Pronounced changes in the content of collagen, non-collagen proteins and the activity of a number of lysosomic enzymes have been established in the bone tissue of the vertebral bodies obtained on diskepiphysectomy in the patients with dysplastic scoliosis. Numerous correlation links between the metabolic parameters and the spinal deformation a year after diskepiphysectomy have been determined.
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Arch Orthop Trauma Surg
August 2024
Department of Orthopaedic Surgery, Nagoya University Graduate School of Medicine, 65 Tsurumai, Showa-ku, Nagoya, 466-8550, Japan.
Purpose: Factors affecting direction of pelvic obliquity (PO) in dysplastic hip osteoarthritis (DHOA) remains unclear. This retrospective cohort study evaluates morphological characteristics, spinal alignment, and hip function in patients with unilateral DHOA.
Methods: Between 2018 and 2022, 104 patients with unilateral DHA were enrolled.
J Pediatr Orthop
September 2024
Medical University of South Carolina, Charleston, SC.
Background: During radiographic assessment of adolescent idiopathic scoliosis (AIS), upright images frequently capture the hip. The purpose of this study was to assess the prevalence of radiographic hip dysplasia on postero-anterior (PA) scoliosis radiographs, as defined as a lateral center edge angle (LCEA) ≤25 degrees.
Methods: All patients with upright PA scoliosis radiographs over a one-year study period at a single tertiary academic medical center (2020 to 2021) were included in the study.
J Orthop Case Rep
May 2024
Spinal Unit, Alderhey Children's NHS Foundation Trust, Liverpool, L14 5AB, United Kingdom.
Introduction: Thoracic myelopathy in neuro fibromatosis-1 (NF-1) is most commonly due to intra-spinal neurofibromas/dumb-bell tumors/intra-canal rib head penetration (RHP) causing cord compression. However, acute thoracic myelopathy due to rapid progression of the kyphoscoliotic curve alone in NF-1 without a significant spinal cord compression occurs very rarely. This case report discusses our experience with one such patient and we also discuss intraoperative and post-operative challenges encountered with this patient and a rare complication of hemothorax postoperatively.
View Article and Find Full Text PDFWorld Neurosurg
May 2024
Center for Neurosciences and Spine and Division of Neurosurgery, Virginia Mason Medical Center, Seattle, Washington, USA; Department of Health Services, University of Washington, Seattle, Washington, USA.
Background: The treatment of symptomatic pseudarthrosis via posterior-only approaches in the setting of neurofibromatosis 1 (NF1) is challenging due to dural ectasias, resulting in erosion of the posterior elements. The purpose of this report is to illustrate a minimally invasive method for performing anterior thoracic fusion for pseudarthrosis in a patient with NF1-associated scoliosis and dysplastic posterior elements. To the best of our knowledge, this is the first documented case of using video-assisted thoracoscopic lateral interbody fusion to treat pseudarthrosis for NF1-associated spinal deformity.
View Article and Find Full Text PDFMol Genet Genomic Med
January 2024
Department of Neurology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Background: Epiphyseal, Vertebral, Ear, and Nose (EVEN)-PLUS syndrome is a rare condition characterized by the involvement of the Epiphyses, Vertebrae, Ears, and Nose, plus other associated findings, due to pathogenic variants in the HSPA9 gene. Due to the sparse number of patients, the clinical phenotypic spectrum is not clear.
Methods: We report two patients with pathogenic HSPA9 variants from a Chinese family.
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