Utility of intracranial pressure monitoring as a diagnostic tool in pediatric ventriculomegaly.

OBJECTIVE: Intracranial pressure (ICP) monitoring is commonly utilized for identifying pathologic ICP in cases of traumatic brain injury; however, its utility in hydrocephalic children has not been elucidated. Although patients with typical (pressure-active) hydrocephalus present with clear signs and/or symptoms and the need for cerebrospinal fluid (CSF) diversion is often clear, others may have arrested or pressure-compensated hydrocephalus with pathologic ICP elevation masked by ambiguous signs or are completely asymptomatic. Without treatment these pathologic ICP elevations may affect neurologic development or crescendo over time leading to neurological decline. The purpose of this study is to investigate the utility of ICP monitoring as a diagnostic tool in this relatively common patient population and identify ventriculomegaly patients with and without pathologic ICP, thus improving accuracy of identifying those with and without surgical needs. METHODS: 36 patients (≤ 17 years old) underwent 41 inpatient ICP recording sessions between 2016 and 2022 and were retrospectively reviewed. This included patients with a history of severe, nonprogressive ventriculomegaly and normal fundoscopic examinations lacking traditional signs and symptoms concerning for elevated ICP. Nighttime pathological plateau waves were defined as sustained elevations of ICP ≥ 2x baseline for a duration of ≥ 5 minutes. RESULTS: The mean age of patients was 5.5 years old (range 0-17 years old). 46.3% of patients had prior endoscopic third ventriculostomy (ETV), 14.6% had prior ventriculoperitoneal shunt (VPS), and 39% were without prior surgical intervention. Roughly half (51.2%) of patients had congenital ventriculomegaly while other patients had ventriculomegaly due to other pathologies such as germinal matrix hemorrhage/intraventricular hemorrhage (GMH/IVH) (29.3%), stroke (4.9%), cerebral infections/meningitis (2.4%), or unknown etiology (12.2%). The average procedure time was 19.1 ± 10.5 minutes, and mean length of stay was 2.8 ± 0.7 days. Pathologic ICP was demonstrated in 12 cases (29.3%), 4 (33.3%) of which were asymptomatic. Pathologic ICP was found in 7 of 19 (36.8%) in the prior ETV group, 1 of 6 (16.7%) in prior shunt group, and 4 of 16 (25%) in the non-surgical group (p = 0.649). Among those with pathologic ICP, 6 (50%) cases received an ETV, 5 (41.7%) cases underwent VPS placement, and 1 (8.3%) case underwent a VPS revision. There were no infectious complications or cases of hemorrhage. 4 patients required repositioning of the ICP monitor due to dislodgement. CONCLUSION: Inpatient ICP monitoring is a safe and effective diagnostic tool for evaluating the presence of pathologic ICP in severe, persistent non-progressive ventriculomegaly. The use of ICP monitoring may aid in identifying patients with pressure-compensated hydrocephalus who demonstrate pathologic ICP where surgical intervention may be warranted, while preventing unnecessary CSF diversion in those without pathology.

Neurosurgery for psychiatric disorders: reviewing the past and charting the future.

Surgical techniques targeting behavioral disorders date back thousands of years. In this review, the authors discuss the history of neurosurgery for psychiatric disorders, starting with trephination in the Stone Age, progressing through the fraught practice of prefrontal lobotomy, and ending with modern neurosurgical techniques for treating psychiatric conditions, including ablative procedures, conventional deep brain stimulation, and closed-loop neurostimulation. Despite a tumultuous past, psychiatric neurosurgery is on the cusp of becoming a transformative therapy for patients with psychiatric dysfunction, with an ever-increasing evidence base suggesting reproducible and ethical therapeutic benefit.

Comparison of outcomes after stereoelectroencephalography and subdural grid monitoring in pediatric tuberous sclerosis complex.

OBJECTIVE: Patients with tuberous sclerosis complex (TSC) epilepsy present with unique clinical challenges such as early seizure onset and high rates of intractability and multifocality. Although there are numerous studies about the safety and efficacy of stereoelectroencephalography (SEEG), this topic has not been studied in TSC patients who have distinct epilepsy profiles. The authors investigated subdural grid (SDG) and SEEG monitoring to determine whether these procedures lead to similar seizure and safety outcomes and to identify features unique to this pediatric population. METHODS: TSC patients who underwent SDG or SEEG placement and a second epilepsy surgery during the period from 2007 to 2021 were included in this single-center retrospective cohort analysis. Various patient, hospitalization, and epilepsy characteristics were collected. RESULTS: A total of 50 TSC patients were included in this study: 30 were included in the SDG cohort and 20 in the SEEG cohort. Baseline weekly seizure count did not significantly differ between the 2 groups (p = 0.412). The SEEG group had a greater mean baseline number of antiepileptic drugs (AEDs) (3.0 vs 2.0, p = 0.003), higher rate of previous surgical interventions (25% vs 0%, p = 0.007), and larger proportion of patients who underwent bilateral monitoring (50% vs 13.3%, p = 0.005). Despite this, there was no significant difference in seizure freedom between the SDG and SEEG cohorts. The mean reduction in seizure count was 84.9% and 47.8% of patients were seizure free at last follow-up (mean 79.4 months). SEEG trended toward being a safer procedure than SDG monitoring, with a shorter mean ICU stay (0.7 days vs 3.9 days, p < 0.001), lower blood transfusion rate (0% vs 13.3%, p = 0.140), and lower surgical complication rate (0% vs 10%, p = 0.265). CONCLUSIONS: In the comparison of the SDG and SEEG cohorts, the SEEG group included patients who appeared to receive more aggressive management and have a higher rate of multifocality, more prior surgical interventions, more AEDs at baseline, and a higher rate of bilateral invasive monitoring. Despite this, the SEEG cohort had similar seizure outcomes and a trend toward increased safety. Based on these findings, SEEG appears to allow for monitoring of a wider breadth of TSC patients given its minimally invasive nature and its relative simplicity for monitoring numerous regions of the brain.

Molecular Classification and Therapeutic Targets in Ependymoma.

Ependymoma is a biologically diverse tumor wherein molecular classification has superseded traditional histological grading based on its superior ability to characterize behavior, prognosis, and possible targeted therapies. The current, updated molecular classification of ependymoma consists of ten distinct subgroups spread evenly among the spinal, infratentorial, and supratentorial compartments, each with its own distinct clinical and molecular characteristics. In this review, the history, histopathology, standard of care, prognosis, oncogenic drivers, and hypothesized molecular targets for all subgroups of ependymoma are explored. This review emphasizes that despite the varied behavior of the ependymoma subgroups, it remains clear that research must be performed to further elucidate molecular targets for these tumors. Although not all ependymoma subgroups are oncologically aggressive, development of targeted therapies is essential, particularly for cases where surgical resection is not an option without causing significant morbidity. The development of molecular therapies must rely on building upon our current understanding of ependymoma oncogenesis, as well as cultivating transfer of knowledge based on malignancies with similar genomic alterations.

Utility of a pediatric fast magnetic resonance imaging protocol as surveillance scanning for traumatic brain injury.

OBJECTIVE: Traumatic brain injury (TBI) is a prevalent pediatric pathology in the modern emergency department. Computed tomography (CT) is utilized for detection of TBI and can result in cumulatively high radiation exposure. Recently, a fast brain magnetic resonance imaging (fbMRI) protocol has been employed for rapid imaging of hydrocephalus in pediatric patients. The authors investigate the utility of a modified trauma-focused fbMRI (t-fbMRI) protocol as an alternative to surveillance CT in the setting of acute TBI in pediatric patients, thus reducing radiation exposure while improving diagnostic yield. METHODS: A retrospective review was performed at the authors' institution for all pediatric patients who had undergone t-fbMRI within 72 hours of an initial CT scan, using a 1.5- or 3-T MR scanner for trauma indications. Forty patients met the study inclusion criteria. The authors performed a comparison of findings on the reads of CT and fbMRI, and a board-certified neuroradiologist conducted an independent review of both modalities. RESULTS: T-fbMRI outperformed CT in specificity, sensitivity, and negative predictive value for all injury pathologies measured, except for skull fractures. T-fbMRI demonstrated a sensitivity of 100% in the detection of extraaxial bleed, intraventricular hemorrhage, and subarachnoid hemorrhage and had a sensitivity of 78% or greater for epidural hematoma, subdural hematoma, and intraparenchymal hemorrhage. T-fbMRI yielded a specificity of 100% for all types of intracranial hemorrhages, with a corresponding negative predictive value that exceeded that for CT. CONCLUSIONS: In pediatric populations, the t-fbMRI protocol provides a valid alternative to CT in the surveillance of TBI and intracranial hemorrhage. Although not as sensitive in the detection of isolated skull fractures, t-fbMRI can be used to monitor pathologies implicated in TBI patients while minimizing radiation exposure from traditional surveillance imaging.

Interactions between programmable shunt valves and magnetically controlled growing rods for scoliosis.

OBJECTIVE: Although the advent of magnetic growing rod technology for scoliosis has provided a means to bypass multiple hardware lengthening operations, it is important to be aware that many of these same patients have a codiagnosis of hydrocephalus with magnet-sensitive programmable ventricular shunts. As the magnetic distraction of scoliosis rods has not previously been described to affect the shunt valve setting, the authors conducted an investigation to characterize the interaction between the two devices. METHODS: In this ex vivo study, the authors carried out 360 encounters between four different shunt valve types at varying distances from the magnetic external remote control (ERC) used to distract the growing rods. Valve settings were examined before and after every interaction with the remote control to determine if there was a change in the setting. RESULTS: The Medtronic Strata and Codman Hakim valves were found to have setting changes at distances of 3 and 6 inches but not at 12 inches. The Aesculap proGAV and Codman Certas valves, typically described as MRI-resistant, did not have any setting changes due to the magnetic ERC regardless of distance. CONCLUSIONS: Although it is not necessary to check a shunt valve after every magnetic distraction of scoliosis growing rods, if there is concern that the magnetic ERC may have been within 12 inches (30 cm) of a programmable ventricular shunt valve, the valve should be checked at the bedside with a programmer or with a skull radiograph along with postdistraction scoliosis radiographs.

Simultaneous Bilateral Carotid Thrombectomies: A Technical Note.

BACKGROUND: Although extremely rare, acute bilateral large vessel occlusion (LVO) is a morbid condition that requires prompt intervention. OBJECTIVE: To report the technique used to achieve recanalization of bilateral internal carotid artery (ICA) terminus occlusions. METHODS: This is a case of bilateral ICA terminus occlusions managed with simultaneous bilateral thrombectomies with poor collateral circulation. RESULTS: Recanalization of bilateral ICA with thrombolysis in cerebral infarction (TICI) grade 0 to left TICI 2b flow with distal left A1 occlusion and right TICI 3 flow was achieved in 32 min with the use of simultaneous catheterization and aspiration thrombectomies. CONCLUSION: The described technique offers an efficient and feasible means to reduce time to recanalization and radiation in cases of bilateral LVO.

Impact of a Residency-Integrated Wellness Program on Resident Mental Health, Sleepiness, and Quality of Life.

BACKGROUND: Physicians are poorly trained in balancing the demands of a career in medicine and maintaining personal health. Physician burnout occurs due to demanding hours and psychological conditions unique to the field. Programs that address overall well-being early in residency are necessary to prevent physician burnout and promote physician mental health. OBJECTIVE: To determine the impact of a wellness initiative on anxiety, depression, quality of life, and sleepiness among the resident participants. METHODS: A wellness program was initiated and available to resident physicians in the Medical University of South Carolina Department of Neurosurgery. Participants attended weekly group workout sessions with biweekly lectures on mental health and sleep hygiene. Eight resident participants underwent baseline and final psychological testing in July 2015 and June 2016 including the Personal Health Questionnaire Depression Scale, the Generalized Anxiety Disorder 7-Item Scale, the Quality of Life Scale, and the Epworth Sleepiness Scale. Participant perceptions of the program were also assessed with an anonymous survey. RESULTS: At the conclusion of the pilot year, improvements were observed in anxiety scores (4 to 2.1; P = .039), quality-of-life scores (82.4 to 95.4; P = .007), and sleepiness (8.3 to 5.7; P = .019). In general, resident perceptions of the program were favorable. CONCLUSION: Residency-incorporated wellness programs are achievable and can benefit resident mental health. Lack of a control group limits the interpretation of the results. Programs such as these may be implemented to promote well-being and combat physician burnout and its associated mental health abnormalities.

Transgenerational Inheritance of Familial Lipomyelomeningocele.

Lipomyelomeningocele is a type of neural tube defect characterized by lipomatous tissue causing a defect in the vertebrae, infiltrating the dura, and tethering the spinal cord. Despite significant neurologic consequences, the underlying etiology remains poorly understood. We present a father and son with remarkably similar presentations of lipomyelomeningocele. Genetic testing did not reveal an underlying cause but whole exome sequencing identified variants in the ARHGAP29 and RADIL genes in the proband and his affected father. Genetic analyses of asymptomatic family members revealed several carriers of the ARHGAP29 or RADIL variants, but only the proband and his father carried both variants, suggesting a possible shared genetic mechanism. Rare cases of siblings affected with lipomyelomeningocele have suggested the possibility of autosomal recessive or germline mosaicism. We present the first documented cases of transgenerational lipomyelomeningocele with important implications for family counseling about the recurrence of lipomyelomeningocele.

Pediatric Intraparenchymal Meningioma: Case Report and Comparative Review.

Intra-axial (intraparenchymal) meningiomas are an extremely rare pathology with only dozens of cases reported. In children, the unusual characteristics of intraparenchymal meningiomas can easily create an atypical preoperative differential. The authors present the case of an otherwise healthy 14-year-old girl presenting with new-onset seizures and an intracranial lesion upon investigation. CT and MRI revealed a lesion both cystic and calcified in nature, residing completely within the brain parenchyma. Upon operative examination, the mass was found to be completely surrounded by cortex and to be of two disparate consistencies. Pathological analysis revealed the mass to be a meningioma. Despite the rarity in children, meningiomas must be kept on the differential for cystic, calcified lesions. The differential diagnosis based on imaging as well as the operative strategy taken are further discussed. In addition, the authors review the current understanding of pediatric meningiomas as compared with adult meningiomas.

The feasibility of establishing a free clinic for uninsured patients with neurologic disorders.

The Dream Center Neurology Clinic (DCNC) is a free specialty clinic associated with the Medical University of South Carolina that provides health care for uninsured patients with neurologic disorders. Routine neurologic care is often neglected by free primary care clinics, leaving indigent and uninsured patients to suffer from treatable neurologic ailments. The DCNC was established by supplementing existing resources from a free primary care facility called the Dream Center. Our strategy of building a high-need specialty service into a preexisting primary care infrastructure may provide a blueprint for neurologists who are eager to address the neurologic needs of the underserved in their local communities. According to local charge estimates, the DCNC has provided roughly $120,000 worth of outpatient neurologic care over the past year. The clinic runs through the collaborative effort of medical students as well as academic and private health care providers. Donated services such as EEG, diagnostic lab work, botulinum toxin, supplies, and imaging are also critical to clinic operations. In addition to providing the uninsured with services that are normally inaccessible to them, the DCNC provides a unique educational opportunity for medical students, residents, and all volunteers who are eager to help and learn.

Aneurysmal subarachnoid hemorrhage: a statewide assessment of outcome based on risk factors, aneurysm characteristics, and geo-demography.

OBJECTIVE: Aneurysmal subarachnoid hemorrhage (aSAH) is a devastating illness with nationwide mortality rates reaching almost 50% within the first 30 days. A study was undertaken to evaluate how treatment modality, physical findings, and geo-demography contribute to the outcome of these cases, including complications and disposition status. METHODS: All cases of aSAH in the fiscal year of 2012 (July 2011-June 2012) at the Medical University of South Carolina and Palmetto Health Richland were studied. These healthcare facilities represent 88.5% of aneurysm treatment in the state of South Carolina. Information including aneurysm properties, Hunt-Hess grade, Fisher grade, and symptoms occurring at and after admission were analyzed. RESULTS: 131 patients (94 women and 37 men) with aSAH were treated. 92.4% of cases were treated endovascularly, with more than a third of all cases using balloon-assisted coiling. Hypertension, tobacco use, and hyperlipidemia were the most prevalent comorbidities. Headache, followed by hydrocephalus, motor disturbance, and nausea/vomiting were the most common presenting symptoms. The most common adverse event occurring after hospital admission was acute respiratory failure followed by urinary tract infection, hydrocephalus, and vasospasm. 42.0% were discharged home and nine patients (6.9%) died during hospitalization. CONCLUSIONS: Previously established risk factors such as hypertension and smoking were identified as the most prevalent comorbidities, with disparity between subgroups, particularly women and African Americans. Endovascular treatment was the primary modality of treatment. Mortality rates were lower than previously reported.