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BACKGROUND: Pediatric supracondylar humerus (SCH; AO/OTA13-M/3.1) and medial epicondyle fractures (AO/OTA13u-M/7.1) are common. Concomitant SCH with ipsilateral medial epicondyle fractures remain scarcely reported. We investigated the epidemiology, treatment, and outcomes of this rare, combined injury. METHODS: A retrospective review of pediatric patients with concomitant SCH and medial epicondyle fractures at a level 1 hospital from 2010 to 2020 was performed. Patient data, treatments, and outcomes were assessed. Radiographs were reviewed for fracture classification and alignment. Patients aged above 18 years and those with inaccessible imaging were excluded. Descriptive statistics were performed. RESULTS: Of 3344 patients undergoing surgery for SCH fractures, 14 (6 females, mean: 10.59 y) with concomitant SCH and medial epicondyle fractures were included. Overall, 28.6% of patients exhibited preoperative nerve palsies (3 PIN, 1 median nerve). There was 1 flexion type and 13 Gartland type III SCH fractures. Medial epicondyle fracture displacement averaged 4.13 mm (range: 2 to 7 mm). Thirteen medial epicondyle fractures occurred medial to the physis with 1 through the physis. Eight patients (57.1%) had medial fixation-7 medial pins, 1 medial screw-which captured both the medial epicondyle and medial column of the SCH fracture. Six medial epicondyles were treated closed. The average time to pin pull was 33.1 days (range: 27 to 51 d) with average follow-up of 138.6 days (range: 27 to 574 d). Overall, 50% of patients completed physical therapy (PT). Complications occurred in 4 cases: prominence of a medial pin, 1 patient required additional PT and dynamic splinting for loss of functional extension, 1 patient underwent a manipulation under anesthesia 3.5 months postoperatively for flexion contracture, and 1 patient developed medial epicondyle nonunion and SCH malunion that underwent corrective osteotomy 10.5 months postoperatively. CONCLUSIONS: Concurrent SCH and medial epicondyle fractures exhibited a high rate of nerve palsy (28.6%) and complications (28.6%) and were frequently referred to physical therapy. While patients treated without medial fixation went on to union, this combined injury might represent a relative indication for medial pinning of the SCH fracture. Further studies on this rare injury pattern are needed to determine optimal treatment methods. LEVEL OF EVIDENCE: Level IV-therapeutic.
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Fracturas del Húmero , Humanos , Estudios Retrospectivos , Fracturas del Húmero/cirugía , Femenino , Niño , Masculino , Adolescente , Incidencia , Resultado del Tratamiento , Preescolar , Fijación Interna de Fracturas/métodosRESUMEN
OBJECTIVE: Chat generative pre-trained transformer (ChatGPT) has garnered attention in health care for its potential to reshape patient interactions. As patients increasingly rely on artificial intelligence platforms, concerns about information accuracy arise. In-toeing, a common lower extremity variation, often leads to pediatric orthopaedic referrals despite observation being the primary treatment. Our study aims to assess ChatGPT's responses to pediatric in-toeing questions, contributing to discussions on health care innovation and technology in patient education. METHODS: We compiled a list of 34 common in-toeing questions from the "Frequently Asked Questions" sections of 9 health care-affiliated websites, identifying 25 as the most encountered. On January 17, 2024, we queried ChatGPT 3.5 in separate sessions and recorded the responses. These 25 questions were posed again on January 21, 2024, to assess its reproducibility. Two pediatric orthopaedic surgeons evaluated responses using a scale of "excellent (no clarification)" to "unsatisfactory (substantial clarification)." Average ratings were used when evaluators' grades were within one level of each other. In discordant cases, the senior author provided a decisive rating. RESULTS: We found 46% of ChatGPT responses were "excellent" and 44% "satisfactory (minimal clarification)." In addition, 8% of cases were "satisfactory (moderate clarification)" and 2% were "unsatisfactory." Questions had appropriate readability, with an average Flesch-Kincaid Grade Level of 4.9 (±2.1). However, ChatGPT's responses were at a collegiate level, averaging 12.7 (±1.4). No significant differences in ratings were observed between question topics. Furthermore, ChatGPT exhibited moderate consistency after repeated queries, evidenced by a Spearman rho coefficient of 0.55 ( P = 0.005). The chatbot appropriately described in-toeing as normal or spontaneously resolving in 62% of responses and consistently recommended evaluation by a health care provider in 100%. CONCLUSION: The chatbot presented a serviceable, though not perfect, representation of the diagnosis and management of pediatric in-toeing while demonstrating a moderate level of reproducibility in its responses. ChatGPT's utility could be enhanced by improving readability and consistency and incorporating evidence-based guidelines. LEVEL OF EVIDENCE: Level IV-diagnostic.
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Educación del Paciente como Asunto , Humanos , Niño , Reproducibilidad de los Resultados , Educación del Paciente como Asunto/métodos , Inteligencia Artificial , Pediatría/métodosRESUMEN
PURPOSE: Hip dysplasia reportedly occurs in up to 12% of the general population and may be higher in patients with adolescent idiopathic scoliosis (AIS). When using lateral center edge angle (LCEA) measurements to help identify hip dysplasia, it is uncertain if this measurement can be performed reliably on scoliosis radiographs due to parallax. The purpose of this study is to evaluate the reliability of measuring LCEA on scoliosis radiographs compared with standard pelvis radiographs. METHODS: This study evaluated 40 hips on 20 patients (mean age 12.5 years ± 3.1; 50% Female) who received PA scoliosis radiographs and AP pelvis radiographs within 1 year of each other. Review was performed by 4 pediatric radiologists (2 general, 2 MSK), 2 pediatric orthopaedic surgeons, and 1 research fellow. Radiographic measurement of the LCEA for each hip was first performed on the scoliosis radiograph. After a minimum of 3 days, the LCEA was measured on a pelvis radiograph of the same individual obtained within 1 year of the scoliosis radiograph. Pearson coefficient was used to measure agreement between scoliosis and pelvis radiograph measurements. Intraclass correlation coefficient (ICC) was used to evaluate intraobserver and interobserver agreement. ICC values <0.5 were classified as poor reliability, 0.5 to 0.75 were classified moderate, 0.75 to 0.90 were classified good, and >0.9 indicated excellent reliability. RESULTS: The mean difference between scoliosis and pelvis radiographs was 54 ± 79 days. There was good-to-excellent interobserver agreement on LCEA measurements made between readers on scoliosis radiographs (ICC: 0.94, 95% CI: 0.90-0.96, P <0.001) and pelvis radiographs (ICC: 0.91, 95% CI: 0.83-0.95, P <0.001), and moderate-to-excellent intraobserver agreement for scoliosis radiographs (ICC range: 0.68 to 0.98; P <0.001) and pelvis radiographs (ICC range: 0.62 to 0.96; P <0.001). There was a strong correlation between LCEA measurements made on scoliosis and pelvis radiographs ( r2 =0.66, P <0.001), and the intermodality agreement between scoliosis and pelvis radiograph LCEA measurements were moderate to good (ICC range: 0.68 to 0.89, P <0.001). CONCLUSIONS: Overall, there was good-to-excellent agreement between readers on scoliosis and pelvis radiographs, respectively, and moderate-to-excellent intraobserver agreement between LCEA measurements made on scoliosis radiographs and pelvis radiographs, respectively. LCEA measurements made on scoliosis radiographs strongly correlated to the measurements made on pelvis radiographs, and the intermodality ICC was also considered moderate to good. Dedicated pelvis radiographs may not be necessary during scoliosis workup and follow-up surveillance, thereby decreasing radiation exposure, cost, and improving patient care workflow. LEVEL OF EVIDENCE: Level IV-diagnostic study.
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Variaciones Dependientes del Observador , Radiografía , Escoliosis , Humanos , Escoliosis/diagnóstico por imagen , Femenino , Adolescente , Reproducibilidad de los Resultados , Masculino , Niño , Radiografía/métodos , Acetábulo/diagnóstico por imagen , Luxación de la Cadera/diagnóstico por imagen , Estudios RetrospectivosRESUMEN
OBJECTIVE: To assess the clinical impact of diagnostic musculoskeletal (MSK) injections on treatment decision-making in adolescent and adult patients at a children's hospital. MATERIALS AND METHODS: Retrospective study in patients who underwent diagnostic MSK injections by fluoroscopy or ultrasound (US) between 8/2020 and 3/2023 at a children's hospital. Patients received ropivacaine and triamcinolone acetonide at pain site, reporting quantitative FACES pain score prior to, immediately following, and 2-3 days following injection. Impact on patient care was subsequently assessed. RESULTS: A total of 109 diagnostic fluoroscopic or US MSK injection referrals (mean: 17.6 years old) were included, most commonly hip (76.2%), ankle (9.2%), and iliopsoas tendon sheath (8.3%). Pain improvement occurred in 89.0% immediately and 67.9% 2-3 days after MSK injection, with net 84.4% exhibiting improvement based on pain scores and clinical exams. When there was pain improvement at the site of injection, there was a statistically higher incidence of operative intervention or additional therapeutic injections compared with the cohort that did not have symptom improvement (88% versus 35.3%, P < 0.0001). For the 15.6% (N = 17) of referrals that did not have pain improvement, 17.6% (n = 3) ultimately had an operative intervention at a separate site from the diagnostic injection, as an alternative etiology for the pain was found. CONCLUSION: Image-guided MSK injections play an important role in the management of musculoskeletal disorders. 84.4% of referrals experienced symptom relief, improving confidence for treatment decision-making. Importantly, 15.6% of patients were found to have an alternative etiology for symptoms, altering management altogether.
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Anestésicos Locales , Hospitales Pediátricos , Dolor Musculoesquelético , Dimensión del Dolor , Ultrasonografía Intervencional , Humanos , Adolescente , Femenino , Masculino , Dolor Musculoesquelético/tratamiento farmacológico , Dolor Musculoesquelético/diagnóstico por imagen , Estudios Retrospectivos , Ultrasonografía Intervencional/métodos , Anestésicos Locales/administración & dosificación , Adulto , Fluoroscopía , Ropivacaína/administración & dosificación , Triamcinolona Acetonida/administración & dosificación , Niño , Radiografía Intervencional/métodosRESUMEN
This review describes our institution's standardized technique as well as potential pitfalls for therapeutic steroid injections in children with symptomatic neuromuscular hip dysplasia. Symptomatic, painful neuromuscular hip dysplasia can dramatically affect quality of life. Steroid injections are used to identify the source of perceived pain, temporarily treat pain while awaiting surgical intervention, or for therapeutic management for nonoperative hip joints.
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Cuidados Paliativos , Humanos , Cuidados Paliativos/métodos , Niño , Inyecciones Intramusculares/métodos , Enfermedades Neuromusculares/diagnóstico por imagen , Enfermedades Neuromusculares/complicaciones , Enfermedades Neuromusculares/terapia , Inyecciones Intraarticulares , Masculino , Femenino , Preescolar , Luxación de la Cadera/diagnóstico por imagen , Luxación de la Cadera/terapiaRESUMEN
Acetabular underdevelopment (acetabular dysplasia) is a common finding in children with hip dislocation, and residual acetabular dysplasia can remain after hip reduction. Residual dysplasia leads to unsatisfactory long-term outcomes and osteoarthritis. Dynamics of acetabular dysplasia [measured as Acetabular Index (AI)] in a pediatric cohort that underwent open (OR) or closed reduction are reported. Retrospective data from six tertiary pediatric orthopedic centers were gathered. Hips were classified as having 'Critical', 'Monitoring', or 'Normal' acetabular dysplasia based on age-adjusted normative AI measurements. From 193 hips, 108 (56%) underwent open reduction. Children younger than 24 months had a strong AI decline but children > 24 months did not. Among 78 hips with critical dysplasia at time of OR, 36 (46.2%) remained critical and 19 (24.4%) underwent an acetabular osteotomy (AO) during follow-up. CR hips had a similar AI decline in patients younger and older than 12 months. Among 51 hips with critical dysplasia at the time of CR, 13 (25.5%) remained critical and 21 (41.2%) underwent AO during follow-up. Acetabular dysplasia improves with AI decreasing in children who undergo OR and CR under the age of 2 years with slower acetabular remodeling afterwards. Around 2/3 of patients with AI in the critical range at CR or OR either underwent AO or had significant acetabular dysplasia at final follow-up. Our data supports considering simultaneous AO at the time of OR for hips with AI in the critical range or children who undergo hip open reduction after 24 months of age. Level of Evidence: Level III.
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INTRODUCTION: Adolescent idiopathic scoliosis radiographic screening will often include the hips. The purpose of this study is to evaluate the prevalence of hip dysplasia by lateral center edge angle (LCEA) on scoliosis radiographs in patients presenting with severe range (>45 deg) idiopathic curves. METHODS: We reviewed 200 consecutive patients (400 hips) with idiopathic scoliosis with major coronal curves ≥45 degrees between October 2009 and October 2022. The presenting scoliosis radiograph PA were reviewed for the following variables: major coronal curve direction, coronal balance, and lateral center edge angles. An LCEA value ≤18 was considered dysplastic. To assess potential measurement variability related to parallax of the scoliosis radiographs, the intermodality intraclass correlation coefficient (ICC) was measured from a subset of 20 hip LCEA values from patients with both scoliosis and pelvis radiographs. RESULTS: A total of 200 patients [mean age: 13.6 (1.8) years; Female: 79%] were reviewed. The mean major curve (SD) was 58.6 degrees (11.4 deg). Overall 19% (37/200) patients or 11% (43/400) of hips had hip dysplasia. Of the dysplastic hips, 16% (6/43, P <0.001) were bilateral and 57% (21/43, P =0.04) were right sided. There was no association between dysplastic hip laterality and ipsilateral or contralateral coronal balance ( P =0.26) or curve direction ( P =0.49). The interobserver ICC for assessment of LCEA on scoliosis radiographs was 0.85, and the intermodality ICC between pelvic and scoliosis radiographs was 0.78. CONCLUSION: Hip dysplasia was present in 19% of patients presenting with large major curves, and many of these patients had right sided hip dysplasia. There was no association between hip dysplasia laterality and the patient's major curve direction or coronal balance. There was good intermodality reliability (ICC=0.78) between LCEA values in patients who had both scoliosis and pelvis radiographs, and good inter-rater reliability between raters. Radiographic interpretation of adolescent idiopathic scoliosis should focus not only on the description of the curve and ruling out underlying segmentation anomalies but should also include critical assessment of the hips to exclude co-existing hip dysplasia. LEVEL OF EVIDENCE: Therapeutic study-level IV.
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Luxación Congénita de la Cadera , Luxación de la Cadera , Escoliosis , Adolescente , Femenino , Humanos , Acetábulo , Luxación de la Cadera/diagnóstico por imagen , Luxación de la Cadera/etiología , Luxación Congénita de la Cadera/complicaciones , Luxación Congénita de la Cadera/diagnóstico por imagen , Luxación Congénita de la Cadera/epidemiología , Articulación de la Cadera , Osteotomía , Reproducibilidad de los Resultados , Estudios Retrospectivos , Escoliosis/diagnóstico por imagen , MasculinoRESUMEN
The objective of this study was to analyze a multicenter cohort of children with developmental dysplasia of the hip (DDH) who underwent treatment with closed reduction. We sought to report the effects that severity of hip dysplasia and age have on the development of femoral head avascular necrosis (AVN) and the need for additional procedures. All patients with DDH and minimum 2 years of follow-up who underwent closed reduction were identified. The following variables were recorded: sex, laterality of hip involvement, age, acetabular index (AI), and International Hip Dysplasia Institute (IHDI) grade. The effects of patient age and pre-procedure IHDI grade on the rate of AVN and need for additional procedures after the closed reduction were analyzed using an alpha of 0.05. Seventy-eight total hips were included in the final analysis. The average patient age was 12 months. AVN of the femoral head was reported in 24 hips (30.8%) and 32 hips (41.0%) required additional surgery. Higher pre-op IHDI grade was associated with higher risk of developing Bucholz-Ogden grades II-IV AVN of the femoral head (P = 0.025) and requiring additional surgery (P= 0.033) regardless of patient age. There were no statistically significant differences for the effect of age on the measured outcomes (Pâ >â 0.05). These findings suggest that severity of dislocation (IHDI grade) is a significant risk factor for the development of AVN and need for additional procedure.
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BACKGROUND: Fractures involving the anterolateral distal tibia, often referred to as Tillaux or Triplane fractures, are often treated operatively to achieve an anatomic reduction of the ankle joint articular surface. Although axial images from computed tomography scans are commonly obtained to measure displacement, no studies have examined these fracture patterns in the axial plane. This information may be useful to guide screw trajectory with only fluoroscopic imaging intraoperatively. METHODS: We queried an institutional radiology report database for all "Tillaux" or "triplane" fractures and manually reviewed to identify fractures with an anterolateral tibia fragment. The axial image immediately caudal to the physis or physeal scar was used for measurements. The angle between the incisura and the fracture line exiting the incisura was measured. The width of the fracture fragment and the anterior distal tibia was then measured perpendicular to the incisura, representing the width that would be seen on an intraoperative mortise. A cluster analysis was performed to identify fracture patterns. RESULTS: The average age of patients included in the study was 16 ± 2 years. Sixty-nine patients met inclusion criteria 32 patients with Tillaux fractures (46.4%) and 37 patients with triplane fractures (53.6%) met study inclusion criteria for a total of 69 patients. The cluster analysis demonstrated 3 fracture patterns. For type 1 (n=16), the mean was 24.5 ± 6.5 degrees from the incisura and the mean distance from the tibiofibular joint was 21.1 ± 6.3%. For type 2 (n=20), the mean was 58.6 ± 7.2 degrees and the mean distance was 48.7 ± 8.9%. For type 3 (n=33), the mean was 88.6 ± 6.3 degrees and the mean distance was 49.1 ± 9.7%. CONCLUSIONS: This is the first study to identify that pediatric distal tibia physeal fractures occur in 3 common patterns with consistent fragment sizes and fracture planes. This information can optimize screw start point and trajectory in the axial plane when referencing a mortise x-ray and C-arm beam. LEVEL OF EVIDENCE: Diagnostic, Level III.
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BACKGROUND: In addition to the clinical exam, several quantitative measurement tools are performed on pelvic radiographs in the assessment of adolescent hip dysplasia at most dedicated pediatric hip preservation clinics, with the most commonly used measurement called the lateral center edge angle (LCEA). However, most pediatric radiologists do not use these quantitative measuring tools and instead make the diagnosis of adolescent hip dysplasia based on subjective review. OBJECTIVE: The purpose of this study is to determine the additive value of a measurement-based diagnosis of adolescent hip dysplasia using LCEA versus subjective radiographic interpretation by pediatric radiologists. METHODS: A review of pelvic radiographs for the binomial diagnosis of hip dysplasia was performed by four pediatric radiologists (two general, two musculoskeletal). The review included 97 pelvic AP radiographs (mean age 14.4 years [range 10-20 years], 81% female) for a total of 194 hips (58 cases of adolescent hip dysplasia and 136 normal) all of whom were evaluated in a tertiary care pediatric subspecialty hip preservation clinic. Subjective radiographic interpretation of each hip for a binomial diagnosis of hip dysplasia was performed. 2 weeks later and without knowledge of the subjective radiographic interpretation results, the same review was performed with LCEA measurement and a diagnosis of hip dysplasia made when LCEA angles were under 18 degrees. A comparison of sensitivity/specificity between methods per reader was conducted. A comparison of accuracy between methods for all readers combined was performed. RESULTS: For all four reviewers, the sensitivity of subjective versus LCEA measurement-based diagnosis of hip dysplasia was 54-67% (average 58%) versus 64-72% (average 67%), respectively, and specificity was 87-95% (average 90%) versus 89-94% (average 92%), respectively. All four readers demonstrated an intra-reader trend for improvement in the diagnosis of adolescent hip dysplasia after the addition of LCEA measurements but was only statistically significant in one of the four readers. The combined accuracy of all four readers for subjective and LCEA measurement-based interpretation was 81% and 85%, respectively with p = 0.006. CONCLUSION: Compared with subjective interpretation, LCEA measurements demonstrate increased diagnostic accuracy amongst pediatric radiologists for the correct diagnosis of adolescent hip dysplasia.
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Luxación de la Cadera , Adolescente , Adulto , Niño , Femenino , Humanos , Masculino , Adulto Joven , Acetábulo , Artroscopía , Luxación de la Cadera/diagnóstico por imagen , Articulación de la Cadera/diagnóstico por imagen , Radiografía , Estudios RetrospectivosRESUMEN
AIM: The COVID-19 pandemic drastically altered human behaviour and socialisation and may have created an environment that could lead to increased incidence of domestic abuse and non-accidental trauma, or child physical abuse (CPA). Initial reports about the effect of the COVID-19 pandemic on the rates of CPA have been mixed. The purpose of this study is to describe the effects of COVID-19 on rates of CPA in a large metropolitan paediatric hospital and level I paediatric trauma centre. METHODS: We identified and compared all CPA admissions under 18 years from May 2019 to February 2020 and considered that to be the pre-COVID time frame. The ensuing 12-month period of March 2020 to February 2021 was considered to be the intra-COVID time frame. RESULTS: There were 49 (0.32%) unique CPA patients pre-COVID and 83 (0.85%) unique CPA patients intra-COVID (P < 0.001) with lower total admissions for any reason during the intra-COVID time frame. Monthly CPA cases were increased (P < 0.03) during the intra-COVID time period (mean 6.9, 95% confidence interval: 5.8-12.7) compared to the pre-COVID time period (mean 4.9, 95% confidence interval: 3.3-8.2). CONCLUSION: During the COVID-19 pandemic, there were decreased overall hospital admissions in the period of mandated shutdowns and isolation. However, we saw an increased rate of CPA admissions compared to the time period prior to the pandemic. Knowledge of such data, trends and circumstances will help keep health-care providers alert and vigilant in identifying children at risk for maltreatment, and may impact child abuse protocols and guidelines.
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COVID-19 , Maltrato a los Niños , Humanos , Niño , Adolescente , Abuso Físico , Pandemias , COVID-19/epidemiología , Texas/epidemiología , Estudios RetrospectivosRESUMEN
BACKGROUND: Open reduction of the hip is commonly performed in children with severe developmental dysplasia of the hip, or in cases that are refractory to nonoperative forms of treatment. The open reduction has been associated with numerous complications including avascular necrosis (AVN) of the femoral head, the need for reoperation, and residual radiographic dysplasia. This study seeks to determine the effects of preoperative severity of dysplasia, associated procedures (femoral and acetabular osteotomies), age on AVN, and the need for reoperation. METHODS: Children with developmental dysplasia of the hip and a minimum of 2 years of follow-up who underwent open reduction were identified. The following data points were recorded: sex, laterality of hip involvement, simultaneous procedures, surgical approach used, age, acetabular index, and International Hip Dysplasia Institute grade. We analyzed the effects of preoperative International Hip Dysplasia Institute, age, surgical approach (anterior/medial), bilateral reduction, and simultaneous femoral shortening or pelvic osteotomy on the outcomes of AVN and reoperation. RESULTS: One hundred eighty-five hips in 149 patients were included in this study with an average follow-up of 4 years (range: 2 to 5 y). The average age at index surgery was 23 months (range: 1 to 121 mo). Overall, 60 hips (32.4%) required secondary surgical procedures at an average age of 58.5 months. High-grade AVN was noted in 24 hips (13.0%) and was found to be associated with the severity of the hip dislocation ( P = 0.02). A higher rate of reoperation was found in children over 18 months at the time of open reduction who did not receive an acetabular osteotomy ( P = 0.012). CONCLUSION: Approximately 1/3 of patients require another operative intervention within the first 4 years after open reduction of the hip. We found the severity of hip dislocation to be associated with a higher risk of AVN development. These findings support performing an acetabular osteotomy in children over 18 months of age at the time of open reduction to decrease the likelihood of requiring future reoperation during the first 4 years after the index procedure. LEVEL OF EVIDENCE: Level III.
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Displasia del Desarrollo de la Cadera , Luxación Congénita de la Cadera , Luxación de la Cadera , Osteonecrosis , Humanos , Niño , Lactante , Preescolar , Luxación de la Cadera/cirugía , Displasia del Desarrollo de la Cadera/cirugía , Resultado del Tratamiento , Estudios Retrospectivos , Luxación Congénita de la Cadera/cirugía , Osteotomía/métodos , Osteonecrosis/cirugíaRESUMEN
BACKGROUND: Collagen VI-related myopathies with pathologic COL6A1, COL6A2, and COL6A3 variants manifest as a phenotypic continuum of rare disorders, including Bethlem myopathy (BM), characterized by early onset muscle weakness, proximal joint contractures, and distal joint laxity. Herein we discuss the concomitant orthopedic manifestations of BM, potential management strategies, and patient outcomes. METHODS: An IRB-approved retrospective cohort study (n=23) from 2 pediatric institutions with a confirmed diagnosis of BM. Charts were reviewed for demographic data, age of disease presentation and diagnosis, COL6 genotype, diagnosis method, ambulation status, need for assistance, musculoskeletal abnormalities, other systemic comorbidities, advanced imaging and screening diagnostics, previous surgical interventions, and progression of the disease. RESULTS: The mean age was 11.65 years (range 3 to 19 y). Mean age at initial presentation with symptoms was 4.18 years old, whereas diagnosis was delayed until 8.22 years old on average. Muscle weakness was the most common presenting symptom (65.2%), and 73.9% of patients required some use of assistive or mobility devices. Overall, 30.4% of patients were diagnosed with scoliosis; 57.1% required operative intervention for their scoliosis; 43.5% of patients had acetabular dysplasia; 10% required open reduction of a dislocated hip; 10% required closed reduction with hip spica application; 10% required bilateral periacetabular osteotomies for instability; 91.3% of patients developed foot and ankle deformities; 33.3% of patients underwent posteromedial-lateral equinovarus releases; 28.6% required an Achilles tendon lengthening, and 86.9% of patients had muscle tendon contractures, the most common locations being the ankle (55%) and elbow (40%). CONCLUSION: Although often less severe than other more common neuropathies and myopathies like Charcot-Marie-Tooth disease and Duchenne muscular dystrophy, BM does lead to progressive musculoskeletal deformity and disability. Its relative rarity makes it less familiar to providers and likely contributes to delays in diagnosis. Scoliosis, hip dysplasia, and equinus and varus ankle deformities are the most common musculoskeletal deformities. Physicians and surgeons should appropriately counsel patients and families about the clinical course of this disorder and the potential need for mobility assistance or surgical procedures. LEVEL OF EVIDENCE: III, Prognostic. study.
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Contractura , Distrofia Muscular de Duchenne , Escoliosis , Humanos , Niño , Preescolar , Adolescente , Adulto Joven , Adulto , Estudios Retrospectivos , Mutación , Colágeno Tipo VI/genética , Contractura/etiología , Contractura/cirugía , Debilidad Muscular , Progresión de la EnfermedadRESUMEN
BACKGROUND AND OBJECTIVES: The management of intraoperative blood loss in the surgical treatment of paediatric hip dysplasia is resource intensive. There are numerous clinical factors that impact the need for intraoperative transfusion. Identification of patient and surgical factors associated with increased blood loss may reduce the unnecessary use of resources. This study aimed to identify factors predictive of intraoperative transfusion in children undergoing hip dysplasia surgery. MATERIALS AND METHODS: This is a single-centre retrospective review of patients undergoing surgery for hip dysplasia from 1 January 2012 to 15 April 2021. Patient demographic factors, anaesthetic, surgical and transfusion histories were reviewed. Multivariable logistic regression analysis was performed to identify factors predictive of allogeneic red blood cell transfusion requirements during the intraoperative period. RESULTS: This study includes 595 patients who underwent open surgery for hip dysplasia, including 297 (52.6%) classified as developmental dysplasia (DD) and 268 (47.3%) as neuromuscular (NM) with a mean age of 9.1 years (interquartile range 3-14). Intraoperative allogeneic transfusion was identified in 26/297 (8.8%) DD and 73/268 (27.2%) NM patients. Adjusted factors associated with increased odds of intraoperative transfusion were NM (odds ratio [OR] = 2.96, 95% confidence interval [CI] [1.76, 5.00]) and the number of osteotomies performed (OR = 1.82/osteotomy, 95% CI [1.40, 2.35]). Adjusted factors that reduced the odds of transfusion were the use of antifibrinolytics (OR = 0.35, 95% CI [0.17, 0.71]) and regional anaesthesia (OR = 0.52, 95% CI [0.29, 0.94]). CONCLUSION: For children undergoing surgery for hip dysplasia, the number of osteotomies performed is predictive of the need for allogeneic blood transfusion. Antifibrinolytics and regional anaesthesia are associated with reduced risk for allogeneic blood transfusion. Blood management initiatives, such a preoperative optimization of haemoglobin and the use of antifibrinolytics, could target patients at increased risk of intraoperative bleeding and transfusion.
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Antifibrinolíticos , Luxación de la Cadera , Humanos , Niño , Luxación de la Cadera/tratamiento farmacológico , Luxación de la Cadera/etiología , Transfusión Sanguínea , Pérdida de Sangre Quirúrgica/prevención & control , Transfusión de Eritrocitos/efectos adversos , Estudios Retrospectivos , Factores de RiesgoRESUMEN
In situ fixation of slipped capital femoral epiphysis (SCFE) results in residual deformity that can cause femoroacetabular impingement (FAI). It is unknown what factors could help differentiate patients who are more likely to become symptomatic. We performed a retrospective review of 55 hips treated with in situ pinning for SCFE and subsequent secondary deformity correction procedure for symptomatic FAI and compared them to 39 asymptomatic hips with SCFE deformity using multivariable analysis. Case patients were slightly older than controls (12.6 vs 11.3 years, p = 0.0002) but had similar BMI. The mean epiphyseal-diaphyseal angle was 56° in cases versus 44° in controls (p = 0.0019). Cases were significantly more likely to have obligate external rotation with hip flexion, external foot progression, flexion <90°, antalgic limp, and Trendelenburg lurch. On radiographs, most cases had a head-neck offset ≤0 mm, a distinct metaphyseal corner prominence, acetabular retroversion, and an alpha angle ≥60°. Most controls also had head-neck offset ≤0 mm. Pre-pinning, older age (OR = 1.98 per year, p = 0.0016) and initial epiphyseal-diaphyseal angle (OR = 1.04 per degree, p = 0.018) significantly increased the odds of having symptomatic FAI. Post-pinning, external foot progression increased the odds of symptomatic FAI by 10.48 (p = 0.017), and an alpha angle ≥60° resulted in 11.4 times higher odds of symptomatic FAI (p = 0.011). The linear correlation between epiphyseal-diaphyseal and alpha angle was poor (r = 0.28). Older age and initial epiphyseal-diaphyseal pre-pinning mildly increased the odds of eventual symptomatic FAI. This information can help the surgeon to predict which patients may develop symptomatic FAI.
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BACKGROUNDCurrently, there is no disease-specific therapy for osteogenesis imperfecta (OI). Preclinical studies demonstrate that excessive TGF-ß signaling is a pathogenic mechanism in OI. Here, we evaluated TGF-ß signaling in children with OI and conducted a phase I clinical trial of TGF-ß inhibition in adults with OI.METHODSHistology and RNA-Seq were performed on bones obtained from children. Gene Ontology (GO) enrichment assay, gene set enrichment analysis (GSEA), and Ingenuity Pathway Analysis (IPA) were used to identify dysregulated pathways. Reverse-phase protein array, Western blot, and IHC were performed to evaluate protein expression. A phase I study of fresolimumab, a TGF-ß neutralizing antibody, was conducted in 8 adults with OI. Safety and effects on bone remodeling markers and lumbar spine areal bone mineral density (LS aBMD) were assessed.RESULTSOI bone demonstrated woven structure, increased osteocytes, high turnover, and reduced maturation. SMAD phosphorylation was the most significantly upregulated GO molecular event. GSEA identified the TGF-ß pathway as the top activated signaling pathway, and IPA showed that TGF-ß1 was the most significant activated upstream regulator mediating the global changes identified in OI bone. Treatment with fresolimumab was well-tolerated and associated with increases in LS aBMD in participants with OI type IV, whereas participants with OI type III and VIII had unchanged or decreased LS aBMD.CONCLUSIONIncreased TGF-ß signaling is a driver pathogenic mechanism in OI. Anti-TGF-ß therapy could be a potential disease-specific therapy, with dose-dependent effects on bone mass and turnover.TRIAL REGISTRATIONClinicalTrials.gov NCT03064074.FUNDINGBrittle Bone Disorders Consortium (U54AR068069), Clinical Translational Core of Baylor College of Medicine Intellectual and Developmental Disabilities Research Center (P50HD103555) from National Institute of Child Health and Human Development, USDA/ARS (cooperative agreement 58-6250-6-001), and Sanofi Genzyme.
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Osteogénesis Imperfecta , Adulto , Densidad Ósea , Huesos/metabolismo , Niño , Humanos , Vértebras Lumbares/metabolismo , Osteogénesis Imperfecta/tratamiento farmacológico , Osteogénesis Imperfecta/genética , Factor de Crecimiento Transformador beta/genética , Factor de Crecimiento Transformador beta/metabolismoRESUMEN
BACKGROUND: The vast majority of pediatric distal-third tibial shaft fractures can be treated with closed reduction and casting. If conservative measures fail, then these fractures are usually treated with 2 antegrade flexible intramedullary nails. A postoperative cast is usually applied because of the tenuous fixation of the 2 nails. Recent studies have described the use of 4 nails to increase the stability of the fixation, a technique that may preclude the need for postoperative casting. The purpose of this biomechanical study is to quantify the relative increase in stiffness and load to failure when using 4 versus 2 nails to surgically stabilize these fractures. METHODS: Short, oblique osteotomies were created in the distal third of small fourth-generation tibial sawbones and stabilized with 2 (double) or 4 (quadruple) flexible intramedullary nails. After pilot testing, 5 models per fixation method were tested cyclically in axial compression, torsion, and 4-point bending in valgus and recurvatum. At the end of the study, each model was loaded to failure in valgus. Stiffness values were calculated, and yield points were recorded. The data were compared using Student's t tests. Results are presented as mean±SD. The level of significance was set at P≤0.05. RESULTS: Stiffness in valgus 4-point bending was 624±231 and 336±162 N/mm in the quadruple-nail and double-nail groups, respectively (P=0.04). There were no statistically significant differences in any other mode of testing. CONCLUSIONS: The quadruple-nail construct was almost 2 times as stiff as the double-nail construct in resisting valgus deformation. This provides biomechanical support for a previously published study describing the clinical success of this fixation construct.
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Clavos Ortopédicos , Fijación Intramedular de Fracturas/instrumentación , Tibia/lesiones , Fracturas de la Tibia/cirugía , Fenómenos Biomecánicos , Niño , Diáfisis/lesiones , Diáfisis/cirugía , Fijación Intramedular de Fracturas/métodos , Humanos , Masculino , Diseño de Prótesis , Tibia/cirugía , Fracturas de la Tibia/fisiopatologíaRESUMEN
OBJECTIVE: Adequate resources are required to rapidly diagnose and treat pediatric musculoskeletal infection (MSKI). The workload MSKI consults contribute to pediatric orthopaedic services is unknown as prior epidemiologic studies are variable and negative work-ups are not included in national discharge databases. The hypothesis was tested that MSKI consults constitute a substantial volume of total consultations for pediatric orthopaedic services across the United States. STUDY DESIGN: Eighteen institutions from the Children's ORthopaedic Trauma and Infection Consortium for Evidence-based Study (CORTICES) group retrospectively reviewed a minimum of 1 year of hospital data, reporting the total number of surgeons, total consultations, and MSKI-related consultations. Consultations were classified by the location of consultation (emergency department or inpatient). Culture positivity rate and pathogens were also reported. RESULTS: 87,449 total orthopaedic consultations and 7,814 MSKI-related consultations performed by 229 pediatric orthopaedic surgeons were reviewed. There was an average of 13 orthopaedic surgeons per site each performing an average of 154 consultations per year. On average, 9% of consultations were MSKI related and 37% of these consults yielded positive cultures. Finally, a weak inverse monotonic relationship was noted between percent culture positivity and percent of total orthopedic consults for MSKI. CONCLUSION: At large, academic pediatric tertiary care centers, pediatric orthopaedic services consult on an average of ~3,000 'rule-out' MSKI cases annually. These patients account for nearly 1 in 10 orthopaedic consultations, of which 1 in 3 are culture positive. Considering that 2 in 3 consultations were culture negative, estimating resources required for pediatric orthopaedic consult services to work up and treat children based on culture positive administrative discharge data underestimates clinical need. Finally, ascertainment bias must be considered when comparing differences in culture rates from different institution's pediatric orthopaedics services, given the variability in when orthopaedic physicians become involved in a MSKI workup.
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Infecciones/cirugía , Enfermedades Musculoesqueléticas/cirugía , Ortopedia/estadística & datos numéricos , Derivación y Consulta/estadística & datos numéricos , Niño , Femenino , Humanos , Infecciones/diagnóstico , Infecciones/microbiología , Masculino , Enfermedades Musculoesqueléticas/diagnóstico , Enfermedades Musculoesqueléticas/microbiología , Estudios Retrospectivos , Estados UnidosRESUMEN
OBJECTIVES: Patient-reported outcomes (PRO) assessing health-related quality of life (HRQoL) are important outcome measures, especially in Legg-Calvé-Perthes disease (LCPD) where symptoms (pain and limping), activity restrictions, and treatments vary depending on the stage of the disease. The purpose of this study was to investigate the validity of the Patient-reported Outcomes Measurement Information System (PROMIS) for measuring HRQoL of patients with LCPD in various stages of the disease. METHODS: This is a multicenter validity study. Patients with LCPD between 4 and 18 years old were included and classified into modified Waldenström stages of disease: Early (1 or 2A), Late (2B or 3), or Healed (4). Seven PROMIS domains were collected, including Pain Interference, Fatigue, Mobility, Depression, Anger, Anxiety, and Peer Relationships. Convergent, discriminant, and known group validity was determined. RESULTS: A total of 190 patients were included (mean age: 10.4±3.1 y). All 7 domains showed the worst scores in patients in the Early stage (known group validity). Within each domain, all domains positively correlated to each other (convergent validity). Patients who reported more anxiety, depression, and anger were associated with decreased mobility and increased fatigue and pain. Peer relationships had no to weak associations with other domains (discriminant validity). CONCLUSIONS: PROMIS has construct validity in measuring the HRQoL of patients in different stages of LCPD, suggesting that PROMIS has potential to serve as a patient-reported outcome tool for this population. LEVEL OF EVIDENCE: Diagnostic level III study.