Intractable epilepsy impacts many children. Surgically resective and palliative treatments have developed to increase seizure freedom or palliate the seizure burden in those with medically refractory epilepsy. However, surgical epilepsy treatment can confer significant morbidity and death. Endoscope-assisted surgical approaches may be helpful in reducing the morbidity related to traditional open surgical approaches while allowing for good visualization of surgical targets. Here, the authors report a case utilizing an endoscope-assisted keyhole approach to perform a posterior quadrantectomy and corpus callosotomy, achieving the surgical goals of disconnection and reducing the need for large craniotomy exposure. They present the case of a 17-year-old male with medically refractory epilepsy treated with endoscope-assisted posterior quadrantectomy and corpus callosotomy through two mini-craniotomies to achieve a functional disconnection. To the authors’ knowledge, this is the first reported case of an endoscope-assisted approach for a posterior quadrantectomy for surgical epilepsy treatment in an adult or a pediatric patient. The case is reported to highlight the technical nuances and benefits of this approach in select patients as well as the expansion of applications of endoscope-assisted epilepsy surgery.
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Melissa A. LoPresti, Kathryn Wagner, and Sandi Lam
Nathan A. Shlobin, Melissa A. LoPresti, Rebecca Y. Du, and Sandi Lam
OBJECTIVE
Neural tube defects (NTDs) are common congenital neurological defects, resulting in mortality, morbidity, and impaired quality of life for patients and caregivers. While public health interventions that increase folate consumption among women who are or plan to become pregnant are shown to reduce folate-sensitive NTDs, public health policy reflecting the scientific evidence lags behind. The authors aimed to identify the types of policies applied, associated outcomes, and impact of folate fortification and supplementation on NTDs worldwide. By identifying effective legislation, the authors aim to focus advocacy efforts to more broadly effect change, reducing the burden of NTDs in neurosurgery.
METHODS
A systematic review was conducted exploring folate fortification and supplementation policies using the PubMed and Scopus databases. Titles and abstracts from articles identified were read and selected for full-text review. Studies meeting inclusion criteria were reviewed in full and analyzed for study design, aim, population, interventions, and outcomes.
RESULTS
Of 1637 resultant articles, 54 were included. Mandatory folate fortification was effective at reducing folate-sensitive NTDs. Mandatory fortification also decreased hospitalization rates and deaths after discharge and increased 1st-year survival for infants with NTDs. Recommended folate supplementation also resulted in decreased NTDs; however, issues with compliance and adherence were a concern and impacted effectiveness. Folate fortification and/or supplementation resulted in decreased NTD prevalence, although more change was attributed to fortification. Dual policies may hold the most promise. Furthermore, reductions in NTDs were associated with significant cost savings over time.
CONCLUSIONS
Both mandatory folate fortification and recommended supplementation policies were found to effectively decrease folate-sensitive NTD rates when applied. A comprehensive approach incorporating mandatory folate fortification, appropriate folate supplementation, and improved infrastructure and access to prenatal care may lead to decreased NTDs worldwide. This approach should be context-specific, emphasize education, and account for regional access to healthcare and social determinants of health. With wide implications for NTDs, associated health outcomes, quality of life of patients and caregivers, and economic impacts, policy changes can drastically improve global NTD outcomes. As caretakers of children with NTDs, the authors as neurosurgeons advocate for a comprehensive policy, the engagement of stakeholders, and a broader global impact.
V. Jane Horak, Beste Gulsuna, Melissa A LoPresti, and Michael DeCuypere
BACKGROUND
Hydrocephalus is commonly encountered in pediatric neurosurgery. The etiology is diverse, and complexity in management increases in patients with loculated or trapped ventricles. The authors sought to examine a treatment option of endoscopic placement of a triventricular stent in a pediatric patient with complex hydrocephalus and a trapped fourth ventricle.
OBSERVATIONS
In this case, the authors present the treatment of complex hydrocephalus with a trapped fourth ventricle in a pediatric patient using endoscopic placement of a triventricular aqueductal stent. The patient had a complex neurosurgical history, which included over 15 surgeries for shunted hydrocephalus. This case highlights the unique approach used, and the authors discuss surgical nuances of the technique, as well as learning points.
LESSONS
Complex hydrocephalus can be difficult to manage because patients often have multiple catheters, loculated or trapped ventricles, and extensive surgical histories. Endoscopic placement of a triventricular stent can decrease shunt system complexity, restore normal cerebrospinal fluid pathway circulation across the cerebral aqueduct, and promote communication between the ventricles. The authors’ treatment modality resulted in the successful resolution of the trapped fourth ventricle and symptomatic improvement in hydrocephalus.
Denise Brunozzi, Melissa A LoPresti, Jennifer L McGrath, and Tord D Alden
BACKGROUND
Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors.
OBSERVATIONS
The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing.
LESSONS
Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.
Meredith Yang, John Tsiang, Melissa A. LoPresti, and Sandi Lam
BACKGROUND
Neonatal meningitis due to Cronobacter is associated with powdered infant formula. Prompt recognition of this rare but aggressive infection is critical.
OBSERVATIONS
The authors report a unique case of neonatal Cronobacter meningoencephalitis complicated by brain abscess and status epilepticus, requiring surgical intervention in a preterm 4-week-old male and related to contaminated powdered infant formula. They discuss the medical and surgical management in this patient, as well as the role of epilepsy surgery in acute drug-resistant epilepsy. This is paired with a literature review examining Cronobacter infections in infants to provide a summative review of the existing literature.
LESSONS
Cronobacter contamination in powdered infant formula and breast pumps is rare but can cause life-threatening infections. When evaluating patients with Cronobacter central nervous system infections, serial neuroimaging, infection control, and prompt surgical management are essential. Future studies are needed regarding the role of epilepsy surgery in the acute infectious period.
Melissa A. LoPresti, Joshua Nguyen, and Sandi K. Lam
Head immobilization devices with skull pins are commonly used by neurosurgeons to stabilize the head for microsurgical techniques and to maintain accurate intraoperative neuronavigation. Pediatric patients, who may have open fontanelles, unfused sutures, and thin skulls, are vulnerable to complications during placement in pins. We review the various methods of pinning in pediatric neurosurgery and revisit the modified rubber stopper technique using a commonly available rubber stopper from a medication bottle over a standard adult pin of a Mayfield head clamp to prevent the pins from plunging through the thin pediatric skull.
Melissa A. LoPresti, Eric A. Goethe, James C. Bayley, Brandon Bertot, Peter T. Kan, and Sandi Lam
Neonatal intracerebral hemorrhage is associated with substantial morbidity and mortality. Treatment is largely conservative, though interventions to evacuate intraventricular and intraparenchymal hematomas (IPHs) have been applied. Endoscopic ultrasonic aspiration for the treatment of IPH has increasingly been shown to be a useful strategy in adults; however, it has not been studied in children, and the technology has been more commonly applied to intraventricular hemorrhage (IVH). Here, the authors describe, to the best of their knowledge, the first use of endoscopic ultrasonic aspiration for IPH in a newborn.
An 8-week-old female presented with IPH secondary to left M3 aneurysm rupture, which was treated with coil embolization for aneurysm securement and vessel sacrifice, followed by IPH evacuation using endoscopic ultrasonic aspiration. Through applying this approach in a newborn, the authors gained technical insight not previously reported in the application of this technique in similar cases in adults or in cases of IVH. They highlight this case to share learning points and technical challenges regarding the application of endoscopic ultrasonic aspiration in a newborn along with learning points for imaging and visualization. Endoscopic ultrasonic aspiration can be used to treat IPH in select newborns. Further study is needed to improve efficacy and ease when applying this approach in very young patients.
Sabastian Hajtovic, Melissa A. LoPresti, Lu Zhang, Kalman A. Katlowitz, Dominic J. Kizek, and Sandi Lam
OBJECTIVE
Drug-resistant epilepsy (DRE) affects many children. Vagus nerve stimulation (VNS) may improve seizure control; however, its role in children with genetic etiologies of epilepsy is not well described. The authors systematically reviewed the literature to examine the effectiveness of VNS in this cohort.
METHODS
In January 2021, the authors performed a systematic review of the PubMed/MEDLINE, SCOPUS/Embase, Cochrane, and Web of Science databases to investigate the impact of VNS on seizure outcomes in children with genetic etiologies of epilepsy. Primary outcomes included seizure freedom rate, ≥ 90% seizure reduction rate, and ≥ 50% seizure reduction rate. Secondary outcomes were seizure severity and quality of life (QOL), including cognitive, functional, and behavioral outcomes. A random-effects meta-analysis was performed.
RESULTS
The authors identified 125 articles, of which 47 with 216 nonduplicate patients were analyzed. Common diagnoses were Dravet syndrome (DS) (92/216 patients [42.6%]) and tuberous sclerosis complex (TSC) (63/216 [29.2%]). Seizure freedom was not reported in any patient with DS; the pooled proportion (95% CI) of patients with ≥ 50% seizure reduction was 41% (21%–58%). Secondary cognitive outcomes of VNS were variable in DS patients, but these patients demonstrated benefits in seizure duration and status epilepticus. In TSC patients, the pooled (95% CI) seizure freedom rate was 40% (12%–71%), ≥ 90% seizure reduction rate was 31% (8%–56%), and ≥ 50% reduction rate was 68% (48%–91%). Regarding the secondary outcomes of VNS in TSC patients, several studies reported decreased seizure severity and improved QOL outcomes. There was limited evidence regarding the use of VNS to treat patients with other genetic etiologies of epilepsy, such as mitochondrial disease, Rett syndrome, Doose syndrome, Landau-Kleffner syndrome, Aicardi syndrome, Angelman syndrome, ring chromosome 20 syndrome, and lissencephaly; variable responses were reported in a limited number of cases.
CONCLUSIONS
The authors conducted a systematic review of VNS outcomes in children with genetic etiologies of DRE. Among the most studied conditions, patients with TSC had substantial seizure reduction and improvements in QOL, whereas those with DS had less robust seizure reduction. Increased testing, diagnosis, and long-term follow-up studies are necessary to better characterize VNS response in these children.
Emily Winterhalter, Melissa A. LoPresti, Elysa Widjaja, Aman Mohapatra, Nathan A. Shlobin, Lu Zhang, and Sandi Lam
OBJECTIVE
A minority of pediatric patients who may benefit from epilepsy surgery receive it. The reasons for this utilization gap are complex and not completely understood. Patient and caregiver social determinants of health (SDOH) may impact which patients undergo surgery and when. The authors conducted a systematic review examining SDOH and surgical intervention in children with drug-resistant epilepsy (DRE). They aimed to understand which factors influenced time to surgical program referral or receipt of epilepsy surgery among children with DRE, as well as identify areas to characterize the SDOH impacting epilepsy surgery in children and guide efforts aimed to promote health equity in epilepsy.
METHODS
A systematic review was conducted using the PubMed, Embase, and Scopus databases in January 2022. Studies were analyzed by title and abstract, then full text, to identify all studies examining the impact of SDOH on utilization of epilepsy surgery. Studies meeting inclusion criteria were analyzed for SDOH examined, outcomes, and key findings. Quality was assessed using the Grading of Recommendations Assessment, Development and Evaluation system.
RESULTS
Of 4545 resultant articles, 18 were included. Studies examined social, cultural, and environmental factors that contributed to SDOH impacting epilepsy surgery. Patients who underwent surgical evaluation were found to be most commonly White and privately insured and have college-educated caregivers. Five studies found differences in time to referral/surgery or rates of surgery by racial group, with most finding an increased time to referral/surgery or lower rates of surgery for those who were Hispanic and/or non-White. Four studies found that private insurance was associated with higher surgical utilization. Three studies found higher household income was related to surgical utilization. No studies examined biological, psychological, or behavioral factors that contributed to SDOH impacting epilepsy surgery.
CONCLUSIONS
The authors conducted a systematic review exploring the impact of SDOH in DRE surgery utilization. They found that race, ethnicity, insurance type, caregiver educational attainment, and household income demonstrate relationships with pediatric epilepsy surgery. Further study is necessary to understand how these factors, and others not identified in this study, contribute to the low rates of utilization of epilepsy surgery and potential target areas for interventions aiming to increase equity in access to epilepsy surgery in children.
