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In previous reports, hypothyroidism, hypopituitrism, and hypogonadism were common endocrine causes of SCFE, but this is the first time that congenital adrenal hyperplasia has been observed. As such, patients who have undergone long-term endocrine treatment for congenital adrenal hyperplasia could potentially be subjected to a higher risk for SCFE.
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Epiphysiolysis is a relatively common disease in the adolescent population (9-16 years); however, it is rare in the adult population. It is characterized by non-traumatic proximal femur slipping. When it occurs in this population it is associated with some disease that slows sexual development and physis closure, such as endocrine diseases or brain tumors. The aim of the present study is to report a case of epiphysiolysis in a 22-year-old patient with hypogonadotropic hypogonadism. There are only 63 cases reported in the world literature on epiphysiolysis in the adult population.
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OBJECTIVES: The aim of the present study was to investigate whether diagnostic assessment methods used on radiographs in humans with slipped capital femoral epiphysis (SCFE) can be used in cats. METHODS: The ventrodorsal (VD) extended-leg and VD frog-leg pelvic radiographs of 20 cats with SCFE without fully displaced femoral capital epiphyses (FCE), eight cats with fully displaced FCE and five control cats with normal pelvic anatomy were assessed by five observers on two separate occasions 3 months apart. The Klein's line and modified Klein's line were assessed on each VD extended-leg radiograph, and the S-sign was assessed on each VD extended-leg and VD frog-leg radiograph. RESULTS: Excluding cases of fully displaced FCE, the S-sign on the VD frog-leg radiographs more accurately diagnosed SCFE than the S-sign on the VD extended-leg radiographs and the Klein's line (92.4% vs 88.8% vs 60.6%, respectively), and had the greatest sensitivity (93.9% vs 79.2% vs 30.6%, respectively). The S-sign on the VD extended-leg radiographs had greater specificity than the Klein's line and S-sign on the VD frog-leg radiographs (99.2% vs 97.9% vs 90.9%, respectively). The modified Klein's line detected SCFE in 40.2% of cases that were negative for the Klein's line. CONCLUSIONS AND RELEVANCE: The S-sign in both VD extended-leg and VD frog-leg views successfully detected SCFE in cats and can be used to increase early diagnosis and treatment in cats with SCFE that have only subtle radiographic changes.
Assuntos
Doenças do Gato , Escorregamento das Epífises Proximais do Fêmur , Humanos , Gatos , Animais , Escorregamento das Epífises Proximais do Fêmur/diagnóstico por imagem , Escorregamento das Epífises Proximais do Fêmur/veterinária , Fêmur , Radiografia , Diagnóstico Precoce , Epífises , Estudos Retrospectivos , Doenças do Gato/diagnóstico por imagemRESUMO
The knee is an anatomical structure that can provide a great deal of data for research on age estimation. The aim of this study was to evaluate and apply a method for semi-automatic measurements of the area under the growth plate closure of the femur distal epiphysis and the growth plate closure itself on the 2D coronary slices using T2 weighted images (T2WI) generated on magnetic resonance (MRI) devices of different technical and technological characteristics. After the semi-automatic segmentation of the femur distal epiphysis under the growth plate closure and the growth plate closure itself, the areas of the measured closures were calculated using MATLAB version: 9.12. (R2022a), MathWorks Inc., Natick, MA, USA, for each individual coronal slice. The area ratio index (ARI) was calculated as the ratio between the area under the growth plate closure of the femur distal epiphysis and the growth plate closure itself. The study sample consisted of 27 female and 23 male Caucasian participants aged 10 to 26 years. A total of 339 T2WI images were used for ARI calculations. There was a positive correlation between chronological age and the average ARI measured by three independent observers (r = 0.8280, p < 0.001). Multiple regression analysis did not show any significant impact of the technical and technological characteristics of the MRI devices on ARI. The results of this study showed that ARI could serve as a useful tool for age estimation using knee MRI as well as for the further development of artificial intelligence (AI) applications.
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Fibroblast growth factor receptor (FGFR) tyrosine kinase inhibitors (TKIs) are increasingly being used off label in pediatrics. Long-term safety data are limited, and serious toxicities unique to pediatrics may emerge. In a retrospective analysis of patients less than 18 years of age with recurrent/refractory FGFR altered gliomas treated with FGFR TKIs at MSKCC (n = 7), we observed slipped capital femoral epiphyses in three of seven patients along with increased linear growth velocity. Clinicians should closely monitor bone health and have a low index of suspicion for serious orthopedic complications including slipped capital femoral epiphyses and inform patients of related risks as part of consent when treating with FGFR TKIs.
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Pubertal children with significant growth retardation represent a considerable therapeutic challenge. In growth hormone (GH) deficiency, and in those without identifiable pathologies (idiopathic short stature), the impact of using GH is significantly hindered by the relentless tempo of bone age acceleration caused by sex steroids, limiting time available for growth. Estrogen principally modulates epiphyseal fusion in females and males. GH production rates and growth velocity more than double during puberty, and high-dose GH use has shown dose-dependent increases in linear growth, but also can raise insulin-like growth factor I concentrations supraphysiologically, and increase treatment costs. Gonadotropin-releasing hormone analogs (GnRHas) suppress physiologic puberty, and when used in combination with GH can meaningfully increase height potential in males and females while rendering adolescents temporarily hypogonadal at a critical time in development. Aromatase inhibitors (AIs) block androgen to estrogen conversion, slowing down growth plate fusion, while allowing normal virilization in males and stimulating longitudinal bone growth via androgen receptor effects on the growth plate. Here, we review the physiology of pubertal growth, estrogen and androgen action on the epiphyses, and the therapeutic impact of GH, alone and in combination with GnRHa and with AIs. The pharmacology of potent oral AIs, and pivotal work on their efficacy and safety in children is also reviewed. Time-limited use of AIs is a viable alternative to promote growth in pubertal males, particularly combined with GH. Use of targeted growth-promoting therapies in adolescence must consider the impact of sex steroids on growth plate fusion, and treatment should be individualized.
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Inibidores da Aromatase , Hormônio do Crescimento Humano , Masculino , Criança , Feminino , Adolescente , Humanos , Inibidores da Aromatase/farmacologia , Inibidores da Aromatase/uso terapêutico , Hormônio Liberador de Gonadotropina , Androgênios/farmacologia , Transtornos do Crescimento , Puberdade , Estrogênios/uso terapêutico , EsteroidesRESUMO
Hypothalamic hamartomas (HHs) are nonneoplastic mass lesions located in the hypothalamus that can cause central precocious puberty (CPP) and/or gelastic seizures. Seckel syndrome 5 (OMIM210600, SCKL5) is a rare autosomal recessive genetic spectrum disorder characterized by intrauterine growth retardation, proportionate osteodysplastic primordial dwarfism, a wide range of intellectual disability, "bird-headed" facial features, and microcephaly with various structural brain abnormalities. Two siblings presented with short stature and small head circumference and were diagnosed with SCKL 5. The younger sister had HH with CPP and experienced a slipped capital femoral epiphysis during treatment. The 2 siblings had the same genetic variant but showed different phenotypes, which has not been reported previously; this study also as presents the first cases of SCKL5 diagnosed by genetic confirmation in Korea.
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Abstract Epiphysiolysis is a relatively common disease in the adolescent population (9-16 years); however, it is rare in the adult population. It is characterized by non-traumatic proximal femur slipping. When it occurs in this population it is associated with some disease that slows sexual development and physis closure, such as endocrine diseases or brain tumors. The aim of the present study is to report a case of epiphysiolysis in a 22-year-old patient with hypogonadotropic hypogonadism. There are only 63 cases reported in the world literature on epiphysiolysis in the adult population.
Resumo A epifisiólise é uma doença relativamente comum na população adolescente (de 9-16 anos), entretanto rara na população adulta. Se caracteriza pelo escorregamento metáfiso-epifisário do fêmur proximal não-traumático. Quando ocorre nessa população, está associada a alguma doença que retarda o desenvolvimento sexual e fechamento fisário, como doenças endocrinológicas ou tumores cerebrais. O objetivo do presente estudo é relatar um caso de epifisiólise numa paciente com 22 anos de idade e hipogonadismo hipogonadotrófico. Existem apenas 63 casos relatados na literatura mundial sobre epifisiólise na população adulta.
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Humanos , Feminino , Adulto , Síndrome de Kallmann , Epifise Deslocada , Cabeça do FêmurRESUMO
Objective: To compare the clinical outcomes between patients with moderate and severe slipped capital femoral epiphysis (SCFE) treated with osteotomy at the base of neck and osteoplasty and with healthy individuals. Methods: Comparative cohort with 12 patients (14 hips) with moderate and severe SCFE who underwent osteotomy at the base of neck and osteoplasty between 2007 and 2014. The mean age at surgery was 13.3 ± 2.5 years and the mean follow-up was 3.8 ± 2.2 years. We assessed the level of hip pain by the visual analog scale (VAS) and anterior impingement test (AIT); the level of function using the Harris Hip Score (HHS) and 12-Item Short Form Health Survey (SF-12), the range of motion (ROM) by goniometry and Drehmann sign, and the hip muscular strength by isokinetic and Trendelenburg sign. Results: The level of pain was slightly higher in the SCFE cohort compared with healthy hips (VAS, 0.8 ± 1.4 vs 0 ± 0, 0.007; AIT, 14% vs 0%, p = 0.06; respectively). No differences were observed between the SCFE and control cohort for the functional scores (HHS, 94 ± 7 vs 100 ± 1, p = 0.135); except for ROM, with increased internal rotation (37.3º ± 9.4º vs 28.7º ± 8.2º, p < 0.001), and strength, with decreased abduction torque (75.5 ± 36.9 Nm/Kg vs 88.5 ± 27.6 Nm/Kg, p = 0.045) in the SCFE cohort. Conclusion: The osteotomy at the base of neck and the osteoplasty restored the hip motion and muscle strength, except for the abductor strength, to near normal levels, representing a viable option for the treatment of moderate and severe SCFE. Level of Evidence III, Ambidirectional Cohort Study.
Objetivo: Comparar resultados clínicos de pacientes com escorregamento epifisário proximal do fêmur (EEPF) moderado e grave tratados com osteotomia basocervical e cervicoplastia com indivíduos saudáveis. Métodos: Coorte comparativa com 12 voluntários saudáveis e 12 pacientes (14 quadris) com EEPF moderado e grave submetidos à osteotomia basocervical e cervicoplastia entre 2007 e 2014. A média de idade na cirurgia foi de 13,3 ± 2,5 anos e o seguimento médio de 3,8 ± 2,2 anos. Avaliou-se nível de dor no quadril utilizando a escala visual analógica (EVA) e o teste de impacto anterior (TIA); nível de função usando o Harris Hip Score (HHS) e o 12-Item Short Form Health Survey (SF-12); amplitude de movimento (ADM) com goniometria e sinal de Drehmann; e força muscular do quadril com dinamômetro isocinético e sinal de Trendelenburg. Resultados: O nível de dor foi ligeiramente maior na coorte de EEPF comparado a quadris saudáveis (EVA, 0,8 ± 1,4 vs 0 ± 0, 0,007; TIA, 14% vs 0%, p = 0,06; respectivamente). Não foram observadas diferenças entre os grupos EEPF e controle para os escores funcionais (HHS, 94 ± 7 vs 100 ± 1, p = 0,135), exceto para ADM, com aumento da rotação interna (37,3º ± 9,4º vs 28,7º ± 8,2º, p < 0,001), e força, com diminuição do torque de abdução (75,5 ± 36,9 Nm/Kg vs 88,5 ± 27,6 Nm/Kg, p = 0,045), para o grupo EEPF. Conclusão: A osteotomia basocervical e a cervicoplastia restauraram o movimento do quadril e a força muscular, com exceção da força abdutora, a níveis próximos do normal, representando uma opção viável para o tratamento de EEPF moderado e grave. Nível de Evidência III, Estudo de Coorte Ambidirecional.
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Objective: To investigate the efficiency and rates of correction by hemiepiphysiodesis using 8-plate to manage genu valgum deformity in children with skeletal dysplasia. Methods: Eleven children with skeletal dysplasia (three female, eight male; mean age = 10.5 years; age range = 7-13) who underwent temporary hemiepiphysiodesis using 8-plates for genu valgum deformity were retrospectively reviewed. There were nine bilateral cases and two unilateral cases. The mean follow-up time from the index surgery to the final follow-up was 45 (ranging from 24 to 72) months. Radiographical assessment including preoperative and final follow-up measurements of joint orientation angles and mechanical axis deviation (MAD) were conducted. Results: Deformities were completely corrected in nine lower extremities (45%) and partially corrected in seven extremities (35%). In four extremities of two children with Morquio syndrome, MAD worsened. The correction rate of MAD was 1.25 ± 1.62 mm/mo. Conclusion: Though hemiepiphysiodesis using 8-plate requires a longer treatment period, it seems to be an effective treatment for correction of genu valgum in children with skeletal dysplasia. Level of Evidence IV, Case Series.
Objetivo: Investigar a eficiência e as taxas de correção da hemiepifisiodese usando placa-8 no tratamento da deformidade de geno valgo em crianças com displasia esquelética. Métodos: Foram avaliadas retrospectivamente 11 crianças com displasia esquelética (três meninas e oito meninos; idade média = 10,5 anos; faixa etária = 7-13) que foram submetidas à hemiepifisiodese temporária com placa-8 devido à deformidade do geno valgo. Havia nove casos bilaterais e dois casos unilaterais. O acompanhamento médio desde a cirurgia de implante até o acompanhamento final foi de 45 (variação de 24 a 72) meses. Foi feita avaliação radiográfica incluindo medidas de acompanhamento pré e pós-operatórias dos ângulos de orientação da articulação e desvio mecânico do eixo (MAD). Resultados: As deformidades foram completamente corrigidas em nove extremidades inferiores (45%) e parcialmente corrigidas em sete (35%). Em quatro extremidades de duas crianças com síndrome de Morquio, o MAD piorou. A taxa de correção do MAD foi de 1,25 ± 1,62 mm/mês. Conclusão: Embora a hemiepifisiodese com placa-8 necessite de um período de tratamento mais longo, a técnica parece ser um tratamento eficaz para a correção do geno valgo em crianças com displasia esquelética. Nível de Evidência IV, Série de Casos.
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Objective The present study analyzed the incidence of epiphyseal avascular necrosis in patients with slipped capital femoral epiphysis (SCFE) treated using a modified Dunn technique. In addition, this study determined the correlation of other variables with this incidence and described treatment complications. Methods This is a retrospective study with 20 patients treated by the same surgical team from 2009 to 2019 and followed up for 2 to 12 years. The analysis included general features, time from presentation to surgical procedure, classification, and intraoperative blood perfusion of the epiphysis, as well as complications and their treatment. Results All cases were severe; 65% were acute on chronic, and 55% of the SCFEs were unstable. Our complication rate was 45%, with 5 cases of avascular necrosis, 2 cases of deep infection, 1 case of material failure, and 1 case of joint instability. The statistical analysis revealed that the risk of necrosis was higher when the surgery occurred after a long hospitalization time and there was no intraoperative epiphyseal perfusion. Four necrosis cases happened within the first 5 years, and 1 case in the last 5 years of the study. Conclusion Our study showed that necrosis was the most common complication. It also revealed that surgery delay and lack of intraoperative epiphysis perfusion potentially predispose to avascular necrosis. Although with no statistical significance, coxofemoral instability occurred in chronic SCFE, and surgical fixation with threaded wires was less effective than fixation with a cannulated screw. The modified Dunn procedure should be reserved for severe cases in which other techniques are not feasible and performed by an experienced, trained, and qualified team.
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Introduction: Trevor's disease also known as Dysplasia Epiphysealis Hemimelica is an uncommon epiphyseal affliction of childhood, developmental in nature with unknown etiology. It is an osteochondral lesion arising from the epiphyses and progressively increasing in size until skeletal maturity is reached. Surgical management is not advisable in cases, where there is absence of articular symptoms and mass evolution is present, until there is achievement of skeletal maturity. Pain, joint deformity, and impingement mandate surgical intervention and the result is good in cases, where the mass is juxta articular or extra-articular in location. Case Report: We report such a rare case of an 8-year-old male child who presented to us with a hard swelling over ankle with restricted movement and was promptly treated by us with medial malleolus osteotomy and surgical excision of the tumorous mass with subsequent malleolar screw fixation after establishing a diagnosis of osteochondroma of talus. Conclusion: Making a correct diagnosis with imaging and confirmation by histology is necessary, after which careful surgical planning should be carried out in cases, where bony masses are causing pain, ankle mobility hindrance, and impingement with footwear hampering daily activities, this helps prevent recurrence and its further complications.
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Abstract Objective The present study analyzed the incidence of epiphyseal avascular necrosis in patients with slipped capital femoral epiphysis (SCFE) treated using a modified Dunn technique. In addition, this study determined the correlation of other variables with this incidence and described treatment complications. Methods This is a retrospective study with 20 patients treated by the same surgical team from 2009 to 2019 and followed up for 2 to 12 years. The analysis included general features, time from presentation to surgical procedure, classification, and intraoperative blood perfusion of the epiphysis, as well as complications and their treatment. Results All cases were severe; 65% were acute on chronic, and 55% of the SCFEs were unstable. Our complication rate was 45%, with 5 cases of avascular necrosis, 2 cases of deep infection, 1 case of material failure, and 1 case of joint instability. The statistical analysis revealed that the risk of necrosis was higher when the surgery occurred after a long hospitalization time and there was no intraoperative epiphyseal perfusion. Four necrosis cases happened within the first 5 years, and 1 case in the last 5 years of the study. Conclusion Our study showed that necrosis was the most common complication. It also revealed that surgery delay and lack of intraoperative epiphysis perfusion potentially predispose to avascular necrosis. Although with no statistical significance, coxofemoral instability occurred in chronic SCFE, and surgical fixation with threaded wires was less effective than fixation with a cannulated screw. The modified Dunn procedure should be reserved for severe cases in which other techniques are not feasible and performed by an experienced, trained, and qualified team.
Resumo Objetivo Analisar a incidência da necrose avascular da epífise no tratamento do escorregamento da epífise proximal do fêmur pela técnica de Dunn modificada, correlacionando-a com outras variáveis. Como objetivo secundário, descrevemos outras complicações encontradas. Métodos Estudo retrospectivo com 20 pacientes tratados entre 2009 e 2019, com seguimento de 2 a 12 anos, tratados pela mesma equipe cirúrgica. A análise incluiu características gerais, tempo entre apresentação e procedimento cirúrgico, classificação, presença de perfusão sanguínea intraoperatória da epífise, avaliação das complicações e seus respectivos tratamentos. Resultados Todos os casos eram graves, 65% crônicos agudizados e 55% dos escorregamentos eram instáveis. Nossa taxa de complicações foi de 45%, sendo 5 casos de necrose avascular, 2 de infecção profunda, uma falha do material e uma instabilidade articular. Pacientes operados com maior tempo após a internação e os sem perfusão intraoperatória da epífise tiveram maior risco de necrose na análise estatística. Considerando o tempo do estudo, tivemos 4 casos de necrose nos primeiros 5 anos e 1 caso nos últimos 5 anos. Conclusão Nosso estudo demonstrou que a necrose foi a complicação mais comum e que o atraso para a realização da cirurgia e a ausência de perfusão da epífise no intraoperatório podem predispor à necrose avascular. Embora não estatisticamente significante, a instabilidade coxofemoral foi observada na forma de apresentação crônica e a fixação cirúrgica com fios rosqueados se mostrou menos eficaz que a fixação com parafuso canulado. Este procedimento deve ser reservado para casos graves nos quais outras técnicas não sejam possíveis e realizado por equipe experiente, treinada e capacitada.
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Humanos , Masculino , Feminino , Criança , Adolescente , Osteotomia , Estudos Retrospectivos , Necrose da Cabeça do Fêmur , Escorregamento das Epífises Proximais do Fêmur/complicações , NecroseRESUMO
Objective: To assess the complications in patients with severe slipped capital femoral epiphysis treated with the Dunn or modified Dunn procedure from 2012 to 2018 at the Institute of Orthopedics and Traumatology, Medical School, Universidade de São Paulo. Methods: Analysis of medical records, preoperative and postoperative radiographs with at least one year of radiographic follow-up of patients with severe slipped capital femoral epiphysis. Results: We reviewed the complications in 19 operated cases from 2012 to 2018, out of which 36.8% had osteonecrosis of the femoral head, one patient had chondrolysis, and another had postoperative infection. Conclusion: The osteonecrosis rate observed in this series of cases is similar to that described in other orthopedic facilities. We assessed factors that could potentially influence this outcome, including other characteristics of the patient (obesity, endocrine diseases, and history of sports activities) and of the health system. Level of Evidence III, Restropective Case Series.
Objetivo: Avaliar as complicações apresentadas nos pacientes com epifisiolistese grave tratados por meio do procedimento de Dunn ou Dunn Modificado entre 2012 e 2018 no IOT-FMUSP. Métodos: Análise de prontuários, radiografias pré-operatórias e radiografias pós-operatórias com pelo menos 1 ano de seguimento radiográfico dos pacientes com epifisiolistese grave. Resultados: Analisou-se a presença de complicações em 19 casos operados de 2012 a 2018. Desses pacientes, 36,8% apresentaram osteonecrose da cabeça femoral, oi observada condrólise em 1 paciente e infecção pós-operatória em 1 paciente. Conclusão: A taxa de osteonecrose observada nesta série de casos está próxima à descrita em outros serviços. Alguns fatores que potencialmente influenciariam neste desfecho poderiam ter sido avaliados, como outras características do paciente (obesidade, doenças endócrinas e atividade esportiva) e do sistema de saúde. Nível de Evidência III, Série Retrospectiva de Casos .
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ABSTRACT Objective: To compare the clinical outcomes between patients with moderate and severe slipped capital femoral epiphysis (SCFE) treated with osteotomy at the base of neck and osteoplasty and with healthy individuals. Methods: Comparative cohort with 12 patients (14 hips) with moderate and severe SCFE who underwent osteotomy at the base of neck and osteoplasty between 2007 and 2014. The mean age at surgery was 13.3 ± 2.5 years and the mean follow-up was 3.8 ± 2.2 years. We assessed the level of hip pain by the visual analog scale (VAS) and anterior impingement test (AIT); the level of function using the Harris Hip Score (HHS) and 12-Item Short Form Health Survey (SF-12), the range of motion (ROM) by goniometry and Drehmann sign, and the hip muscular strength by isokinetic and Trendelenburg sign. Results: The level of pain was slightly higher in the SCFE cohort compared with healthy hips (VAS, 0.8 ± 1.4 vs 0 ± 0, 0.007; AIT, 14% vs 0%, p = 0.06; respectively). No differences were observed between the SCFE and control cohort for the functional scores (HHS, 94 ± 7 vs 100 ± 1, p = 0.135); except for ROM, with increased internal rotation (37.3º ± 9.4º vs 28.7º ± 8.2º, p < 0.001), and strength, with decreased abduction torque (75.5 ± 36.9 Nm/Kg vs 88.5 ± 27.6 Nm/Kg, p = 0.045) in the SCFE cohort. Conclusion: The osteotomy at the base of neck and the osteoplasty restored the hip motion and muscle strength, except for the abductor strength, to near normal levels, representing a viable option for the treatment of moderate and severe SCFE. Level of Evidence III, Ambidirectional Cohort Study.
RESUMO Objetivo: Comparar resultados clínicos de pacientes com escorregamento epifisário proximal do fêmur (EEPF) moderado e grave tratados com osteotomia basocervical e cervicoplastia com indivíduos saudáveis. Métodos: Coorte comparativa com 12 voluntários saudáveis e 12 pacientes (14 quadris) com EEPF moderado e grave submetidos à osteotomia basocervical e cervicoplastia entre 2007 e 2014. A média de idade na cirurgia foi de 13,3 ± 2,5 anos e o seguimento médio de 3,8 ± 2,2 anos. Avaliou-se nível de dor no quadril utilizando a escala visual analógica (EVA) e o teste de impacto anterior (TIA); nível de função usando o Harris Hip Score (HHS) e o 12-Item Short Form Health Survey (SF-12); amplitude de movimento (ADM) com goniometria e sinal de Drehmann; e força muscular do quadril com dinamômetro isocinético e sinal de Trendelenburg. Resultados: O nível de dor foi ligeiramente maior na coorte de EEPF comparado a quadris saudáveis (EVA, 0,8 ± 1,4 vs 0 ± 0, 0,007; TIA, 14% vs 0%, p = 0,06; respectivamente). Não foram observadas diferenças entre os grupos EEPF e controle para os escores funcionais (HHS, 94 ± 7 vs 100 ± 1, p = 0,135), exceto para ADM, com aumento da rotação interna (37,3º ± 9,4º vs 28,7º ± 8,2º, p < 0,001), e força, com diminuição do torque de abdução (75,5 ± 36,9 Nm/Kg vs 88,5 ± 27,6 Nm/Kg, p = 0,045), para o grupo EEPF. Conclusão: A osteotomia basocervical e a cervicoplastia restauraram o movimento do quadril e a força muscular, com exceção da força abdutora, a níveis próximos do normal, representando uma opção viável para o tratamento de EEPF moderado e grave. Nível de Evidência III, Estudo de Coorte Ambidirecional.
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ABSTRACT Objective: To assess the complications in patients with severe slipped capital femoral epiphysis treated with the Dunn or modified Dunn procedure from 2012 to 2018 at the Institute of Orthopedics and Traumatology, Medical School, Universidade de São Paulo. Methods: Analysis of medical records, preoperative and postoperative radiographs with at least one year of radiographic follow-up of patients with severe slipped capital femoral epiphysis. Results: We reviewed the complications in 19 operated cases from 2012 to 2018, out of which 36.8% had osteonecrosis of the femoral head, one patient had chondrolysis, and another had postoperative infection. Conclusion: The osteonecrosis rate observed in this series of cases is similar to that described in other orthopedic facilities. We assessed factors that could potentially influence this outcome, including other characteristics of the patient (obesity, endocrine diseases, and history of sports activities) and of the health system. Level of Evidence III, Restropective Case Series.
RESUMO Objetivo: Avaliar as complicações apresentadas nos pacientes com epifisiolistese grave tratados por meio do procedimento de Dunn ou Dunn Modificado entre 2012 e 2018 no IOT-FMUSP. Métodos: Análise de prontuários, radiografias pré-operatórias e radiografias pós-operatórias com pelo menos 1 ano de seguimento radiográfico dos pacientes com epifisiolistese grave. Resultados: Analisou-se a presença de complicações em 19 casos operados de 2012 a 2018. Desses pacientes, 36,8% apresentaram osteonecrose da cabeça femoral, oi observada condrólise em 1 paciente e infecção pós-operatória em 1 paciente. Conclusão: A taxa de osteonecrose observada nesta série de casos está próxima à descrita em outros serviços. Alguns fatores que potencialmente influenciariam neste desfecho poderiam ter sido avaliados, como outras características do paciente (obesidade, doenças endócrinas e atividade esportiva) e do sistema de saúde. Nível de Evidência III, Série Retrospectiva de Casos .
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ABSTRACT Objective: To investigate the efficiency and rates of correction by hemiepiphysiodesis using 8-plate to manage genu valgum deformity in children with skeletal dysplasia. Methods: Eleven children with skeletal dysplasia (three female, eight male; mean age = 10.5 years; age range = 7-13) who underwent temporary hemiepiphysiodesis using 8-plates for genu valgum deformity were retrospectively reviewed. There were nine bilateral cases and two unilateral cases. The mean follow-up time from the index surgery to the final follow-up was 45 (ranging from 24 to 72) months. Radiographical assessment including preoperative and final follow-up measurements of joint orientation angles and mechanical axis deviation (MAD) were conducted. Results: Deformities were completely corrected in nine lower extremities (45%) and partially corrected in seven extremities (35%). In four extremities of two children with Morquio syndrome, MAD worsened. The correction rate of MAD was 1.25 ± 1.62 mm/mo. Conclusion: Though hemiepiphysiodesis using 8-plate requires a longer treatment period, it seems to be an effective treatment for correction of genu valgum in children with skeletal dysplasia. Level of Evidence IV, Case Series.
RESUMO Objetivo: Investigar a eficiência e as taxas de correção da hemiepifisiodese usando placa-8 no tratamento da deformidade de geno valgo em crianças com displasia esquelética. Métodos: Foram avaliadas retrospectivamente 11 crianças com displasia esquelética (três meninas e oito meninos; idade média = 10,5 anos; faixa etária = 7-13) que foram submetidas à hemiepifisiodese temporária com placa-8 devido à deformidade do geno valgo. Havia nove casos bilaterais e dois casos unilaterais. O acompanhamento médio desde a cirurgia de implante até o acompanhamento final foi de 45 (variação de 24 a 72) meses. Foi feita avaliação radiográfica incluindo medidas de acompanhamento pré e pós-operatórias dos ângulos de orientação da articulação e desvio mecânico do eixo (MAD). Resultados: As deformidades foram completamente corrigidas em nove extremidades inferiores (45%) e parcialmente corrigidas em sete (35%). Em quatro extremidades de duas crianças com síndrome de Morquio, o MAD piorou. A taxa de correção do MAD foi de 1,25 ± 1,62 mm/mês. Conclusão: Embora a hemiepifisiodese com placa-8 necessite de um período de tratamento mais longo, a técnica parece ser um tratamento eficaz para a correção do geno valgo em crianças com displasia esquelética. Nível de Evidência IV, Série de Casos.
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Teaching point: Age-related variability in endochondral ossification of the femoral condyles in children is a normal variant of skeletal maturation and should not be misdiagnosed as osteochondritis dissecans or any other epiphyseal abnormality.
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Monogenic nephrotic syndrome (NS) is associated with a resistance to initial glucocorticoid therapy and causative variants, which may be found in several genes influencing podocyte stability and kidney development. The TTC21B gene, which encodes the retrograde intraflagellar transport protein IFT139, is found mostly in association with ciliopathies in humans. The role of this protein in podocyte cytoskeleton stability was confirmed later and the mutated TTC21B also may be associated with proteinuric diseases, such as nephrotic syndrome. Our patient manifested as an infant with brachydactyly, nephrotic-range proteinuria, and renal tubular acidosis, and a kidney biopsy revealed focal segmental glomerulosclerosis (FSGS). Multiple phalangeal cone-shaped epiphyses of the hand were seen on X-ray. Next-generation sequencing revealed the well-described p.Pro209Leu heterozygous variant and a novel heterozygous p.Cys14Arg variant in the TTC21B gene. Our finding confirmed that the causative variants in the TTC21B gene may contribute to a spectrum of clinical features, such as glomerular proteinuric disease with tubulointerstitial involvement and skeletal abnormalities.
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BACKGROUND: Previous studies have identified young age as a risk factor for anterior cruciate ligament (ACL) revision. However, few studies have looked separately at pediatric patients and adolescents with regard to outcomes after ACL reconstruction. PURPOSE: To determine whether patient age at ACL reconstruction affects the risk of undergoing revision surgery in young patients. STUDY DESIGN: Cohort study; Level of evidence, 3. METHODS: This study was based on data from the Swedish National Knee Ligament Registry. Patients aged 5 to 35 years who underwent a primary ACL reconstruction with a hamstring tendon autograft between January 1, 2005, and December 31, 2015, were included. The cohort was stratified into different age groups of pediatric patients, adolescents, and young adults to estimate patients with open, recently closed, and closed epiphyses, respectively. The primary endpoint was ACL revision. A multivariable Cox regression model was used to assess the ACL revision rate. The results were expressed as hazard ratios (HRs) and 95% CIs. RESULTS: A total of 36,274 ACL reconstructions were registered during the study period. Of these, 2848 patients were included in the study: 47 pediatric patients (mean age, 13.6 years; range, 9-15 years), 522 adolescents (mean age, 17.4; range, 14-19 years), and 2279 young adults (mean age, 27.0; range, 20-35 years). A total of 31 patients (1.1%) underwent ACL revision within 2 years (0 pediatric patients, 9 adolescents [1.7%], and 22 young adults [1.0%]) and a total of 53 patients (2.6%) underwent ACL revision within 5 years (2 pediatric patients [6.9%], 15 adolescents [3.9%], and 36 young adults [2.2%]). The adolescent age group had a 1.91 times higher rate of ACL revision compared with the young adults (HR = 1.91 [95% CI, 1.13-3.21]; P = .015). There were no differences in revision rates between the pediatric age group and the young adults (HR = 2.93 [95% CI, 0.88-9.79]; P = .081). CONCLUSION: Adolescents had almost twice the rate of revision ACL reconstruction compared with young adults.