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High Shear Stress Reduces ERG Causing Endothelial-Mesenchymal Transition and Pulmonary Arterial Hypertension.
Arterioscler Thromb Vasc Biol
December 2024
Department of Pediatrics (T.S., J.-R.M., Y.H.C., J.M.S., J. Kaplan, A.C., L.W., D.G., S.T., S.I., M.D., W.Y., A.L.M., M.R.).
Background: Computational modeling indicated that pathological high shear stress (HSS; 100 dyn/cm) is generated in pulmonary arteries (PAs; 100-500 µm) in congenital heart defects causing PA hypertension (PAH) and in idiopathic PAH with occlusive vascular remodeling. Endothelial-to-mesenchymal transition (EndMT) is a feature of PAH. We hypothesize that HSS induces EndMT, contributing to the initiation and progression of PAH.
View Article and Find Full Text PDFComparison of Perioperative Complication Rates in Congenital Scoliosis Patients With and Without Tethered Spinal Cord.
J Pediatr Orthop
December 2024
Department of Orthopaedic Surgery, Division of Pediatric Orthopaedic Surgery, Montefiore Einstein, Bronx.
Background: Congenital early onset scoliosis (C-EOS) often co-occurs with tethered spinal cord syndrome (TSCS), necessitating surgical intervention to address both conditions to prevent worsening neuromuscular function. Detethering can be done concurrently with spinal deformity correction (SDC), before SDC, or not done at all. This study explores perioperative complications in C-EOS patients with and without TSCS who underwent SDC with growing instrumentation or fusion.
View Article and Find Full Text PDFA rare case of split notochord syndrome with neurenteric cyst: clinical challenges, surgical approach, and literature review.
Childs Nerv Syst
December 2024
Division of Neurosurgery, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
Split notochord syndrome (SNS) is a rare congenital anomaly characterized by incomplete midline notochordal integration during gastrulation, leading to segmental clefts of the spine. This case report describes a female patient with symptomatic cervico-dorsal SNS associated with a neurenteric cyst (NEC), intrathoracic gut duplication, and secondary hydrocephalus. Multistep surgical interventions were performed, including hydrocephalus management, excision of the cyst and gut duplication, and detethering with sectioning of the filum.
View Article and Find Full Text PDFThe importance of timing: evaluating the optimal age for surgical intervention in asymptomatic dermal sinus tracts.
J Neurosurg Pediatr
November 2024
1Department of Neurosurgery, Children's National Medical Center.
Article Synopsis
- Dermal sinus tracts (DSTs) are rare congenital lesions that pose risks of infection and neurological issues due to their connection between the skin and spinal cord, making surgical intervention recommended, though the timing for surgery in asymptomatic cases is uncertain.
- A retrospective review from 1998 to 2022 studied 52 patients who underwent DST excision and detethering, excluding those with pre-existing complications.
- Results indicated a median surgery age of 7 months, with complications in 8% of cases, and younger age at surgery was linked to higher risks of postoperative problems and continued neurological issues.
Tethered cord syndrome in an adult with filum terminale lipoma: A case report.
Surg Neurol Int
October 2024
Department of Spinal Neurosurgery, Kyoto Katsura Hospital, Kyoto, Japan.
Background: Filum terminale lipomas (FTLs) are congenital lumbosacral anomalies that can cause tethered cord syndrome (TCS). Most patients with TCS caused by FTL are children, and these are only rarely present in adults.
Case Description: A 64-year-old male presented with long-standing bilateral lower-limb weakness and bladder dysfunction.
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