Publications by authors named "Shih-Shan Lang"

Objective: Spinal epidural abscesses (SEAs) are rare infections in children but can lead to significant neurological sequelae. The authors sought to describe the presentation, management, and outcomes of children with these infections at their institution.

Methods: This was a retrospective study of pediatric patients admitted with SEA to a quaternary children's hospital between 2013 and 2023.

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Introduction: Cerebral cavernous malformations (CCMs) are pathologic lesions comprised of clusters of thin-walled capillaries characterized by abnormal proliferation, angiogenesis, and bleeding secondary to somatic or germline mutations in endothelial cells. CCMs can cause headaches, seizures and/or neurological defects. There is a clinical need to develop better tools to detect CCMs and follow their progression in conjunction with the current use of neuroimaging techniques.

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Objective: Intracranial complications of acute bacterial sinusitis are rare pathologies that occur in children, and are associated with significant neurological morbidity and mortality. There is a subjective concern among neurosurgeons that the incidence of this rare disease has increased since the onset of the novel COVID-19 pandemic. The primary objective of this study was to review the presentation and management of patients admitted at the authors' institution with intracranial extension of sinusitis, to better understand the local disease burden relative to the COVID-19 pandemic.

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Objective: Cerebral revascularization surgery (CRS) has been used to prevent stroke in children with sickle cell disease (SCD) and cerebral vasculopathy (e.g., moyamoya syndrome).

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Article Synopsis
  • - The study investigates the effectiveness of cranial vault expansion, particularly through posterior vault distraction osteogenesis (PVDO), in treating patients with refractory idiopathic intracranial hypertension (IIH) or chronic shunted hydrocephalus complicated by chronic shunt issues.
  • - It involved a retrospective review of 13 patients over 15 years, revealing that a significant majority (69.2%) had shunted hydrocephalus, with most (92.3%) undergoing PVDO and reporting improved symptoms, especially those with IIH.
  • - Although 30.8% of patients experienced postoperative complications and some required additional surgeries for recurring symptoms, the need for CSF diversion procedures significantly decreased after surgery, indicating a promising medium
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Background: Hereditary hemorrhagic telangiectasia is an autosomal dominant vascular dysplasia characterized by mucocutaneous telangiectasias, recurrent epistaxis, and organ vascular malformations including in the brain, which occur in about 10% of patients. These brain vascular malformations include high-flow AVMs and AVFs as well as low-flow capillary malformations. High-flow lesions can rupture, causing neurologic morbidity and mortality.

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Background: Unlike expired sevoflurane concentration, propofol lacks a biomarker for its brain effect site concentration, leading to dosing imprecision particularly in infants. Electroencephalography monitoring can serve as a biomarker for propofol effect site concentration, yet proprietary electroencephalography indices are not validated in infants. The authors evaluated spectral edge frequency (SEF95) as a propofol anesthesia biomarker in infants.

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Objective: Patients with normocephalic pansynostosis, who have a grossly normal head shape, are often overlooked early in life and present late with elevated intracranial pressure (ICP) requiring timely cranial vault expansion. This study evaluates the long-term clinical outcomes of patients with normocephalic pansynostosis.

Methods: We retrospectively reviewed patients with a clinical and radiographic diagnosis of primary pansynostosis who underwent vault reconstruction between 2000 and 2023.

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Objective: Abusive head trauma (AHT) is one of the most devastating forms of pediatric traumatic brain injury (TBI). It commonly presents with seizures, which may contribute to poor neurological outcome following trauma. Noninvasive near-infrared spectroscopy (NIRS) neuromonitoring may provide information on cerebral oxygenation and perfusion.

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Introduction: Traumatic injuries of the spine requiring surgery are rare in infancy. Fusion procedures in the very young are not well-described at the atlanto-occipital junction or subaxial spine. Here we describe novel segmental posterior instrumentation in a severe spinal column disruption in an infant.

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Purpose: Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) represent a rare pediatric-type tumor that most commonly presents as medically refractory epilepsy. PLNTY has only recently been recognized as a distinct clinical entity, having been first described in 2016 and added to the World Health Organization classification of CNS tumors in 2021. Molecular studies have determined that PLNTY is uniformly driven by aberrant MAPK pathway activation, with most tumors carrying either a BRAF V600E mutation or activating FGFR2 or FGFR3 fusion protein.

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Electroencephalogram (EEG) can be used to assess depth of consciousness, but interpreting EEG can be challenging, especially in neonates whose EEG undergo rapid changes during the perinatal course. EEG can be processed into quantitative EEG (QEEG), but limited data exist on the range of QEEG for normal term neonates during wakefulness and sleep, baseline information that would be useful to determine changes during sedation or anesthesia. We aimed to determine the range of QEEG in neonates during awake, active sleep and quiet sleep states, and identified the ones best at discriminating between the three states.

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Middle meningeal artery (MMA) embolization has gained acceptance as a treatment for chronic subdural hematoma (cSDH) in adult patients but has not been well described in pediatric patients. Standard cSDH treatment has historically consisted of burr hole drainage with or without subdural drain placement. However, due to the high rate of recurrence and frequency of comorbidities within this population, as both pediatric and adult patients with cSDH frequently have concurrent cardiac disease and a need for anticoagulant therapies, MMA embolization has increasingly demonstrated its value as both an adjunctive and primary treatment.

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Objective: Gunshot wounds to the head (GSWH) are a cause of severe penetrating traumatic brain injury (TBI). Although multimodal neuromonitoring has been increasingly used in blunt pediatric TBI, its role in the pediatric population with GSWH is not known. We report on 3 patients who received multimodal neuromonitoring as part of clinical management at our institution and review the existing literature on pediatric GSWH.

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Article Synopsis
  • Surgical revascularization can lower long-term stroke risks in children with moyamoya arteriopathy, but increases stroke risk during surgery, highlighting a need for improved perioperative care practices.
  • Experts used a modified Delphi process to gather insights from 30 specialists on effective management strategies for these patients, culminating in 39 consensus statements.
  • Key recommendations include preadmission for high-risk children, mandatory intravenous fluids around surgery, continuous aspirin usage, and rigorous monitoring of vital signs and neurological status post-surgery.
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Purpose: The purpose of this study was to characterize a novel type of calvarial thickening and provide objective measurements of skull thickness and calvarial suture morphology in patients with bronchopulmonary dysplasia.

Methods: Infants with severe bronchopulmonary dysplasia who also had undergone computed tomography (CT) scans were identified from the neonatal chronic lung disease program database. Thickness analysis was performed using Materialise Mimics.

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Purpose: The incidence of metabolic bone diseases in pediatric neurosurgical patients is rare. We examined our institutional experience of metabolic bone diseases along with a review of the literature in an effort to understand management for this rare entity.

Methods: Retrospective review of the electronic medical record database was performed to identify patients with primary metabolic bone disorders who underwent craniosynostosis surgery between 2011 and 2022 at a quaternary referral pediatric hospital.

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Background: Elevated intracranial pressure (ICP) in sagittal craniosynostosis has a wide spectrum of reported incidence, and patterns are not well understood across infancy and childhood. Characterizing the natural history of ICP in this population may clarify risks for neurocognitive delay and inform treatment decisions.

Methods: Infants and children with sagittal craniosynostosis and unaffected control subjects were prospectively evaluated with spectral-domain optical coherence tomography from 2014 to 2021.

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Background: Recent studies suggest that cerebral revascularization surgery may be a safe and effective therapy to reduce stroke risk in patients with sickle cell disease and moyamoya syndrome (SCD-MMS).

Methods: We performed a multicenter, retrospective study of children with SCD-MMS treated with conservative management alone (conservative group)-chronic blood transfusion and/or hydroxyurea-versus conservative management plus surgical revascularization (surgery group). We monitored cerebrovascular event (CVE) rates-a composite of strokes and transient ischemic attacks.

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Objective: Arteriovenous malformations (AVMs) located in eloquent brain regions are historically associated with a poor prognosis. Awake craniotomy (AC) with the adjunct of brain mapping has the potential of identifying non-eloquent gyri to maximize resection, thereby theoretically decreasing the risk of neurologic deficits. With limited evidence regarding the efficacy of AC in treatment of eloquent AVMs, this review aims to investigate its surgical outcomes.

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Article Synopsis
  • The study focused on understanding vein of Galen malformations (VOGMs), which are severe congenital brain abnormalities, by analyzing the genetic data from 310 affected individuals and their families, as well as a large database of human cerebrovascular cells.
  • Researchers identified significant genetic mutations, particularly in the gene p120 RasGAP and the Ephrin receptor-B4, which play roles in limiting Ras activation and are key to VOGM development.
  • Experiments on mice with specific genetic alterations revealed that abnormal activation of signaling pathways in endothelial cells leads to developmental issues in blood vessel formation, providing insights into both the biology of VOGMs and potential clinical applications for treatment.
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Invasive neuromonitoring has become an important part of pediatric neurocritical care, as neuromonitoring devices provide objective data that can guide patient management in real time. New modalities continue to emerge, allowing clinicians to integrate data that reflect different aspects of cerebral function to optimize patient management. Currently, available common invasive neuromonitoring devices that have been studied in the pediatric population include the intracranial pressure monitor, brain tissue oxygenation monitor, jugular venous oximetry, cerebral microdialysis, and thermal diffusion flowmetry.

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Head and neck positioning is a key element of craniofacial reconstructive surgery and can become challenging when intervention necessitates broad exposure of the calvarium. We present a case of craniosynostosis secondary to Apert's syndrome requiring anterior and posterior cranial vault access during surgical correction. A modified sphinx position was used that required significant neck extension.

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Background: Ketamine has traditionally been avoided as an induction agent for tracheal intubation in patients with neurologic conditions at risk for intracranial hypertension due to conflicting data in the literature. The objective of this study was to evaluate and compare the effects of ketamine versus other medications as the primary induction agent on peri-intubation neurologic, hemodynamic and respiratory associated events in pediatric patients with neurologic conditions at risk for intracranial hypertension.

Methods: This retrospective observational study enrolled patients < 18 years of age at risk for intracranial hypertension who were admitted to a quaternary children's hospital between 2015 and 2020.

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