Age-Stratified Outcomes of Mehta Casting in Idiopathic Early-Onset Scoliosis: A Multicenter Review.

Background: This study provides age-stratified outcomes of cast treatment for idiopathic early-onset scoliosis.

Methods: This is a multicenter, retrospective review of patients with idiopathic early-onset scoliosis treated with Mehta casting at 4 tertiary children's hospitals between 2001 and 2016. The inclusion criteria were idiopathic early-onset scoliosis and a minimum follow-up of 2 years after casting ended.

What Is the Cost of a "Cast Holiday" in Treating Children With Early Onset Scoliosis (EOS) With Elongation Derotation Flexion (EDF, "Mehta") Casting?

Introduction: Serial casting of children with early onset scoliosis (EOS) is an established treatment option. A break from cast treatment often called a "cast holiday," (CH) is often allowed by some centers, particularly over the summer months. The impact of CHs on treatment duration or outcome has not been examined.

Minimum 5-Year Follow-up of Mehta Casting to Treat Idiopathic Early-Onset Scoliosis.

Background: With the exception of Mehta's 2005 report on her experience treating early-onset scoliosis with serial casting, all subsequent studies have had limited follow-up. This current study sought to assess the results of serial casting at a minimum 5-year follow-up and to identify the predictors of the sustained resolution of scoliosis.

Methods: This study is a retrospective review of children treated for idiopathic early-onset scoliosis with serial casting at a children's hospital between 2001 and 2013 with a minimum 5-year follow-up.

Prevalence of Hip Dysplasia and Associated Conditions in Children Treated for Idiopathic Early-onset Scoliosis-Don't Just Look at the Spine.

Background: Hip dysplasia, congenital muscular torticollis, plagiocephaly, and metatarsus adductus are known to be associated. The etiology of infantile idiopathic scoliosis and its association with the aforementioned conditions is unknown. This study reviews a series of infantile scoliosis patients to address this gap.

Optimization of Casting in Early-onset Scoliosis.

Background: Early-onset scoliosis is a spine deformity that presents before the age of 3 years. When compared with age-matched controls, children with the condition are known to be at risk for significant morbidity and mortality. Although many works support the use of casting for this condition, the key technical factors to optimize the outcomes of casting are not clear.

NF1 Somatic Mutation in Dystrophic Scoliosis.

Scoliosis is a common manifestation of neurofibromatosis type 1, causing significant morbidity. The etiology of dystrophic scoliosis in neurofibromatosis type 1 is not fully understood and therapies are lacking. Somatic mutations in NF1 have been shown in tibial pseudarthrosis providing rationale for similar processes in neurofibromatosis type 1-associated dystrophic scoliosis.

Infantile Idiopathic Scoliosis: Factors Affecting EDF Casting Success.

Study Design: IRB-approved retrospective single cohort study.

Objectives: To review our ten-year history with EDF (Elongation Derotation Flexion) casting in patients with infantile idiopathic scoliosis (IIS) to better understand which factors predict successful outcomes.

Summary Of Background Data: Numerous studies have demonstrated the efficacy of EDF casting in the treatment of progressive infantile idiopathic scoliosis.

Initial Cast Correction as a Predictor of Treatment Outcome Success for Infantile Idiopathic Scoliosis.

Background: Cast treatment for infantile idiopathic scoliosis patients ultimately corrects deformity in varying amounts. As the reasons for these differential outcomes are not fully elucidated, the aim of this study was to identify clinical and radiographic variables correlated with better cast correction.

Methods: Patients in the Children's Spine Study Group and Growing Spine Study Group registries who underwent cast treatment for idiopathic scoliosis between 2005 and 2013 with 1-year minimum follow-up were included.

Utilization of Whole-Exome Next-Generation Sequencing Variant Read Frequency for Detection of Lesion-Specific, Somatic Loss of Heterozygosity in a Neurofibromatosis Type 1 Cohort with Tibial Pseudarthrosis.

A subset of neurofibromatosis type 1 patients develop tibial dysplasia, which can lead to pseudarthrosis. The tissue from the tibial pseudarthrosis region commonly has a somatic second hit in NF1: single-nucleotide variants, small deletions, or loss of heterozygosity (LOH). We used exome next-generation sequencing (NGS) variant frequency data (allelic imbalance analysis) to detect somatic LOH in pseudarthrosis tissue from three individuals with clinically and diagnostically confirmed neurofibromatosis type 1, and verified the results with microarray.

Utilization and efficacy of computational gait analysis for hamstring lengthening surgery.

A retrospective analysis of computational gait studies performed in a single lab over a 12 year period was undertaken to characterize how recommendations to perform or not to perform hamstring lengthenings were utilized by physicians and the effect on outcomes. 131 Subjects were identified as either having hamstring lengthening considered by the referring surgeon, recommended by gait analysis data, or performed. A subset of this data meeting inclusion criteria for pre- and post-surgical timeframes, and bilateral diagnosis was further analyzed to assess the efficacy of the recommendations.

Spontaneous Fatal Intraoperative Rupture of Great Vessel During Growing Rod Lengthening: Do Children With Ehlers-Danlos Syndrome Require the Availability of Vascular Expertise? A Case Report and Review of the Literature.

Background: Ehlers-Danlos syndrome (EDS) is a family of inherited connective tissue diseases. Kyphoscoliotic EDS (kEDS) is associated with severe and early spinal deformity. Very little has been reported regarding the orthopaedic surgical care of kEDS likely due to its rare incidence.

Kinematics and kinetics of normal and planovalgus feet during walking.

Planovalgus deformity is prevalent in cerebral palsy patients, but very few studies have quantitatively reported differences between planovalgus and normal foot function. Intersegmental foot kinetics have not been reported in this population. In this study, a three segment (hindfoot, forefoot, hallux) kinematic and kinetic model was applied to typically developing (n=10 subjects, 20 feet) and planovalgus (n=10 subjects, 18 feet) pediatric subjects by two clinicians for each subject.

Physical Significance of the Rib Vertebra Angle Difference and Its 3-Dimensional Counterpart in Early-Onset Scoliosis.

Study Design: Analysis of the rib vertebra angle difference (RVAD) on coronal plane radiographs and the 3-dimensional (3D) RVAD and Local RVAD in the vertebral reference frame from 3D reconstructions of the spine and rib cage of early-onset scoliosis patients (cross-sectional study).

Objectives: To determine the relationship of RVAD with the geometry of the chest wall.

Summary Of Background Data: Although scoliosis is a complex 3D deformity, RVAD is measured on coronal plane radiographs.

A multi-segment foot model based on anatomically registered technical coordinate systems: method repeatability and sensitivity in pediatric planovalgus feet.

Several multisegment foot models have been proposed and some have been used to study foot pathologies. These models have been tested and validated on typically developed populations; however application of such models to feet with significant deformities presents an additional set of challenges. For the first time, in this study, a multisegment foot model is tested for repeatability in a population of children with symptomatic abnormal feet.

Lower extremity strength and hopping and jumping ground reaction forces in children with neurofibromatosis type 1.

The purpose of this study was to (1) extend the research findings of decreased muscular force production in grip strength to the lower extremity strength of children with NF1 and (2) to determine if there was a relationship between isometric strength and functional activities in children with NF1. Force production was assessed using a hand held dynamometer (HHD) and a functional task (hopping and jumping) on a force plate. Data from twenty-six children with NF1 were compared to data from 48 typically developing children of similar sex, weight and height.

Functional decline in children undergoing selective dorsal rhizotomy after age 10.

Aim: To compare function and gait in a group of children older than most children who received selective dorsal rhizotomy (SDR) with age- and function-matched peers who received either orthopedic surgery or no surgical intervention.

Method: A retrospective study examined ambulatory children with diplegic cerebral palsy, aged between 10 years and 20 years and categorized in Gross Motor Function Classification System (GMFCS) levels I or II. Three groups were considered: (1) children who had selective dorsal rhizotomy (n=8; two females, six males; mean age 15y 4mo at SDR, 16y 8mo at follow-up); (2) children who had orthopedic surgery (n=9; three females, six males; mean age 14y 6mo at SDR, 15y 1mo at follow-up), and (3) children who had no surgical intervention (n=9; two females, seven males; mean age 15y 6mo at follow-up).

Pediatric 25-hydroxyvitamin D concentrations in neurofibromatosis type 1.

Objective: Low 25-hydroxyvitamin D (25OHD) concentrations have been associated with tumors and osteopenia or fractures in adults with neurofibromatosis type 1 (NF1). We report 25OHD concentrations in 109 children with NF1 and 218 controls matched for age, sex, geographic location, and time of year.

Methods: Children with NF1 were recruited (n=109; 2-17 years), and clinical data and dual-energy X-ray absorptiometry measurements were obtained.

Motor proficiency in children with neurofibromatosis type 1.

Purpose: Neurofibromatosis type 1 (NF1) is a genetic disorder with associated musculoskeletal abnormalities, tumors, and developmental delays. The purpose of this study was to investigate and characterize the motor proficiency of children with NF1.

Methods: Children with NF1 were assessed using the Bruininks-Oseretsky Test (BOT 2) instrument.

Orthopaedic manifestations of Navajo familial neurogenic arthropathy.

Background: Navajo Familial Neurogenic Arthropathy is a disease identified in Navajo children, primarily residing in Arizona, New Mexico, and Utah. To date, there are no reports in the orthopaedic literature regarding this disorder, particularly the clinical manifestations and treatment considerations.

Methods: We carried out a retrospective chart and radiographic review of 2 patients with Navajo familial neurogenic arthropathy.

Derotational casting for progressive infantile scoliosis.

Background: Serial cast correction by using the Cotrel derotation technique is one of several potential treatments for progressive infantile scoliosis. This study reviews our early experience to identify which, if any, patients are likely to benefit from or fail this technique.

Methods: We followed all patients treated at our institutions for progressive infantile scoliosis since 2003 prospectively at 1 institution and retrospectively at the other 2.

Analysis of radiographic characteristics of anterolateral bowing of the leg before fracture in neurofibromatosis type 1.

Background: Anterolateral leg bowing is associated with neurofibromatosis type 1 (NF1) frequently leading to fracture and nonunion of the tibia. The objective of the study was to characterize the radiographic findings of tibial dysplasia in NF1.

Methods: This study is a retrospective review of radiographs of tibial dysplasia obtained within 52 years, between 1950 and 2002, from the Shriners Hospitals for Children, Salt Lake City, and of peripheral quantitative computed tomographic images of 3 individuals with anterolateral bowing of the leg without fracture compared with age- and sex-matched controls.

Tibial geometry in individuals with neurofibromatosis type 1 without anterolateral bowing of the lower leg using peripheral quantitative computed tomography.

Introduction: Lower leg bowing with tibial pseudarthrosis is associated with neurofibromatosis type 1 (NF1). The objective of the study is to determine if the geometry of the lower limb in individuals with neurofibromatosis type 1 (NF1) differs from controls, and to characterize the osseous components of the tibia in NF1.

Methods: Peripheral quantitative computed tomography (pQCT) of the lower limb was performed (90 individuals with NF1 without tibial and/or fibular dysplasia: 474 healthy individuals without NF1).

Evidence of increased bone resorption in neurofibromatosis type 1 using urinary pyridinium crosslink analysis.

Although neurofibromatosis type 1 (NF1) is a neurocutaneous disorder, skeletal abnormalities such as long-bone dysplasia, scoliosis, sphenoid wing dysplasia, and osteopenia are observed. To investigate the role of bone resorption as a mechanism for the bony abnormalities, we selected urinary pyridinium crosslinks (collagen degradation products excreted in urine) as a measure of bone resorption in NF1. Bone resorption was evaluated by quantitative assessment of the urinary excretion of pyridinium crosslinks [pyridinoline (Pyd) and deoxypyridinoline (Dpd)].