Effect of Distal Ulna Osteochondroma Excision and Distal Ulnar Tether Release on Forearm Deformity in Preadolescent Patients With Multiple Hereditary Exostosis.

Background: Multiple hereditary exostosis is a benign condition that can lead to significant forearm deformity secondary to physeal disturbances. As the child grows, the deformity can worsen as relative shortening of the ulna causes tethering, which may lead to increased radial articular angle, carpal slippage, and radial bowing, over time this tethering can also result in radial head subluxation or frank dislocation. Worsening of forearm deformities often require corrective reconstructive osteotomies to improve anatomic alignment and function.

Cohesin complex-associated holoprosencephaly.

Marked by incomplete division of the embryonic forebrain, holoprosencephaly is one of the most common human developmental disorders. Despite decades of phenotype-driven research, 80-90% of aneuploidy-negative holoprosencephaly individuals with a probable genetic aetiology do not have a genetic diagnosis. Here we report holoprosencephaly associated with variants in the two X-linked cohesin complex genes, STAG2 and SMC1A, with loss-of-function variants in 10 individuals and a missense variant in one.

Amputation Versus Staged Reconstruction for Severe Fibular Hemimelia: Assessment of Psychosocial and Quality-of-Life Status and Physical Functioning in Childhood.

Background: Fibular hemimelia, a congenital disorder characterized by the partial or complete absence of the fibula, tibial growth inhibition, and foot and ankle deformity and deficiency, is the most common deficiency of long bones. The purpose of the present study of children with congenital fibular hemimelia was to examine the functional and psychosocial outcomes at a minimum of 2 years after treatment either with amputation and a prosthesis or with reconstruction and lengthening.

Methods: Twenty children who were managed with primary amputation were compared with 22 children who were managed with staged limb reconstruction.

Congenital Pseudarthrosis of the Tibia: Results, at Skeletal Maturity, of the Charnley-Williams Procedure.

Background: This study assessed the outcomes, at skeletal maturity, for 34 patients in whom congenital pseudarthrosis of the tibia (CPT) had been treated with intramedullary (IM) rod fixation.

Methods: The results in skeletally mature patients in whom type-4 CPT had been treated with an IM rod at an average of 11.9 years earlier were reviewed.

Quadriceps Tendon Graft Anatomy in the Skeletally Immature Patient.

Background: The quadriceps tendon (QT) is increasingly considered for primary and revision anterior cruciate ligament reconstruction in skeletally immature patients, as it may be harvested as a purely soft tissue graft with considerable tissue volume. Because of distinct rectus tendon (RT) separation from the QT complex, the potential for RT retraction exists and could lead to QT weakness after QT graft harvest.

Purpose: To describe the anatomy of the pediatric QT and clarify decussation of the RT and QT to avoid the risk of delayed RT retraction and QT weakness after QT graft harvest.

Validation of Pediatric Self-Report Patient-Reported Outcomes Measurement Information System (PROMIS) Measures in Different Stages of Legg-Calvé-Perthes Disease.

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.

Rebound Deformity After Growth Modulation in Patients With Coronal Plane Angular Deformities About the Knee: Who Gets It and How Much?

Background: With observed success and increased popularity of growth modulation techniques, there has been a trend toward use in progressively younger patients. Younger age at growth modulation increases the likelihood of complete deformity correction and need for implant removal before skeletal maturity introducing the risk of rebound deformity. The purpose of this study was to quantify magnitude and identify risk factors for rebound deformity after growth modulation.

The Community Orthopaedic Surgeon Taking Trauma Call: Lateral Humeral Condyle Fracture Pearls and Pitfalls.

Lateral condyle fractures are second only to supracondylar humeral fractures when it comes to pediatric elbow fractures. Whether minimally displaced or substantially displaced, these growth plate fractures may present significant treatment challenges. These include nonunion, malunion, avascular necrosis, and growth disturbance.

Real-Time Monitoring with a Controlled Advancement Drill May Decrease Plunge Depth.

Background: Although drill use is fundamental to orthopaedic surgery, the risk of plunging past the far cortex and potentially damaging the surrounding soft tissues remains unavoidable with conventional drilling methods. A dual motor drill may decrease that risk by providing controlled drill-bit advancement and real-time monitoring of depth and energy expenditure. We hypothesized that using the dual motor drill would decrease plunge depth regardless of the user's level of experience.

The Influence of Body Habitus on Documented Brace Wear and Progression in Adolescents With Idiopathic Scoliosis.

Background: Although studies have been published stating obese patients are less compliant with brace use, no objective studies measuring hours of daily brace wear have been performed to correlate brace wear and success with body mass index (BMI). The purpose of this study was to establish the relationship between BMI and brace compliance, and between BMI and progression to surgical magnitude of 50 degrees or more.

Methods: A total of 175 adolescents were prescribed thoracolumbarsacral orthosis for the treatment of an adolescent idiopathic scoliosis and followed to completion of treatment.

Pain catastrophizing, anxiety, and depression in hip pathology.

Aims: Psychological factors play a critical role in patient presentation, satisfaction, and outcomes. Pain catastrophizing, anxiety, and depression are important to consider, as they are associated with poorer outcomes and are potentially modifiable. The aim of this study was to assess the level of pain catastrophizing, anxiety, and depression in patients with a range of hip pathology and to evaluate their relationship with patient-reported psychosocial and functional outcome measures.

Physical Therapy Scoliosis-Specific Exercises May Reduce Curve Progression in Mild Adolescent Idiopathic Scoliosis Curves.

Purpose: To evaluate the curve magnitude in participants with mild adolescent idiopathic scoliosis (AIS) at high risk of progression who received outpatient physical therapy scoliosis-specific exercises (PSSEs).

Methods: Participants with AIS curves 12° to 20° and Risser grade 0 chose either the PSSE or the control group. The PSSE group was instructed in the Barcelona Scoliosis Physical Therapy School.

The Natural History of Early-onset Scoliosis.

Background: Early-onset scoliosis (EOS) is defined as the diagnosis of a spinal deformity before the age of 5 years. It can be divided into idiopathic, neuromuscular/syndromic, and congenital etiologies.

Methods: The literature on the natural history of EOS was summarized.

Comparison of Anderson-Green Growth-Remaining Graphs and White-Menelaus Predictions of Growth Remaining in the Distal Femoral and Proximal Tibial Physes.

Background: Epiphysiodesis of the distal femoral and/or proximal tibial physes is commonly performed in children and adolescents to treat lower-limb length discrepancy. It is important to determine the amount of growth remaining in the physes of these patients. Two main methods are used to make this determination: the White-Menelaus arithmetic method, which assumes a consistent amount of growth per year until skeletal maturity, and the Anderson-Green growth-remaining charts, which diagram average growth per year ± 2 standard deviations.

2-Pyrrolidinone and Succinimide as Clinical Screening Biomarkers for GABA-Transaminase Deficiency: Anti-seizure Medications Impact Accurate Diagnosis.

Broad-scale untargeted biochemical phenotyping is a technology that supplements widely accepted assays, such as organic acid, amino acid, and acylcarnitine analyses typically utilized for the diagnosis of inborn errors of metabolism. In this study, we investigate the analyte changes associated with 4-aminobutyrate aminotransferase (ABAT, GABA transaminase) deficiency and treatments that affect GABA metabolism. GABA-transaminase deficiency is a rare neurodevelopmental and neurometabolic disorder caused by mutations in and resulting in accumulation of GABA in the cerebrospinal fluid (CSF).

Association Between Sleep Quality and Recovery Following Sport-Related Concussion in Pediatrics.

Our objective was to determine the association between sleep quality, symptom severity, and recovery following sport-related concussion in pediatric athletes. A review of data from the North Texas Concussion Network Prospective Registry (ConTex) was performed. Participants were diagnosed with a sport-related concussion and were ≤18 years old.

Patellar-Trochlear Morphology in Pediatric Patients From 2 to 11 Years of Age: A Descriptive Analysis Based on Computed Tomography Scanning.

Background: Despite the critical role the trochlea plays in patellofemoral (PF) pathology, the development of the trochlea is poorly understood. The purpose of this study was 2-fold: (1) Describe quantitative osseous and soft tissue anatomy of the patella and trochlea in skeletally immature cadaveric specimens utilizing known measurements used in PF instability, and (2) evaluate additional measurement techniques in the sagittal plane as they relate to PF morphologic development.

Methods: Thirty-one skeletally immature fresh frozen cadaveric knees between the ages of 2 and 11 years old were evaluated using 0.

A Novel Posterior Rod-Link-Reducer System Provides Safer, Easier, and Better Correction of Severe Scoliosis.

Study Design: Retrospective review.

Objectives: To compare the Cobb >75° scoliosis correction obtained using a novel Rod-Link-Reducer (RLR) system versus traditional corrective techniques (TCT) in patients with severe adolescent idiopathic scoliosis (AIS).

Summary Of Background Data: Current implant strategies provide for good correction, especially for moderate curves; however, severe scoliosis continues to be challenging to obtain correction in a safe and effective manner.

Delay to Surgery Greater Than 6 Months Leads to Substantial Deformity Progression and Increased Intervention in Immature Adolescent Idiopathic Scoliosis (AIS) Patients: A Retrospective Cohort Study.

Design: A retrospective, multi-institution series of adolescent idiopathic scoliosis (AIS) patients whose date of surgery exceeded six months from date of surgical recommendation were identified. A case-matched comparison of surgical outcomes of skeletally immature patients who delayed surgery versus a cohort of nondelayed patients.

Objectives: We sought to identify 1) whether patients at risk for significant curve progression when delaying surgery could be identified with available clinical and radiographic data and 2) whether patients who delay surgery have longer fusions/more complex procedures.

Halo Gravity Traction for Severe Pediatric Spinal Deformity: A Clinical Concepts Review.

For the past 35 years, we have used halo gravity traction (HGT) to treat patients with a wide variety of underlying spinal deformities. This clinical concepts review will cover the indications, contraindications, HGT technique details, and our preferred method of dynamic HGT. Emphasis will be placed on our protocol, and recommendations that help to avoid complications.

Pharmacokinetics and Tolerability of Multiple Doses of Pharmaceutical-Grade Synthetic Cannabidiol in Pediatric Patients with Treatment-Resistant Epilepsy.

Background: Prior studies have evaluated the use of various constituents of cannabis for their anti-seizure effects. Specifically, cannabidiol, a non-psychoactive component of cannabis, has been investigated for treatment-resistant epilepsy, but more information is needed particularly on its use in a pediatric population.

Objective: The objective of this study was to evaluate the pharmacokinetics and safety of a synthetic pharmaceutical-grade cannabidiol oral solution in pediatric patients with treatment-resistant epilepsy.

Delaying Surgery in Type III Supracondylar Humerus Fractures Does Not Lead to Longer Surgical Times or More Difficult Reduction.

Objective: To determine if delay in surgical treatment of type III supracondylar humerus fracture would affect the length of operative time.

Design: Retrospective cohort study.

Setting: Level 1 trauma center.

Mutation update for the SATB2 gene.

SATB2-associated syndrome (SAS) is an autosomal dominant neurodevelopmental disorder caused by alterations in the SATB2 gene. Here we present a review of published pathogenic variants in the SATB2 gene to date and report 38 novel alterations found in 57 additional previously unreported individuals. Overall, we present a compilation of 120 unique variants identified in 155 unrelated families ranging from single nucleotide coding variants to genomic rearrangements distributed throughout the entire coding region of SATB2.

Hip Surveillance in Children With Cerebral Palsy.

Hip dysplasia is common in children with cerebral palsy (CP), especially in those children with notable functional impairment. Severity of hip dysplasia has been shown to correlate with higher Gross Motor Function Classification System levels. Migration percentage measured on AP pelvis radiographs is the key radiographic measure quantifying hip displacement in CP.