frustrating problem that was seen in 40% of patients at 2 years. However, lower follow-up BMI and performing a greater trochanter apophysiodesis, particularly in patients younger than 8 years of age, correlated with a lower risk of postoperative limp.

Animal Model Standardization for Studying Avascular Necrosis of the Femoral Head in Legg-Calvé-Perthes Disease / Padronização de modelo animal para o estudo da necrose isquêmica da cabeça femoral na doença de Legg-Calvé-Perthes

Abstract Objective Testing an experimental model for ischemic necrosis of the femoral head in Legg-Calvé-Perthes disease by evaluating gait, imaging and morphohistology. Methods The operation was done in 11 piglets. Necrosis by cerclage in the right femoral neck was induced. Piglets were divided into group A, with 8 animals, euthanizing two in the 2nd, 4th, 6th, and 8th weeks, respectively; and group B, with 2 animals (sham), submitted to the surgical procedure without cerclage of the right femoral neck. The gait classification used was that of Etterlin. The frozen femurs were submitted to digital radiography and computed tomography. The height and width of the epiphysis and epiphysary coefficient were measured at study times. Light microscopy and immunohistochemistry with TGF-β1 were performed. Results One animal died of sepsis in Group A. In this group, claudication was observed in all animals. On digital radiography and computed tomography, bone sclerosis, enlargement of the right femoral neck, flattening, collapse, and fragmentation of the right femoral head were observed. All epiphysis height and epiphysary coefficient values of the right femoral head were lower than the contralateral ones, in which were observed chondrocytes disordered and separated by gaps. A reduction in TGF-β1 expression was observed at 2 and 6 weeks in the right femoral head and at eight in the left. In group B, there were no signs of necrosis and gait was normal. Conclusions The model presented reproduced macroscopic necrosis on digital radiography, computed tomography, and microscopy. Gait evaluation showed a good correlation with other ischemia findings. Level of EvidenceV. Diagnostic studies.

Resumo Objetivo Testar um modelo experimental para necrose isquêmica da cabeça femoral na doença de Legg-Calvé-Perthes avaliando a marcha, exames de imagens e morfohistologia. Métodos Operaram-se 11 leitões. Induziu-se a necrose por cerclagem no colo femoral direito. Dividiram-se os leitões em grupo A com 8 animais, sacrificando-se dois na 2ª, 4ª, 6ª e 8ª semanas, respectivamente; e grupo B, com 2 animais (sham), submetidos ao procedimento cirúrgico sem a cerclagem do colo femoral direito. A classificação da marcha utilizada foi a de Etterlin. Os fêmures congelados foram submetidos à radiografia digital e tomografia computadorizada. Mediram-se a altura e largura da epífise e o coeficiente epifisário nos tempos de estudo. Realizou-se, microscopia de luz e imunohistoquímica com TGF-β1. Resultados Um animal morreu por sepse no grupo A. Neste grupo, observou-se claudicação em todos os animais. Na radiografia digital e tomografia computadorizada observaram-se: esclerose óssea, alargamento do colo femoral direito, achatamento, colapso e fragmentação da cabeça femoral direita. Todos os valores da altura da epífise e coeficiente epifisário da cabeça femoral direita foram menores que os contralaterais, nos quais observaram-se condrócitos desordenados e separados por lacunas. Observou-se redução da expressão do TGF-β1 com 2 e 6 semanas nas cabeças femorais direitas e nas esquerdas com oito. No grupo B, não ocorreram sinais de necrose e a marcha foi normal. Conclusões O modelo apresentado reproduziu a necrose macroscopicamente, na radiografia digital, tomografia computadorizada e microscopia. A avaliação da marcha demonstrou boa correlação com os demais achados de isquemia. Nível de EvidênciaV. Estudos diagnósticos.

Does Acetabulum Remodel Following Varus Derotation Osteotomy for Perthes' Disease? / O acetábulo sofre remodelamento após a osteotomia derrotatória varizante em pacientes com doença de Perthes?

Abstract Objective We investigated the effect of disease stage, patient's age and final contour of femoral head on acetabulum contour following varus derotation osteotomy of proximal femur (VDRO) in unilateral Perthes's disease. Methods The study is a retrospective analysis of case records of 23 children aged ≥6 years with unilateral Perthes' disease who underwent primary VDRO procedure for containment. Acetabular index (AI) and center edge angle (CEA) were calculated bilaterally in preoperative and follow-up radiographs and compared statistically. Results There were 15 boys and 8 girls. Six hips were in Ib, 8 in IIa and 9 in IIb modified Waldenström stage while undergoing VDRO. The mean age at surgical intervention was 8.7 years. The mean follow-up duration was 3.5 years. All femoral heads were healed at final follow-up and the final Stulberg grades were I = 3, II = 8, III = 7, IV = 5. A significant acetabular dysplasia on the affected side was present preoperatively. At follow-up, the patients operated had significantly raised AI and reduced CEA. There was no significant acetabular remodeling of the affected hips at follow-up even in children operated at younger age (< 8 years) or early stages (stage Ib or IIa). The acetabulum remodeling did not correspond to the final Stulberg grade as well. Conclusion Acetabulum was found involved in early stages of Perthes' disease. Varus derotation femoral osteotomy for the diseased hip showed no significant improvement in acetabular dysplasia even when operated in early disease stages or younger age group. Residual acetabular changes were also noted even with favorable Stulberg grades.

Resumo Objetivo Investigamos o efeito do estágio da doença, idade do paciente e contorno final da cabeça femoral no contorno do acetábulo após a osteotomia derrotatória varizante (VDRO) do fêmur proximal na doença de Perthes unilateral. Métodos O estudo é uma análise retrospectiva de prontuários de 23 crianças com idade ≥ 6 anos com doença de Perthes unilateral que foram submetidas ao procedimento primário de VDRO para contenção. O índice acetabular (AI) e o ângulo da borda central (CEA) foram calculados bilateralmente em radiografias pré-operatórias e de acompanhamento e submetidos à comparação estatística. Resultados Os pacientes eram 15 meninos e oito meninas. À VDRO, seis quadris estavam no estágio de Waldenström modificado Ib, oito no estágio IIa e nove no estágio IIb. A média de idade à intervenção cirúrgica foi de 8,7 anos. A duração média do acompanhamento foi de 3,5 anos. Todas as cabeças femorais estavam consolidadas no último acompanhamento e os graus finais de Stulberg foram I = 3, II = 8, III = 7 e IV = 5. Havia displasia acetabular significativa do lado acometido no período pré-operatório. No acompanhamento, os pacientes operados apresentaram elevação significativa de AI e redução de CEA. Não houve remodelamento acetabular significativo nos quadris acometidos durante o acompanhamento, mesmo em crianças operadas em idade menor (< 8 anos) ou estágios iniciais (estágio Ib ou IIa). O remodelamento do acetábulo também não correspondeu ao grau final de Stulberg. Conclusão A VDRO do fêmur do quadril acometido não levou à melhora significativa da displasia acetabular, mesmo quando a cirurgia foi realizada nos estágios iniciais da doença ou em pacientes mais jovens. Alterações acetabulares residuais também foram observadas mesmo com graus de Stulberg favoráveis.

Cirugía de prótesis de cadera en el adulto con enfermedad de Legg-Calvé-Perthes / Total hip arthroplasty in adults with Legg-Calvé-Perthes disease

Objetivos: Revisar las cirugías de prótesis totales de cadera realizadas en nuestro hospital, determinar el origen de la artrosis e identificar cuántas se colocaron por coxartrosis secundarias a enfermedad de Legg-Calvé-Perthes. Materiales y Métodos: Se realizó un estudio retrospectivo en el que se revisaron todas las cirugías de prótesis totales de cadera desde 2008 hasta diciembre de 2021. Se evaluaron las radiografías prequirúrgicas para determinar la etiología de la artrosis, y se consideraron variables, como lateralidad, sexo y edad en el momento de la intervención. Resultados: Se revisaron 1103 caderas en 935 pacientes. El 81% correspondía a coxartrosis primaria. En 11 caderas de 10 pacientes (1%), se detectó coxartrosis secundaria a la enfermedad de Legg-Calvé-Perthes. La media de la edad de estos pacientes era de 61 años. Conclusiones: Hay evidencia de que las alteraciones del crecimiento de la fisis femoral proximal o el sobrecrecimiento del trocánter mayor, propias de la enfermedad de Legg-Calvé-Perthes, pueden contribuir a la aparición de un choque femoroacetabular, con su consiguiente coxartrosis precoz. Es posible que algunas "mal clasificadas" coxartrosis primarias fueran identificadas así porque no existía otro dato sugerente de coxartrosis secundarias, y escondieran otra etiología evolucionada. Asimismo, proponemos el seguimiento del paciente joven con enfermedad de Legg-Calvé-Perthes, más allá del final del crecimiento, para identificar el choque femoroacetabular en sus inicios y poder ofrecer opciones terapéuticas artroscópicas. Nivel de Evidencia: III

Objectives: To review the number of total hip replacements (THA) performed in our hospital, determine their aetiology and identify how many of them were performed for hip osteoarthritis secondary to Legg-Calvé-Perthes disease (LCPD). Materials and Methods: We conducted a retrospective study reviewing all THA surgeries from 2008 to December 2021. We studied the pre-operative radiographs, determining the aetiology of the osteoarthritis, laterality, sex and age of the patient at the time of surgery. Results: We reviewed a total of 1103 hips in 935 patients. Primary hip osteoarthritis accounted for 81% of the cases. We gathered a total of 11 hips from 10 individuals (1%), with a mean age of 61 years, for hip osteoarthritis secondary to LCPD. Conclusions:There is evidence that femoro-acetabular impingement (FAI), which results in early secondary hip osteoarthritis, may be influenced by changes in the growth of the proximal femoral physis or overgrowth of the greater trochanter, which are characteristics of LCPD. We believe that certain cases of "misclassified" primary hip osteoarthritis may have been incorrectly identified since no additional information was found to support the diagnosis of secondary hip osteoarthritis, hiding the potential of an alternate, evolved aetiology. Furthermore, we suggest monitoring young patients with LCPD after their growth is complete in order to detect early FAI and provide arthroscopic therapeutic options. Level of Evidence: III

Cementless Total Hip Arthroplasty in Patients with Osteoarthrosis Secondary to Legg-Calvé-Perthes Disease Compared with Primary Osteoarthrosis: A Case-control Study / Artroplastia total do quadril não cimentada em pacientes com osteoartrose secundária à doença de Legg-Calvé-Perthes em comparação com a osteoartrose primária: Um estudo caso-controle

Abstract Objective To perform a comparative clinical, functional and radiographic evaluation of total hip arthroplasty (THA) performed with a cementless prosthesis in cases of osteoarthrosis secondary to Legg-Calvé-Perthes Disease (LCPD) and in cases of primary osteoarthrosis. Methods In the present case-control study, we reviewed medical records of patients admitted to a university hospital between 2008 and 2015 to undergo THA due to LCPD sequelae and compared them with a control group of patients who underwent the same surgery due to primary hip osteoarthrosis. We recruited patients for clinical, functional, and radiographic analysis and we compared the evaluations in the immediate postoperative period and at the last follow-up visit, considering surgical time, size of prosthetic components, and complications. Results We compared 22 patients in the study group (25 hips) with 22 patients (25 hips) in the control group, all of whom had undergone THA with the same cementless prosthesis. There was greater functional impairment in the group of patients with LCPD sequelae (p= 0.002). There were 4 intraoperative femoral periprosthetic fractures in the LCPD group and none in the primary osteoarthrosis group (p= 0.050). Conclusions There is an increased risk of intraoperative periprosthetic femoral fracture and worse clinical-functional results in patients undergoing cementless THA due to osteoarthrosis secondary to LCPD sequelae than in those who have undergone the same surgery due to primary hip osteoarthrosis.

Resumo Objetivo Realizar uma avaliação clínica, funcional e radiográfica comparativa da artroplastia total do quadril (ATQ) realizada com prótese não cimentada em casos de osteoartrose secundária à doença de Legg-Calvé-Perthes (DLCP) e em casos de osteoartrose primária. Métodos No presente estudo caso-controle, foram revisados os prontuários dos pacientes internados em um hospital universitário entre os anos de 2008 e 2015. Os pacientes foram submetidos a ATQ devido a sequelas da DLCP, sendo comparados com um grupo controle de pacientes submetidos à mesma cirurgia por osteoartrose primária do quadril. Os pacientes foram recrutados para a realização de uma análise clínica, funcional e radiográfica, na qual foram comparadas as avaliações no pós-operatório imediato e na última consulta de acompanhamento, levando em consideração o tempo cirúrgico, o tamanho dos componentes protéticos e as complicações. Resultados Comparamos 22 pacientes do grupo de estudo (25 quadris) com 22 pacientes (25 quadris) do grupo controle, todos os quais foram submetidos a ATQ com a mesma prótese não cimentada. Houve um maior comprometimento funcional no grupo de pacientes com sequelas da DLCP (p= 0,002). Ocorreram 4 fraturas periprotéticas femorais no intraoperatório do grupo DLCP, sendo que não ocorreu nenhuma no grupo de osteoartrose primária (p= 0,050). Conclusões Existe um risco elevado de fratura periprotética femoral no intraoperatório com resultados clínico-funcionais mais desfavoráveis aos pacientes que foram submetidos à ATQ não cimentada devido a osteoartrose secundária às sequelas da DLCP do que naqueles que foram submetidos à mesma cirurgia por osteoartrose primária de quadril.

Association of MTHFR rs1801133 and homocysteine with Legg-Calvé-Perthes disease in Mexican patients.

BACKGROUND: Legg-Calvé-Perthes disease (LCPD) is the avascular osteonecrosis of the proximal femoral epiphysis. It is a rare disease of unclear etiology in children, although alterations in coagulation or the collagen gene have been described and could be associated with its etiology. Our objective was to evaluate the following alterations: COL1A1 (rs1107946, rs2412298), COL2A1 (rs121912891 and rs387106558), MTHFR rs1801133, CBS rs115742905, and PT rs1799963 and their relationship with LCPD. METHODS: DNA was obtained and genotyped by real-time PCR with TaqMan probes. Prothrombin (FII) and homocysteine (Hcy) were determined by a coagulometric method. The variables were described as mean and standard deviation or percentages, and genotypic and allelic distributions were analyzed using the Student's t-test. The Hardy-Weinberg equilibrium and OR were also used. RESULTS: We studied 23 patients with LCPD and 46 controls. We did not find any association of the MTHFR, CBS, PT, COL1A1, and COL2A1 genetic variants with LCPD. However, when adjusting the data with the Hcy values for the MTHFR C677T polymorphism, the C/C genotypes showed an association with the recessive model (p = 0.038), with susceptibility to LCPD. CONCLUSION: No association was found with the CBS, PT, COL1A1, and COL2A1 genes. Nevertheless, our results suggest a significant link between moderately elevated Hcy levels and the MTHFR C677T polymorphism in a cohort of Mexican children with LCPD.

Legg-Calvé-Perthes disease overview.

BACKGROUND: Legg-Calvé-Perthes Disease (LCPD) is a necrosis of the femoral head which affects the range of motion of the hips. Its incidence is variable, ranging from 0.4/100,000 to 29.0/ 100,000 children. Although LCPD was first described in the beginning of the past century, limited is known about its etiology. Our objective is to describe the main areas of interest in Legg-Calve-Perthes disease. METHODS: A review of the literature regarding LCPD etiology was performed, considering the following inclusion criteria: Studies reporting clinical or preclinical results. The research group carried out a filtered search on the PubMed and Science Direct databases. To maximize the suitability of the search results, we combined the terms ''Perthes disease" OR "LCPD" OR "children avascular femoral head necrosis" with "diagnostic" OR "treatment" OR "etiology" as either key words or MeSH terms. RESULTS: In this article been described some areas of interest in LCPD, we include topics such as: history, incidence, pathogenesis, diagnosis, treatment and possible etiology, since LCPD has an unknown etiology. CONCLUSIONS: This review suggests that LCPD has a multifactorial etiology where environmental, metabolic and genetic agents could be involved.

Hip Morphology in Early-stage LCPD: Is There an Argument for Anatomic-specific Containment?

BACKGROUND: Early containment surgery has become increasingly popular in Legg-Calvé-Perthes Disease (LCPD), especially for older children. These procedures treat the proximal femur, the acetabulum, or both, and most surgeons endorse the same surgical option regardless of an individual patient's anatomy. This "one-surgery-fits-all" approach fails to consider potential variations in baseline anatomy that may make one option more sensible than another. We sought to describe hip morphology in a large series of children with newly diagnosed LCPD, hypothesizing that variation in anatomy may support the concept of anatomic-specific containment. METHODS: A retrospective review of a prospectively collected multicenter database was conducted for patients aged 6 to 11 at diagnosis. To assess anatomy before significant morphologic changes secondary to the disease itself, only patients in Waldenström stages IA/IB were included. Standard hip radiographic measurements including acetabular index, lateral center-edge angle, proximal femoral neck-shaft angle (NSA), articulotrochanteric quartiles, and extrusion index (EI) were made on printed anteroposterior pelvis radiographs. Age-specific percentiles were calculated for these measures using published norms. Significant outliers (≤10th/≥90th percentile) were reported where applicable. RESULTS: A total of 168 patients with mean age at diagnosis of 8.0±1.3 years met inclusion criteria (81.5% male). Mean acetabular index for the entire cohort was 16.8±4.1 degrees; 58 hips (34.5%) were significantly dysplastic compared with normative data. Mean lateral center-edge angle was 15.9±5.2 degrees at diagnosis; 110 (65.5%) were ≤10th percentile indicating dysplasia (by this metric). Mean NSA overall was 136.5±7.0 degrees. Fifty-one (30.4%) and 20 (11.9%) hips were significantly varus (≤10th percentile) or valgus (≥90th percentile), respectively. Thirty-five hips (20.8%) were the third articulo-trochanteric quartiles or higher suggesting a higher-riding trochanter at baseline. Mean EI was 15.5%±9.0%, while 63 patients (37.5%) had an EI ≥20%. CONCLUSIONS: The present study finds significant variation in baseline anatomy in children with early-stage LCPD, including a high prevalence of coexisting acetabular dysplasia as well as high/low NSAs. These variations suggest that the "one-surgery-fits-all" approach may lack specificity for a particular patient; a potentially wiser option may be an anatomic-specific containment operation (eg, acetabular-sided osteotomy for coexisting dysplasia, varus femoral osteotomy for valgus NSA). LEVEL OF EVIDENCE: Level IV.

Displasia epifisaria de Meyer. Presentación de un caso / Meyer epiphyseal dysplasia. Case presentation

RESUMEN Se presentó el caso de una niña con el diagnóstico de displasia de Meyer. Consiste en una alteración en el desarrollo de la cadera en el niño dado por irregularidad y retraso en la osificación del núcleo de la epífisis femoral, aunque muchos ortopedistas la consideran como una variante fisiológica normal. Radiológicamente se manifiesta como un patrón granular múltiple de osificación y clínicamente si bien puede ser sintomática en algunos casos, lo más frecuente es que sea asintomática. Se enfatizó en la importancia de considerar a la displasia de Meyer como posibilidad diagnóstica ante alteraciones en la osificación de la epífisis femoral. Se señaló a la displasia congénita de la cadera y la enfermedad de Perthes como principales entidades a tener en cuenta al hacer el diagnóstico diferencial. Se analizó la evolución clínico-radiológica de la paciente a partir de los datos recogidos en la historia clínica. Se concluyó enfatizando que la displasia de Meyer debe tenerse presente como posibilidad diagnóstica ante casos similares, realizar una cuidadosa valoración de cada paciente y tener en cuenta a la displasia congénita de la cadera y la enfermedad de Perthes como diagnóstico diferencial atendiendo a la edad del paciente (AU).

ABSTRACT The authors present the case of a female child diagnosed with Meyers dysplasia. It is an alteration of the hip development in children, given the ossification irregularity and retardation of the femoral epiphysis nucleus, although several orthopedists consider it a normal physiological variant. Radiologically, it shows like a multiple granular pattern of ossification, and clinically it could be symptomatic in several cases, but more frequently it is asymptomatic. It was emphasized the importance of considering Meyer dysplasia as a diagnostic possibility in the presence of alterations in the femoral epiphysis ossification. The authors indicated hip congenital dysplasia and Perthes disease as main entities to take into account when making the differential diagnosis. They also analyzed clinic-radiological evolution of the patient on the basis of the data collected in the clinical record. They concluded emphasizing that Meyer dysplasia must be taken into consideration as a diagnostic possibility in similar cases, each patient should be carefully assessed and that hip congenital dysplasia and Perthes disease have to be considered as differential diagnosis given the age of the patient (AU).

Does Early Proximal Femoral Varus Osteotomy Shorten the Duration of Fragmentation in Perthes Disease? Lessons From a Prospective Multicenter Cohort.

BACKGROUND: The prognosis of Legg-Calvé-Perthes disease (LCPD) is dependent upon several factors, with the length and severity of the fragmentation stage among the most important. Previous retrospectively collected data from a single center have suggested that early proximal femoral varus osteotomy (PFO) may shorten the length of fragmentation and allow 34% of patients to bypass fragmentation altogether resulting in less femoral head deformity. The purpose of this study was to validate these findings in a prospectively collected multicenter cohort. METHODS: Patients with LCPD treated with early PFO (during Waldenström stage I) were prospectively followed with serial radiographs at 3-month intervals until a minimum of 2-year follow-up. Waldenström stages and lateral pillar class were determined by mode assessments from 3 pediatric orthopaedic surgeons. The duration of fragmentation was defined as the interval between the first radiographs demonstrating features of stage IIa and stage IIIa. "Complete" bypass was defined as the absence of stage IIa or IIb findings on sequential radiographs with no development of femoral head deformity or collapse. "Partial" bypass was defined as the absence of advanced features of fragmentation and femoral head collapse (stage IIb). RESULTS: Forty-six patients (80% male individuals) with initial stage LCPD and a mean age of 8.2±1.2 years were identified. The weighted kappa statistics for Waldenström staging and lateral pillar classifications showed excellent (0.833) and substantial (0.707) agreement, respectively. Ninety-eight percent of patients (45/46) underwent some period of fragmentation lasting between 91 and 518 days; the median duration was 206 days (interquartile range, 181 to 280). One patient (2%) bypassed fragmentation completely; 8 patients (17%) demonstrated partial bypass. Patients who completely or partially bypassed fragmentation experienced significantly less severe lateral pillar collapse (P=0.016) and shorter fragmentation duration (P=0.001). CONCLUSIONS: In this prospective multicenter cohort, we found a lower rate of fragmentation bypass than previously reported. Nonetheless, our data support the previous contention that early PFO may shorten fragmentation and minimize collapse in LCPD compared with historical controls. Further study with larger cohorts and a more rigorous definition of what constitutes bypass is warranted to clarify the effect of early PFO on the reparative biology of LCPD. LEVEL OF EVIDENCE: Level IV-therapeutic study.

The possible origin of Legg-Calvé-Perthes disease / El posible origen de la enfermedad de Legg-Calvé-Perthes

Legg-Calvé-Perthes disease (LCPD) is a childhood orthopedic pathology that affects the development of the hip. It is a rare disease with a huge variation in annual incidence. It occurs approximately five times more often in boys than in girls. The objective of this article was to formulate a hypothesis about the possible origin of LCPD, from the paleopathological findings of this disease reported until 2017, and to highlight the importance of anthropology, history, paleontology and paleopathology to the study of the origins of disease. By using eight web-based search engines, we performed a review of articles focused on the history, genetics and paleopathological findings of LCPD; we evaluated in total 133 articles published between 1910 and 2017. Out of these, 20 articles belonging to the same publication period were included in this analysis. LCPD was described for the first time approximately 100 years ago and without knowing it was a new disease. In the last years, human remains have been found in which LCPD has been identified, providing relevant information about the origin of this pathology. These data and their historical context can be a basis to propose the Asian continent as the site of origin of LCPD; however, new anthropological, genetic and paleopathological studies are needed to reinforce or refute this hypothesis.

La enfermedad de Legg-Calvé-Perthes (ELCP) es una afección ortopédica infantil que repercute en el desarrollo de la cadera. Es una enfermedad rara con incidencia anual variable. Es aproximadamente cinco veces más frecuente en niños que en niñas. El objetivo de este artículo fue formular una hipótesis acerca del posible origen de la ELCP a partir de hallazgos paleopatológicos reportados hasta el año 2017, además de resaltar la importancia que ofrecen la antropología, la historia, la paleontología y la paleopatología para el estudio del origen de las enfermedades. Mediante ocho buscadores se hizo una revisión de artículos referentes a la historia, la genética y los hallazgos paleopatológicos de la ELCP; se evaluaron un total de 133 artículos publicados entre 1910 y 2017. De ellos, fueron incluidos en este análisis 20 artículos que abarcaron el mismo periodo de publicación. La ELCP comenzó a describirse hace poco más de 100 años y sin el conocimiento de que se trataba de una entidad nueva. En los últimos años se han encontrado restos humanos en los que se ha identificado la ELCP, lo cual ha brindado información relevante respecto al origen de este padecimiento. Estos datos y su contexto histórico pueden ser fundamentos para plantear al continente asiático como el sitio de origen de la ELCP; sin embargo, se requiere de nuevos estudios antropológicos, genéticos y paleopatológicos para reforzar o refutar esta hipótesis.

Necrose asséptica da cabeça do fêmur em cão da raça spitz alemão: Relato de caso

A necrose asséptica da cabeça do fêmur pode ser conhecida também como doença de Legg-Calvé-Perthes, osteocondrite dissecante, necrose avascular da cabeça do fêmur, dentre outras. É uma afecção não inflamatória da cabeça e colo femoral que ocorre principalmente em animais jovens e de pequeno porte. Os sinais clínicos costumam ser parecidos com as de outras enfermidades ortopédicas por isso é imprescindível um minucioso exame clínico e físico do animal, juntamente com exame de imagem para auxiliar no diagnóstico. A escolha do tratamento a ser utilizado depende da severidade da doença podendo ser conservador ou cirúrgico.

Aseptic necrosis of the femoral head may also be known as Legg-Calvé-Perthes disease, osteochondritis dissecans, avascular necrosis of the femoral head, among others. It is a noninflammatory condition of the femoral head and neck that occurs mainly in young and small animals. The clinical signs are usually similar to those of other orthopedic diseases, so a thorough clinical and physical examination of the animal is essential, along with imaging to aid in diagnosis. The choice of treatment to be used depends on the severity of the disease and may be conservative or surgical.

Necrose asséptica da cabeça do fêmur em cão da raça spitz alemão: Relato de caso

A necrose asséptica da cabeça do fêmur pode ser conhecida também como doença de Legg-Calvé-Perthes, osteocondrite dissecante, necrose avascular da cabeça do fêmur, dentre outras. É uma afecção não inflamatória da cabeça e colo femoral que ocorre principalmente em animais jovens e de pequeno porte. Os sinais clínicos costumam ser parecidos com as de outras enfermidades ortopédicas por isso é imprescindível um minucioso exame clínico e físico do animal, juntamente com exame de imagem para auxiliar no diagnóstico. A escolha do tratamento a ser utilizado depende da severidade da doença podendo ser conservador ou cirúrgico.(AU)

Aseptic necrosis of the femoral head may also be known as Legg-Calvé-Perthes disease, osteochondritis dissecans, avascular necrosis of the femoral head, among others. It is a noninflammatory condition of the femoral head and neck that occurs mainly in young and small animals. The clinical signs are usually similar to those of other orthopedic diseases, so a thorough clinical and physical examination of the animal is essential, along with imaging to aid in diagnosis. The choice of treatment to be used depends on the severity of the disease and may be conservative or surgical.(AU)

Initial experience of use of an articulated external fixator in treating Legg-Calvé-Perthes disease by means of arthrodiastasis during the active phase of the disease / Experiência inicial com o uso de fixador externo articulado no tratamento da doença de Legg-Calvé-Perthes por meio de artrodiástase na fase ativa da moléstia

To present the preliminary results from treating patients with Legg-Calvé-Perthes Disease (LCPD) by means of hip arthrodiastasis using a monolateral external fixator applied to the hip and to succinctly describe the surgical technique used, in a prospective study. METHODS: Prospective study on 18 patients with LCPD who underwent surgical treatment by means of the hip arthrodiastasis technique using a monolateral external fixator. There were 13 male and five female patients of mean age 8.5 years, ranging from five to 13 years. All the patients presented unilateral hip impairment: nine on the right side and nine on the left. The results were evaluated at maturity using clinical and radiological criteria. RESULTS: All the patients evolved with improvement of joint mobility, and pain relief was achieved in 88.9% of them. Reossification of the femoral epiphysis occurred within the first three months of the treatment. The hips operated at the necrosis stage of the disease did not passed through the fragmentation stage, thus shortening the evolution of the disease. The results were 77.8% satisfactory and 22.2% unsatisfactory. CONCLUSION: Hip arthrodiastasis with a monolateral external fixator during the active phase of LCPD improved the degree of joint mobility. Use of the arthrodiastasis technique at the necrosis stage or at the fragmentation stage (active phase of the disease) presented satisfactory results from treatment of LCPD.

Apresentar os resultados preliminares do tratamento da DLCP com o uso de artrodiástase com fixador externo monolateral aplicado ao quadril e descrever sucintamente a técnica operatória usada em um estudo prospectivo. MÉTODOS: Estudo prospectivo de 18 pacientes com DLCP submetidos ao tratamento operatório com a técnica de artrodiástase do quadril por meio de fixador externo unilateral. São 13 pacientes do gênero masculino e cinco do feminino com idade média de 8,5 anos com variação de cinco a 13 anos. Todos os pacientes com acometimento unilateral do quadril, nove à direita e nove à esquerda. A avaliação dos resultados foi feita na maturidade e considerou critérios clínicos e radiográficos. RESULTADOS: Todos os pacientes evoluíram com melhoria da mobilidade articular com alívio da dor obtido em 88,9% dos pacientes. A reossificação da epífise femoral ocorreu nos primeiros três meses do tratamento. Os quadris operados na fase de necrose não passaram pela fase de fragmentação e abreviaram o tempo de evolução da doença. Os resultados foram 77,8% satisfatórios e 22,2% insatisfatórios. CONCLUSÕES: A artrodiástase do quadril com fixador externo monolateral na fase ativa da DLCP melhora o grau de mobilidade articular. O emprego da técnica de artrodiástase nas fases de necrose e fragmentação (fase ativa da doença) apresenta resultados satisfatórios no tratamento da DLCP.

Caracterización de la enfermedad de Legg-Calvé-Perthes en población pediátrica en tres Hospitales de Bogotá DC, Colombia / Characterization of Legg-Calvé-Perthes disease in the pediatric population of three hospitals in Bogotá DC, Colombia

Estudio observacional, descriptivo longitudinal (nivel III). Objetivo: describir las características clínicas, demográficas y posibles factores de riesgo en pacientes con enfermedad de Legg-Calvé-Perthes (LCP) en tres hospitales de Bogotá DC, Colombia entre 2003 y 2013. Materiales y métodos: historias clínicas ortopédicas de pacientes con LCP en rangos de edad entre 4 y 14 años. Resultados: el total de la población fue de 83 pacientes con 89 caderas comprometidas; 88% fueron hombres. Los factores de riesgo encontrados fueron: en 24 casos (29%) trastorno de déficit de atención e hiperactividad (TDAH), 23 (28%) evidenciaron exposición pasiva a humo de cigarrillo de la madre durante el embarazo y 4.8% tenían discrasias sanguíneas. Conclusiones: teniendo en cuenta los resultados no se demostró que la enfermedad de LCP en pacientes pediátricos tuviera relación con madres añosas (>35 años) al momento del parto; 29% cursó con TDAH y 28% con exposición pasiva a humo de cigarrillo durante el embarazo...

Longitudinal, observational, descriptive (level III) study. Objective: to describe the clinical features, demographics and possible risk factors of Legg-Calvé-Perthes disease (LCPD) in patients of three hospitals in Bogotá DC, Colombia between 2003 and 2013. Materials and Methods: review of orthopedic clinical records of patients with LCPD aged 4 to 14 years. Results: a total population of 83 patients with 89 compromised hips were included, 88% were males. Risk factors found were: attention deficit hyperactivity disorder (ADHD) in 24 cases (29%), passive exposure to cigarette smoke of the mother during pregnancy in 23 (28%) and coagulation disorders in 4.8%. Conclusions: the results did not demonstrate that LCPD in pediatric patients had relation to advanced maternal age (>35 years) at delivery; 29% were associated to ADHD and 28% with passive exposure to cigarette smoke of the mother during pregnancy...

Comparison of the results from simple radiography, from before to after Salter osteotomy, in patients with Legg-Calvé-Perthes disease,

Objectives: To determine whether the clinical variables and preoperative classification of patients with Legg-Calvé-Perthes disease (LCPD) who undergo Salter osteotomy correlate with the radiographic result at the time of skeletal maturity. Methods: In this retrospective cohort study, 47 individuals with LCPD who were treated using Salter osteotomy (1984-2004) were evaluated. The patients were evaluated according to sex, skin color, side affected and age at which osteotomy was performed. The preoperative radiographs were analyzed in accordance with the classifications of Waldenstrom, Catter-all, Laredo and Herring. The radiographs obtained at the time of skeletal maturity were classified using the Stulberg method. Results: The mean age at the time of surgical treatment was 82.87 months (6.9 years). The age presented a statistically significant correlation with the Stulberg grades at skeletal maturity (p < 0.001). Patients over the age of 6.12 years tended to present less favorable results. The variables of sex, skin color and side affected did not present any statistically significant correlation with the prognosis (p = 0.425; p = 0.467; p = 0.551, respectively). Only the Laredo classification presented a statistically significant correlation with the final result given by the Stulberg classification (p = 0.001). The other classifications used (Waldenstrom, Catterall and Herring) did not present any correlation between the time at which surgery was indicated and the postoperative result. Conclusions: The age at which the patients underwent surgical treatment and the Laredo classification groups were the only variables that presented significant correlations with the Stulberg classification...

Objetivos: Determinar em pacientes com doenca de Legg-Calvé-Perthes (DLCP) submetidos à osteotomia de Salter se as variáveis clínicas e as classificacões pré-operatórias se correlacionam com o resultado radiográfico na maturidade esquelética. Métodos: Neste estudo de coorte retrospectivo foram avaliados 47 indivíduos portadores da DLCP tratados com osteotomia de Salter (1984-2004). Os pacientes foram avaliados de acordo com sexo, cor, lado acometido e idade em que foi feita a osteotomia. As radiografias pré-operatórias foram analisadas de acordo com as classificacões de Waldenstrõm, Catterall, Laredo e Herring. As radiografias obtidas na maturidade esquelética foram classificadas segundo o método de Stulberg. Resultados: A média da idade no momento do tratamento cirúrgico foi de 82,87 meses (6,9 anos). A idade apresentou correlacão estatisticamente significativa com os graus de Stulberg na maturidade esquelética (p <0,001). Pacientes acima de 6,12 anos tendem a apresentar resultados menos favoráveis. As variáveis sexo, cor e lado acometido não apresentaram correlacão estatisticamente significativa com o prognóstico (p = 0,425; p = 0,467; p = 0,551, respectivamente). Apenas a classificacão de Laredo apresentou correlacão estatisticamente significante com o resultado final dado pela classificacão de Stulberg (p = 0,001). As demais classificacões usadas, Waldenstrõm, Caterall e Herring, não apresentaram correlacão entre o momento em que foi indicada a cirurgia e o resultado pós-operatório. Conclusões: A idade em que os pacientes foram submetidos ao tratamento cirúrgico e os grupos da classificacão de Laredo foram as únicas variáveis que apresentaram correlacão significativa com a classificacão de Stulberg...

Tension band wiring for proximal femoral varus osteotomy fixation in children.

For proximal varus osteotomy of the femur in children, a stable fixation, is a concern regarding the safety and the protection of the surgery. The methods of fixation are mostly represented by plating systems; however, the tension band wiring is a simple and eligible option for fixation of proximal femur osteotomy. The purpose of this retrospective case series study is to evaluate outcomes and bone remodelation after using the tension band wiring technique to fixate proximal femoral varus osteotomies in children.The study enrolled 20 patients who underwent femoral varus osteotomy to treat Legg-Calvé-Perthes disease and other causes of necrosis of the femoral head. The mean patient age at the time of surgery was 7.4 ± 2.3 years (range, 4.3-13.8 y), and the follow-up period averaged 10 ± 4.3 years (range, 4.7-20.8 y). To assess the effects of the osteotomy at the neck-shaft angle and its evolution over time, radiographs obtained preoperatively and during the follow-up were analyzed. The neck-shaft angle was measured independently by 2 observers, and intraclass correlation coefficients (ICCs) were assessed for reliability.The ICC analysis showed good reliability. There was a significant reduction in the neck-shaft angle (P < 0.001) between the preoperative and the immediate postoperative periods, with an estimated difference of 14.3°. Between the immediate and the early postoperative periods, the estimated difference was 2.1°, which indicated a significant increase in the neck-shaft angle (P = 0.003). Between the early postoperative period and the final evaluation, there was a significant increase (P < 0.001), with an estimated difference of 7.0°. No implant failures or other complications were observed, but 1 case of peri-implant femoral fracture occurred >17 years postsurgery.The tension band wiring technique proved to be a simple and effective method for fixating proximal femoral varus osteotomy in children.The level IV is appropriated for our study (case series, retrospective).