Mineral and Bone Consequences of High Dose Denosumab Therapy to Treat an Aneurysmal Bone Cyst, a Child Case Report.

Aneurysmal bone cysts (ABCs) are rare benign pseudotumoral bone lesions with potential aggressive behavior due to the extensive destruction of surrounding bone. Traditionally, these tumors were treated with open surgery, but there is more and more a shift to less invasive procedures. In particular, treatment for spinal ABCs is generally unsatisfactory due to the risk of morbidity, neurological impairment and recurrence, and there is a need for innovative therapies. Denosumab has been reported as a useful treatment in giant cell tumors of bone (GCTB), so its efficacy has been tested also in other fibro-osseus lesions affecting children and adolescents, such as spinal aneurysmal bone cysts. The pediatric literature is limited to case reports and small series, all of which highlight the efficacy of this treatment on lesions growth and associated bone pain. Some of these reports have already reported well known side effects associated with denosumab, such as hypocalcemia at the beginning of the treatment, and rebound hypercalcemia at the discontinuation. The latter seems to be more frequent in children and adolescents than in adults, probably due to the higher baseline bone turnover in children. In addition, the use of denosumab in young patients could affect both bone modeling and remodeling, even if the consequences on the growing skeleton have not been reported in detail. Here we describe the case of a spinal ABC diagnosed in an 8-year old young boy which was not accessible to surgery but responded favorably to denosumab. Our aim is to describe the rapid changes in mineral and bone homeostasis in this patient, that required advice from the experts of the European Reference Network (ERN) for rare bone and endocrine diseases.

Helping Children with Obesity "Move Well" To Move More: An Applied Clinical Review.

ABSTRACT: Children with obesity experience musculoskeletal pain and reduced physical function and well-being, which collectively impact their fitness, strength, motor skills, and even their ability to undertake simple tasks, like walking and climbing stairs. Disrupting obesity-related disability may be critical to increasing children's physical activity. Thus, barriers to movement should be considered by health practitioners to improve the efficacy of prescribed physical activity. This applied clinical review highlights key subjective and objective findings from a hypothetical case scenario, linking those findings to the research evidence, before exploring strategies to enhance movement and increase physical activity.

Distal femoral osteotomy for genu valgum deformity caused by malunited Hoffa fracture.

Hoffa fractures are unstable intra-articular fractures of the femoral condyle that occur in the coronal plane.Insufficient anatomical reduction and internal fixation may lead to non-union or malunion. A 39-year-old man was involved in a traffic accident while riding a motorcycle and was diagnosed with left Hoffa fracture and avulsion fracture of the femoral attachment of the medial collateral ligament. Open reduction and internal fixation were performed 5 days after injury. The patient experienced intermittent knee pain, joint contracture and deformity, and attended our hospital for further treatment 18 months after surgery. CT revealed depression and malunion of the posterior aspect of the lateral femoral condyle, and weight-bearing X-ray showed valgus deformity due to malunion. Distal femoral osteotomy (DFO) was performed and good functional and radiographic results were obtained. This report suggests that DFO is a reasonable treatment for young patients suffering from malalignment due to malunited Hoffa fracture.

Treatment of a combined deformity following tibial tubercle ventromedialization with single proximal tibial closing wedge osteotomy.

Patellar instability in skeletally immature patients must be approached with concern of potential damage to physes. In this article, we present a case of combined knee deformity consisting of genu recurvatum, genu valgum and patella baja which arose subsequent to a pediatric tibial tubercle ventromedialization. Patient was a female who was 22 years old upon first admission. After confirming cartilage to be in good health, the deformity was corrected with a single supratubercular posteriorly and medially based proximal tibial closing wedge osteotomy. At the seventh year follow-up, patient was pain free, functionally satisfied and radiographic evaluation showed correct alignment. This case illustrates the consequent difficulties faced if physeal damage occurs during treatment of patellar instability as well as effectiveness of a single well-planned supratubercular proximal tibial osteotomy treating combined genu recurvatum, genu valgum and patella baja deformities; although anatomic and clinical studies are required to generalize its use.

Effects of a knee valgus unloader brace on medial femoral articular cartilage deformation following walking in varus-aligned individuals.

BACKGROUND: Knee varus alignment may increase loading in the medial tibiofemoral compartment, which can increase strain on the articular cartilage. Knee valgus unloader braces seek to reduce loading through the medial femoral compartment, but their effects on cartilage characteristics during dynamic tasks have not been evaluated. OBJECTIVE: To determine the effects of a knee valgus unloader brace on medial femoral articular cartilage deformation following a single 5000-step walking protocol in individuals with varus-knee alignment. METHODS: Twenty-four healthy individuals (63% female, BMI = 22 ±â€¯3 kg/m2, age = 21 ±â€¯3 years) completed two testing sessions (braced and unbraced) separated by one week. During both sessions, femoral cartilage ultrasound images were acquired prior to and following a 5000-step treadmill walking protocol at self-selected speed. Percent change scores in medial cartilage cross-sectional area (MCCA) were calculated and used as the primary outcome, and compared between the braced and unbraced conditions. RESULTS: There was no difference in percent change of MCCA between conditions (braced = -2.77%, unbraced = -3.15%, p = 0.699). Individuals whose cartilage deformed more than a previously established minimal detectable change (MDC ≥ 1.58 mm2) deformed less during the braced condition (braced = -2.94%, unbraced = -6.34%, p = 0.028), compared to individuals who did not deform greater than the MDC (n = 15, braced = -2.67%, unbraced = -1.23%, p = 0.210). CONCLUSIONS: There was no significant difference in MCCA percent change between the braced and unbraced conditions across the entire cohort; yet a valgus unloader braces may serve as a potential intervention strategy for reducing articular cartilage deformation in certain varus-aligned individuals who normally undergo measurable deformation during walking.

Risk factors of postoperative valgus malalignment in mobile-bearing medial unicompartmental knee arthroplasty.

OBJECTIVE: The aim of this observational study was to investigate the risk factors of postoperative valgus malalignment after mobile-bearing medial unicompartmental knee arthroplasty (UKA). METHODS: We retrospectively evaluated radiographic and surgical characteristics in 122 consecutive Oxford phase 3 UKAs. According to postoperative hip-knee-ankle angle (HKAA), 24 knees were sorted into group valgus with HKAA > 180° and 98 knees were sorted into group non-valgus with HKAA ≤ 180°. Logistic regression was performed to analyze risk factors including age, gender, BMI, side, preoperative limb alignment HKAA, preoperative LDFA, MPTA, FTFA, thickness of polyethylene bearing insert, tibial prothesis size, femoral prothesis size, medial tibial cut thickness, thickness of distal femoral mill, prothesis angle of coronal, and sagittal plane. RESULTS: The mean mechanical preoperative HKAA of 174.39°±4.23° was corrected to 178.18°±3.49° postoperatively (t = - 13.45, p = 0.000). The mean of postoperative HKAA in valgus group and non-valgus group was 183.45 ± 2.21° and 176.88 ± 2.35°, respectively (t = 12.44, p = 0.000). After statistical analysis with univariate analysis, eight risk factor variables among 16 independent variables were identified as potential predictors with p value ≤ 0.1. Multivariate logistic regression analysis for these eight potential predictors revealed that tibial cut (p = 0.046), LDFA (p = 0.003), MPTA (p = 0.011), and FTFA (p = 0.008) were significant risk factors predicting postoperative valgus malalignment after mobile-bearing UKA. CONCLUSIONS: Preoperative smaller LDFA, FTFA, larger MPTA and less medial tibial cut thickness were significantly associated with postoperative valgus malalignment in mobile-bearing UKA.

Concordancia entre el análisis computarizado de la marcha y el examen físico en pacientes con anteversión femoral aumentada sin alteraciones neurológicas / Concordance between computerised gait analysis and physical examination in patients with augmented femoral anteversion

Introducción: La marcha es el punto máximo de independencia funcional de una persona y allí radica su importancia como patrón de movimiento, cuya medición ha sido desarrollada a través de los años. Objetivo: Evaluar la concordancia entre las variables de 2 herramientas de medición: el análisis computarizado de la marcha (ACM) y el examen físico (EF), en pacientes con anteversión femoral aumentada sin alteraciones neurológicas. Metodología: Es un estudio observacional analítico de concordancia; los datos se obtuvieron de forma retrospectiva de los años 2010 al 2014 en el laboratorio de análisis de la marcha del Instituto de Ortopedia Infantil Roosevelt por medio de una sola aplicación del ACM y del EF. Resultados: Se observó que existen alteraciones evidentes en el EF, pues a nivel de la cadera existe una disminución de la rotación externa y un aumento de la rotación interna. En el ACM se encontró que en la cadera existe una disminución del rango de rotación interna-externa en la fase de apoyo y balanceo; que en la rodilla hay una disminución del rango flexión-extensión durante la fase de balanceo, y en el tobillo se halló una disminución del rango dorsiflexión-plantiflexión en la fase de apoyo y balanceo. Conclusión: Este estudio determinó que el EF y el ACM son pruebas que no se correlacionan, por lo tanto, se puede deducir que son complementarias y aportan información con un abordaje diferencial para la toma de decisiones a nivel clínico

Introduction: Human gait is the highest point in a person's functional independence; therefore, its importance as a movement pattern has led to the development of measuring tools. Objective: To assess the concordance between 2 measuring tools: computerised gait analysis (CGA) and physical examination (PE) in patients with increased femoral anteversion without neurological alterations. Methods: We conducted an observational analytic study of concordance. Data were obtained retrospectively from 2010 to 2014 in the gait analysis laboratory of the Roosevelt Children's Orthopaedics Institute through a single application of CGA and PE. Results: There were evident alterations in the PE because, at the hip, the internal-external rotation range decreases in the stance and swing phases. In the knee, there was a decrease in the flexion-extension range during the swing phase and in the ankle there was also a decrease in the dorsiflexion-plantarflexion range in the stance and swing phases. Conclusion: This study found that there was no concordance between PE and CGA. Therefore, these tests are complementary and provide information for a differential approach in clinical decision-making

[Conservative treatment of congenital patellar dislocation]. / Konservative Therapie bei kongenitaler Patellaluxation.

This article presents the rare case of a boy who was born in our hospital with valgus deformity and external rotation of the right lower leg because of congenital patellar dislocation. In the case presented a stable repositioning of the patella could be achieved by redressment with a plaster cast and leg brace. During a 4-year follow-up there were no tendencies towards dislocation during the clinical examination and no dislocation events were documented. In selected cases an attempt at conservative repositioning and retention treatment appears to be worthwhile before surgical treatment is indicated.

Prediction of rebound phenomenon after removal of hemiepiphyseal staples in patients with idiopathic genu valgum deformity.

AIMS: Our aim was to investigate the predictive factors for the development of a rebound phenomenon after temporary hemiepiphysiodesis in children with genu valgum. PATIENTS AND METHODS: We studied 37 limbs with idiopathic genu valgum who were treated with hemiepiphyseal stapling, and with more than six months remaining growth at removal of the staples. All children were followed until skeletal maturity or for more than two years after removal of the staples. RESULTS: On multivariate logistic regression analysis, the rate of correction, body mass index (BMI), age, and initial valgus angle were significantly associated with a rebound phenomenon. With those characteristics, a predictive model for rebound was generated using recursive partitioning analysis. Children with a rapid rate of correction had the most frequent and severe rebound phenomenon (incidence 79%, mean 4°), whereas those with a slow rate of correction had less rebound when they had low BMI (43%, 2°) and none when the BMI was ≥ 21 kg/m(2). CONCLUSION: This is the first study to evaluate a predictive model for a rebound phenomenon after temporary hemiepiphysiodesis in children with idiopathic genu valgum. Cite this article: Bone Joint J 2016;98-B:1270-5.

[Patella dislocation in athletes]. / Patellaluxation bei Sportlern.

Most dislocations of the patella occur during sports activities. The entities instability, maltracking and loss of tracking should be defined by patient history, clinical examination and radiological evaluation including magnetic resonance imaging (MRI). Based on these criteria a new classification of patella dislocations (5 types) was established which allows a standardized treatment algorithm. Type 1 is a simple (traumatic) dislocation without maltracking and without instability. Type 2 has a high redislocation risk (defined as instability) without maltracking. An isolated stabilizing surgical procedure, such as medial patellofemoral ligament (MPFL) augmentation is successful in most cases. Type 3 is characterized by instability and maltracking. Maltracking can be caused by soft tissue contracture or muscular deficits (type 3a), patella alta (type 3b), pathological tibial tuberosity to trochlear groove (TT-TG) distance (type 3c), genu valgum (type 3d) and torsional deformities (type 3e). In these types an isolated soft tissue procedure is usually not sufficient. The bony pathologies additionally need to be addressed to regain physiological patella tracking. Type 4 includes severe trochlea dysplasia with loss of patella tracking. Usually trochleaplasty is needed to stabilize the patella and to prevent redislocation. Type 5 is based on patella maltracking without instability and can be found in patients with a pathological knee baseline or special forms of torsional deformities. Although patella dislocations in trained athletes are seldom due to the stabilizing muscular status, the treatment strategy is similar to that of normal persons. Additional cartilage injuries, type of sports and time for rehabilitation have to be considered for optimal treatment.

Timely diagnosis of malalignment of the distal extremities is crucial in morbidly obese juveniles.

BACKGROUND/AIMS: To determine i) whether obesity in childhood can be related to malalignment of the distal extremities, ii) the proportion of genu valgum malalignment and abduction setting, and iii) the respective deviation dominance in children who are morbidly obese. METHODS: 31 morbidly obese Caucasian children (16 males) recruited for the STYJOBS Study (ClinicalTrials.gov Identifier NCT00482924) with a mean age of 13.9 ± 0.5 years, a mean height of 162.3 ± 2.7 cm, a mean weight of 90.62 ± 5.0 kg, and a mean BMI of 33.8 ± 1.2 kg/m(2) were clinically examined using the Mikulicz line in order to assess load distribution on the knee joint. 21 participants received a whole-leg X-ray because of a clinically estimated malalignment. RESULTS: 8/31 participants examined were diagnosed with genu valgum, 1/31 with genu varum, and 22/31 did not have any malalignment of the femur or tibia. The majority of genu valgum presentation was due to femoral deviation. Of those without malalignment, 4/22 participants had an abduction setting, while 2/22 showed an adduction of the leg. CONCLUSION: Genu valgum as a predominant malalignment of the distal extremities is frequent in youth with morbid obesity. Timely guided correction of angular deformity of the knee seems pivotal in order to avoid osteotomy or osteoarthritis later in life.

[Crooked legs- just an aesthetic problem]. / Krumme Beine - mehr als ein ästhetisches Problem?

Because the lower extremities are weight bearing, malalignment plays a key role in the development of degenerative disease of the joints. This is particularly true as degenerative arthropathy is of mechanical and not inflammatory cause. Deviation of the axis has been shown to alter the load and force distribution in the joint and thereby causing excessive wear. Whether or not a deformity is clinically relevant or not depends on the symptoms, the amount of the deformity and the location of the deformity. Relevant deformities can be treated with orthotics or corrective osteotomies in early stages. In late stages fusions or joint replacement is the mainstay of treatment.

Parce que les extrémités inférieures portent un poids, un mauvais alignement de ces dernières joue un rôle clé dans le développement des atteintes dégénératives des articulations. Cela est particulièrement vrai puisque l'arthropathie dégénérative est d'origine mécanique et non pas inflammatoire. Une déviation axiale a été montrée modifier la répartition des charges et des forces dans l'articulation et provoquer ainsi un poids excessif. Qu'une déformation soit significative ou non sur le plan clinique dépend des symptômes, de l'importance et de la localisation de la déformation. Les déformations significatives peuvent être traitées dans des stades précoces par des ostéotomies orthotiques ou correctrices. Dans les stades tardifs des fusions ou des remplacements articulaires représentent les options thérapeutiques principales.

Lamellar ichthyosis with genu valgum: unfolding the link.

We describe a case of lamellar ichthyosis with bilateral genu valgum. The association of genu valgum with congenital ichthyosis is rare. Our patient, a 22-year-old girl, had lamellar ichthyosis and was born with a collodion membrane. She developed progressive valgus deformity of the knees of 5 years duration associated with difficulty in walking. On evaluation, she had generalised scaly skin lesions along with bilateral genu valgum and biochemical evidence of vitamin D deficiency. Skin serves as an important site for vitamin D synthesis and thus skeletal deformities secondary to vitamin D deficiency may occur in cases of congenital ichthyosis, causing a diagnostic dilemma due to the unusual association. This case serves as a reminder that clinicians need to be aware of such an association in order to prevent, appropriately diagnose and adequately treat the rare case of congenital ichthyosis with rickets and osteomalacia.

[Evaluation of alignment on knees through a software]. / Evaluación de la alineación en rodillas mediante un software.

INTRODUCTION: Diseases of the musculoskeletal system are often accompanied by postural deformity or malalignment. Genu varo or genu valgo can be found in the knees. It is necessary to have a diagnostic support test for diagnosis as well as for follow-up of cases since these diseases are frequently progressive and over time have serious repercussions on patient quality of life. Objective. To evaluate a software program that processes digitalized photographs as a diagnostic test for measuring the mechanical axis in patients with genu varo and genu valgo. MATERIALS AND METHODS: The mechanical axis in both knees was measured by means of radiography (golden standard) and by means of a software program (proposed diagnostic test) in one hundred patients. Mechanical axis was considered to be abnormal when the angle was equal to or greater than four degrees. Sensitivity, specificity, positive predictive value, and negative predictive value were determined. Interobserver variation was evaluated with kappa statistics. RESULTS: In relation to right knee genu varo, sensitivity, specificity, positive predictive value, and negative predictivie value were 0.84, 0.87, 0.84, and 0.87, respectively, and for the left knee values were 0.86, 0.87, 0.84, and 0.89, respectively. For genu valgo in the right knee, sensitivity, specificity, positive predictive value, and negative predictive value were 0.78, 0.98, 0.95, and 0.92, respectively, and in the left knee were 0.88, 0.95, 0.88, and 0.95, respectively. Kappa value was 0.9 in the right knee and 0.8 in the left knee. CONCLUSION: The software program (diagnostic test) was useful for diagnosing genu varo or genu valgo, representing a safe and low-cost study.

Correlation between clinical tibiofemoral angle and body mass index in normal Nigerian children.

PURPOSE: The tibiofemoral angle (TFA) is a reliable tool for determining lower-limb axial alignment and defining the degree of deformity in pathologic angular malalignment of the knee in children. METHODS: We clinically examined 471 normal Nigerian elementary school children whose ages ranged from three to ten years to determine the clinical tibiofemoral angle and to establish its relationship with body mass index (BMI). RESULTS: We found maximum knee valgus of 7.87° at three years, decreasing to 1° at ten years. We also found significant negative correlation between the tibiofemoral angle and BMI. All children examined had weights between the 5th and 85th percentile for age and sex. CONCLUSION: We conclude that in normal healthy-weight children, BMI does not cause an increase in tibiofemoral angle.

Characterizing multisegment foot kinematics during gait in diabetic foot patients.

BACKGROUND: The prevalence of diabetes mellitus has reached epidemic proportions, this condition may result in multiple and chronic invalidating long term complications. Among these, the diabetic foot, is determined by the simultaneous presence of both peripheral neuropathy and vasculopathy that alter the biomechanics of the foot with the formation of callosity and ulcerations. To diagnose and treat the diabetic foot is crucial to understand the foot complex kinematics. Most of gait analysis protocols represent the entire foot as a rigid body connected to the shank. Nevertheless the existing multisegment models cannot completely decipher the impairments associated with the diabetic foot. METHODS: A four segment foot and ankle model for assessing the kinematics of the diabetic foot was developed. Ten normal subjects and 10 diabetics gait patterns were collected and major sources of variability were tested. Repeatability analysis was performed both on a normal and on a diabetic subject. Direct skin marker placement was chosen in correspondence of 13 anatomical landmarks and an optoelectronic system was used to collect the data. RESULTS: Joint rotation normative bands (mean plus/minus one standard deviation) were generated using the data of the control group. Three representative strides per subject were selected. The repeatability analysis on normal and pathological subjects results have been compared with literature and found comparable. Normal and pathological gait have been compared and showed major statistically significant differences in the forefoot and midfoot dorsi-plantarflexion. CONCLUSION: Even though various biomechanical models have been developed so far to study the properties and behaviour of the foot, the present study focuses on developing a methodology for the functional assessment of the foot-ankle complex and for the definition of a functional model of the diabetic neuropathic foot. It is, of course, important to evaluate the major sources of variation (true variation in the subject's gait and artefacts from the measurement procedure). The repeatability of the protocol was therefore examined, and results showed the suitability of this method both on normal and pathological subjects. Comparison between normal and pathological kinematics analysis confirmed the validity of a similar approach in order to assess neuropathics biomechanics impairment.