Outcome of Open Hip Reduction, Pelvic Osteotomy, and Varus Derotational Osteotomy in Children With Cerebral Palsy: A Retrospective Study.

Introduction For spastic hip dislocations, a variety of operations are available with open hip reduction and varus derotational osteotomy of the proximal femur combined with pelvic osteotomy ± adductor release being a good option with favourable outcomes. This study aims to assess the outcome and complications of combined open hip reduction with pelvic osteotomy and varus derotational osteotomy. Methods In this study, 70 hips in 52 patients with spastic hip dislocation due to cerebral palsy were included.

Young Patients' Satisfaction Following the Correction of Adolescent Idiopathic Scoliosis in Saudi Arabia: A Cross-Sectional Study.

Introduction: Posterior spinal instrumented fusion remains the mainstay treatment for adolescent idiopathic scoliosis (AIS) with acceptable post-operative patient satisfaction. However, in Saudi Arabia, patient satisfaction after surgical management for AIS has not been thoroughly studied. The purpose of this study was to determine patient satisfaction and quality of life using the Scoliosis Research Society-22r (SRS-22r, the most recent version) questionnaire after surgical correction of AIS in Saudi Arabia.

Outcome of Vertical Expandable Prosthetic Titanium Rib (VEPTR) Instrumentation in Scoliosis Associated With 1p36 Deletion Syndrome: A Case Report.

Monosomy 1p36 deletion is a rare syndrome that consists of developmental delay, intellectual disability, seizures, hearing and vision defects, brain anomalies, orofacial clefting, congenital heart defects, cardiomyopathy, renal anomalies, and scoliosis. We report the case of an eight-year-old boy who presented to the orthopedic clinic with spinal deformity with a background of 1p36 deletion syndrome. The treatment modalities at this age include growing rods, vertical expandable prosthetic titanium rib (VEPTR), or posterior spinal fusion.

Outcomes of Woodward's Procedure for Sprengel's Shoulder Using Neurophysiological Monitoring of the Brachial Plexus Without Clavicular Osteotomy: A Retrospective Study.

Introduction For Sprengel deformity, a variety of operations are available, with Woodward's procedure being a favorable option with good outcomes. This study aims to assess the outcomes of Woodward's procedure with brachial plexus monitoring to prevent the possible complications of nerve injury and consequent deficits. Methods In our study, we included 18 patients with Sprengel deformity treated with Woodward's procedure using intraoperative neuromonitoring for the brachial plexus from 2013 to 2019 at our institute.

Effects of the Rho/Rho-Kinase Pathway on Perfusion Pressure in the Isolated-Perfused Rat Hind Limb Vascular Bed.

Background: Rho/ROCK signaling has been demonstrated to be involved in the vascular reactivity of many arterial networks. However, RhoA expression and the contribution of Rho/ROCK pathway to the control of perfusion pressure have not been investigated in the rat hind limb vascular bed as a skeletal muscle vascular network.

Aims: To investigate the contribution of the Rho/ROCK pathway in the control of perfusion pressure in the isolated-perfused rat hind limb vascular bed.

Sprengel's Deformity With Congenital Scoliosis Successfully Treated With Combined Posterior Spinal Instrumented Fusion and Modified Woodward's Procedure in a 14-Year-Old Patient With MURCS Association.

Müllerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia (MURCS) association is a rare syndrome. This unique condition consists of Müllerian duct aplasia, cervicothoracic somite dysplasia, and renal aplasia, and skeletal abnormalities manifesting in childhood. We report the case of a 14-year-old girl who presented to the orthopedic clinic with spinal deformity and Sprengel's shoulder complicated by a background of MURCS association.

Radiological Outcomes and Complications of Vertical Expandable Titanium Rib Instrumentation in Congenital Scoliosis With or Without Rib Fusion: A Retrospective Study.

Introduction: In congenital anomalies of the thoracic spine, fusion in situ and hemi-epiphysiodesis are unsuitable surgical options, because three-dimensional thoracic deformity and insufficiency are uncorrectable. We aimed to evaluate the radiological outcome of vertical expandable titanium rib (VEPTR) application after follow-up in children with congenital scoliosis with or without rib fusion.

Methods: In our study, we included 58 patients with congenital scoliosis with or without fused ribs; all treated with VEPTR from 2005 to 2015 at our institute.

Mutation spectrum of EXT1 and EXT2 in the Saudi patients with hereditary multiple exostoses.

Background: Hereditary Multiple Exostoses (HME), also known as Multiple Osteochondromas (MO) is a rare genetic disorder characterized by multiple benign cartilaginous bone tumors, which are caused by mutations in the genes for exostosin glycosyltransferase 1 (EXT1) and exostosin glycosyltransferase 2 (EXT2). The genetic defects have not been studied in the Saudi patients.

Aim Of Study: We investigated mutation spectrum of EXT1 and EXT2 in 22 patients from 17 unrelated families.

The Use of Image-Guided Navigation Systems During Spine Surgeries in Saudi Arabia: A Cross-Sectional Study.

Background: We used a cross-sectional study design (questionnaire) to investigate the use of image-guided navigation (IGN) in Saudi Arabia and explore possible differences in implementing IGN for daily practice.

Methods: An internet-based survey was sent to all spine surgeons who are practicing in Saudi Arabia (orthopedics or neurosurgery). The survey is composed of 12 items that collected demographic and academic data.

Cell salvage within adult and pediatric idiopathic scoliosis surgery: A random cross-sectional study.

Introduction: This study aimed to evaluate the use of a cell savage and its impact on the amount of allogenic blood transfused to the patients during idiopathic scoliosis surgery.

Methods: A total of 142 randomly selected patients with scoliosis had been included in this study. The adult group consisted of 78 patients, and the pediatric group, 64 patients.

Functional outcomes in bilateral upper limb Amelia patient with scoliosis post vertical expandable prosthetic titanium rib (VEPTR) application: A case report.

Introduction: Congenital upper limb Amelia is one of the extremely rare conditions in the world. Defined as complete absence of a limb which may present as isolated defect or as a part of syndrome with associated anomalies.

Presentation Of The Case: We report a case of a medically free 6-year-old boy with bilateral upper limb Amelia associated with right thoracolumbar idiopathic Scoliosis.

Synovial sarcoma presenting as an intra-articular mass in a pediatric patient: a case report.

Background: Synovial sarcoma (SS) is one of the reported sarcomas in the pediatric and adult populations. Delay in diagnosis and treatment is common in SS cases. SS may be excised before the correct diagnosis is made.

Exome sequencing identifies novel NTRK1 mutations in patients with HSAN-IV phenotype.

Hereditary sensory autonomic neuropathy type IV (HSAN-IV) is a rare autosomal recessive disorder that usually begins in infancy and is characterized by anhidrosis, insensitivity to noxious stimuli leading to self-mutilating behavior, and intellectual disability. HSAN-IV is caused by mutations in the neurotrophic tyrosine kinase receptor type 1 gene, NTRK1, encoding the high-affinity receptor of nerve growth factor (NGF) which maps to chromosome 1q21-q22. Patients with HSAN-IV lack all NGF-dependent neurons, the primary afferents and sympathetic postganglionic neurons leading to lack of pain sensation and the presence of anhidrosis, respectively.

Study of autosomal recessive osteogenesis imperfecta in Arabia reveals a novel locus defined by TMEM38B mutation.

Background: Osteogenesis imperfecta (OI) is an hereditary bone disease in which increased bone fragility leads to frequent fractures and other complications, usually in an autosomal dominant fashion. An expanding list of genes that encode proteins related to collagen metabolism are now recognised as important causes of autosomal recessive (AR) OI. Our aim was to study the contribution of known genes to AR OI in order to identify novel loci in mutation-negative cases.

Mutations in FKBP10 cause both Bruck syndrome and isolated osteogenesis imperfecta in humans.

Bruck syndrome (BS) is an autosomal recessive syndromic form of osteogenesis imperfecta (OI) that is characterized by the additional presence of pterygium formation. We have recently shown that FKBP10 previously reported as a novel autosomal recessive OI gene also defines a novel Bruck syndrome locus (BKS3). In this manuscript, we extend our analysis to describe a mutation previously described in isolated OI patients and show that it results in BS phenotype in a Saudi family.