Tibiopelvic Rotational Arthroplasty for Pediatric Gorham-Stout Disease: A Case Report and Surgical Technique.

Case: This is a case of a 6-year-old patient diagnosed with Gorham-Stout disease (GSD), a rare lymphangiogenic skeletal disorder, localized to the left femur. Initial nonoperative treatment with pharmaceuticals and bracing was unsuccessful. We describe a definitive operative treatment with radical femoral resection and a modified rotationplasty technique through a tibiopelvic rotational hip arthroplasty.

Successful management of idiopathic early-onset scoliosis: effect of curve correction and bracing after Mehta casting.

Study Design: Patients with idiopathic EOS treated by Mehta casting followed by bracing or observation from a single institution.

Objectives: To determine casting protocol parameters leading to successful management; to determine efficacy of bracing vs. observation after cast discontinuance.

Outcome of distraction-based growing rods at graduation: a comparison of traditional growing rods and magnetically controlled growing rods.

Introduction: Distraction-based growing rods have been considered as an alternative surgical option for the operative treatment of EOS. TGR has been challenged by MCGR, which is reported to have the advantage of non-invasive lengthening with fewer planned returns to theatre. This study explores the radiographic outcomes, Unplanned Returns to the Operating Room (UPROR) and complication profile of both the procedures at the end of the planned growing rod treatment with either TGR or MCGR.

Long-term Outcomes of Surgically Treated Congenital Dislocation of the Knee.

Background: Congenital dislocation of the knee (CDK) may be idiopathic or associated with another condition, such as Larsen syndrome or arthrogryposis. Surgical reduction of type-3 dislocation may require quadricepsplasty (QP) or femoral diaphyseal shortening (FS). Because it is unknown which treatment is more effective, we evaluated long-term outcomes using patient-reported questionnaires and gait analysis, comparing results by surgery type and underlying diagnosis.

Long-Term Follow-up of Untreated Adult Patients with Spondylothoracic Dysostosis (Jarcho-Levin Syndrome).

Background: Spondylothoracic dysostosis (STD), also known as Jarcho-Levin syndrome (JLS), is a rare autosomal recessive disorder affecting the formation of the spine, characterized by a complete bilateral fusion of the ribs at the costovertebral junction, producing a "crablike" appearance of the thorax. Despite being declared a core indication for a V-osteotomy vertical expandable prosthetic titanium rib (VEPTR) expansion thoracoplasty of the posterior thorax, the natural history of STD in untreated subjects remains poorly documented. In this study, we report radiographic and pulmonary function findings and Patient-Reported Outcomes Measurement Information System (PROMIS) and 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) scores for untreated adult subjects with STD to gain insights into the natural history.

Comparison of treatment outcomes between growth-sparing instrumentation and definitive one-stage fusion for EOS patients ages 6-10 years.

Purpose: To compare objective outcomes for EOS patients age 6-10 years treated by growth-sparing (GS) surgery or definitive one-stage correction and fusion (DF).

Methods: We reviewed surgical, radiographic, PFT's, and EOSQ-24 outcomes for EOS patients > age 6 at index surgery treated at a single institution, minimum 2-year follow-up. Neuromuscular diagnoses were excluded.

Definition of Tweener: Consensus Among Experts in Treating Early-onset Scoliosis.

Background: The term "Tweener" is colloquially used to refer to early-onset scoliosis (EOS) patients whose age and development make them candidates for multiple surgical options. The purpose of this study was to establish expert consensus on a definition to formally characterize the Tweener population.

Methods: A 3-round survey of surgeons in an international EOS study group was conducted.

Contraindications to magnetically controlled growing rods: consensus among experts in treating early onset scoliosis.

Purpose: The purpose of this study was to describe contraindications to the magnetically controlled growing rod (MCGR) in patients with early onset scoliosis (EOS) by establishing consensus amongst expert surgeons who treat these patients frequently.

Methods: Nine pediatric spine surgeons from an international EOS study group participated in semi-structured interviews via email to identify factors that influence decision making in the use of MCGR. A 39-question survey was then developed to specify these factors as contraindications for MCGR-these included patient age and size, etiology, medical comorbidities, coronal and sagittal curve profiles, and skin and soft tissue characteristics.

The 18-cm Thoracic-Height Threshold and Pulmonary Function in Non-Neuromuscular Early-Onset Scoliosis: A Reassessment.

Background: Thoracic spine height is cited as a crucial outcome measure in the treatment of early-onset scoliosis (EOS) because of its reported relationship to pulmonary function tests (PFTs). An 18-cm threshold has been proposed, although this single parameter might be overly simplistic for cases of different etiologies and deformity magnitude. We aimed to reevaluate pulmonary function in patients undergoing corrective surgery, assessing the role of residual scoliosis as well as spine elongation.

Comparison of Outcomes by Reconstructive Strategy in Patients with Prostheses for Proximal Femoral Focal Deficiency.

Background: The present study compares prosthetic treatment options for proximal femoral focal deficiency in terms of gait analysis, oxygen consumption, and patient-reported outcomes.

Methods: Twenty-three patients who had been managed with a prosthesis for unilateral proximal femoral focal deficiency underwent gait analysis; this group included 7 patients who had received an equinus prosthesis, 6 who had received a rotationplasty prosthesis, and 10 who had undergone Syme amputation and had received an above-the-knee prosthesis. Cadence parameters, kinematic and kinetic data, and oxygen consumption were measured, and the Gait Deviation Index (GDI) was calculated.

The Effect of Proximal Anchor Choice During Distraction-based Surgeries for Patients With Nonidiopathic Early-onset Scoliosis: A Retrospective Multicenter Study.

Background: It is unclear whether the type of proximal anchor affects the spine length achieved with distraction-based surgeries in patients with nonidiopathic early-onset scoliosis (EOS). Since distraction may produce kyphosis, spine length should be assessed in the sagittal plane using the sagittal spine length (SSL-curved arc length of the spine in the sagittal plane). Our purpose was to determine if the type of proximal anchor in distraction-based surgeries will affect final spine length.

Reliability and validity of a kyphosis-specific spinal appearance questionnaire.

Purpose: Hyperkyphosis, including Scheuermann and postural kyphosis, is associated with decreased perceived cosmesis and well being. No patient reported outcome questionnaire specific to kyphosis exists. We sought to assess the internal consistency, test-retest reliability and concurrent validity of a new Kyphosis-specific Spinal Appearance Questionnaire (K-SAQ).

An initial effort to define an early onset scoliosis "graduate"-The Pediatric Spine Study Group experience.

Purpose: Increasingly, patients with early onset scoliosis (EOS) are completing a growth friendly surgical program followed by observation, removal of implants or a definitive spinal fusion. These patients are colloquially referred to as "graduates". A standardized definition of a graduate is needed for research and comparing the outcomes, family counseling, and a better understanding of the population.

Can distraction-based surgeries achieve minimum 18 cm thoracic height for patients with early onset scoliosis?

Purpose: Karol et al. introduced the concept that 18 cm thoracic height is the critical point where a patient with early onset scoliosis (EOS) can maintain adequate pulmonary function. Our purpose was to determine if distraction-based surgeries will increase thoracic spine height to at least 18 cm in patients with EOS.

Continued deterioration in pulmonary function at average 23-year follow-up from early thoracic fusion in non-neuromuscular scoliosis.

Purpose: Historically, early-onset scoliosis was treated with early fusion to prevent further deformity at the expense of thoracic growth. This has proven to have a detrimental effect on pulmonary function. The purpose of this study is to evaluate patients' pulmonary and functional status at long-term follow-up after undergoing thoracic fusion at a young age.

The growth-friendly surgical treatment of scoliosis in children with osteogenesis imperfecta using distraction-based instrumentation.

Purpose: The study was undertaken to determine the feasibility of growth-friendly distraction-based surgery in children with OI.

Methods: Two multi-center databases were queried for children with OI who had undergone GR or VEPTR surgery. Inclusion criteria were a minimum 2-year follow-up and three lengthening procedures following the initial implantation.

Minimum 5-Year Follow-up on Graduates of Growing Spine Surgery for Early Onset Scoliosis.

Introduction: After discontinuation of growth friendly (GF) surgery for early onset scoliosis, patients undergo spinal fusion or continued observation. This last planned treatment is colloquially called "definitive" treatment, conferring these patients as "graduates" of a growing program. The 5-year radiographic and clinical outcomes of this cohort are unknown.

Distraction-based surgeries increase thoracic sagittal spine length after ten lengthening surgeries for patients with idiopathic early-onset scoliosis.

Study Design: Retrospective, comparative, multicenter.

Objectives: To determine if the choice of proximal anchor affects thoracic sagittal spine length (SSL) for children with idiopathic early-onset scoliosis (EOS). Debate exists as to whether spine growth is maintained during treatment for EOS.

Results of growth-friendly management of early-onset scoliosis in children with and without skeletal dysplasias: a matched comparison.

Aims: The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management.

Patients And Methods: A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified.

Distraction-Based Surgeries Increase Spine Length for Patients With Nonidiopathic Early-Onset Scoliosis-5-Year Follow-up.

Study Design: Retrospective, comparative.

Objectives: To determine if distraction-based surgeries will increase spine length in patients with nonidiopathic EOS and whether etiology affects final spine length.

Summary Of Background Data: As early-onset scoliosis (EOS) has many etiologies, it is unclear whether etiology affects the spine length achieved with distraction-based surgeries.

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.

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.

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.