Moya moya vasculopathy and MECP2 duplication syndrome.

BACKGROUND: Moya moya type vasculopathy (MMV) is a rare disorder in which there is narrowing of bilateral intracranial carotid arteries (Scott and Smith in New Engl J Med 360(12):1226-1237, 2009). MECP2 duplication syndrome (MDS) is a rare genetic disorder that is caused by genetic duplications on Xq28 chromosome (Expanding the clinical picture of the MECP2 duplication syndrome. (Lim et al. in Clin Genet 91(4):557-563, 2017). Both disorders are rare and have not been described together in association. CASE PRESENTATION: Interestingly, we present a child with both MDS and MMV. Upon genetic testing, there was found to be a large, de novo duplication sequence in the patient's genome. Possible correlation between our patient's extensive genetic mutation and MMV has been evaluated. CONCLUSION: Our literature search disclosed no other known patients with both MDS and MMV. Patients with MDS should be monitored carefully for signs or symptoms of vasculopathy.

The landscape of current research on pediatric diffuse midline glioma: a quantitative analysis of shifts, leaders, and future avenues.

PURPOSE: Diffuse midline glioma (DMG) has seen a surge of research interest in recent years with the growth in knowledge of new avenues for potential treatments. However, no bibliometric review of the field has been conducted to visualize the current state of the field. Here, we use bibliometric mapping to visualize the knowledge structure, collaborations, and trends in the field. METHODS: A total of 1079 original and review articles from 1996 to 2023 on diffuse midline glioma were extracted from the Web of Science Core Collection on June 3, 2023. These files were analyzed with R and VOSviewer to construct bibliometric visualizations. RESULTS: Research interest in DMG has continued to grow, driven by publications of original research. Molecular characterization of DMG has been a key focus of recent literature, and terms relating to novel small molecules, mutations, immunotherapy, the blood-brain barrier, and liquid biopsy may be areas for future growth in the literature. Collaborating nations have generally been the North American and European nations, but other nations have begun to make their mark in the field. Leading and rising institutions and journals are described. CONCLUSION: Research in DMG may continue to focus on molecular characterization and new therapeutics based on this knowledge. Novel collaborations between rising nations and institutions in the field may aid in accelerating this research.

Management of an older Marshall-Smith syndrome patient: a review of literature of MSS and craniosynostosis.

Marshall-Smith Syndrome (MSS) is a rare progressive developmental disorder that severely impairs a patient's intellectual development and physical health. The only known cause for MSS is a mutation in the nuclear factor 1 X (NFIX) gene. This mutation affects neuronal development and protein transcription. Historically, most patients with MSS do not survive beyond 3 years of age. Reports of ocular findings are limited. We report a case of a 9-year-old MSS patient with progressive craniosynostosis, elevated intracranial pressure, and catastrophic ocular complications. A comprehensive PubMed literature search from 2018 to August 2022 updating a previous review of older literature produced 72 articles relating to MSS, which are reviewed.

Biopsy of diffuse midline glioma is safe and impacts targeted therapy: a systematic review and meta-analysis.

PURPOSE: To quantify the safety and utility of biopsy of pediatric diffuse midline glioma (DMG). METHODS: This study was conducted in accordance with PRISMA guidelines. PubMed, Embase, Scopus, and Web of Science were queried for relevant articles from inception until June 2023. Two reviewers identified all articles that included diagnostic yield, morbidity, and mortality rates for pediatric DMG patients. Studies that did not present original data or were not in English or peer-reviewed were excluded. Meta-analysis was conducted in R using Freeman-Tukey or logit transformation and DerSimonian-Laird random-effects models. The risk of bias was assessed using the Newcastle-Ottawa Scale. A protocol for this review was not registered. RESULTS: We identified 381 patients from ten studies that met all criteria. DMG biopsy is safe overall (0% mortality, 95% CI: 0-0.6%; 11.0% morbidity, 95% CI: 4.8-18.9%) and has a high diagnostic yield (99.9%, 95% CI: 98.5-100%). The use of stereotactic biopsy is a significant moderator of morbidity (p = 0.0238). Molecular targets can be identified in approximately 53.4% of tumors (95% CI: 37.0-69.0%), although targeted therapies are only delivered in about 33.5% of all cases (95% CI: 24.4-44.1%). Heterogeneity was high for morbidity and identification of targets. The risk of bias was low for all studies. CONCLUSION: We conducted the first meta-analysis of DMG biopsy to show that it is safe, effective, and able to identify relevant molecular targets that impact targeted therapy.

Pediatric skull inflammatory myofibroblastic tumor: a rare case report and literature review.

Inflammatory myofibroblastic tumors (IMTs) represent rare neoplasms, particularly infrequent in the pediatric skull. We present a novel case of a newborn male with a 5 cm right temporal mass and discuss current diagnostic and treatment options for IMTs. A multidisciplinary effort to surgically remove the lesion was successful, and the patient's skull defect healed without neurological deficits. The etiology of IMTs remains elusive, with proposed associations with chromosomal mutations in the anaplastic lymphoma kinase (ALK) gene. Surgical excision remains the primary treatment for IMTs. Promising pharmacological treatments, like Crizotinib, warrant further research into understanding potential alternatives in IMT management.

Congenital unilateral lower lip palsy: a case-based review.

BACKGROUND: Congenital unilateral lower lip palsy (CULLP), also referred to as congenital asymmetric crying facies (ACF), is a rare condition that causes pronounced depression of the unaffected lower lip when crying, despite symmetric appearance of the mouth and lips at rest. Unlike the acquired form of ACF, CULLP is idiopathic and often involves permanent defect. METHODS AND RESULTS: We present a case-based review of CULLP, including a thorough analysis of the relevant literature and a discussion of the exemplary case of 5-year-old patient presenting with unilateral facial asymmetry resulting from left-sided facial weakness. The patient was diagnosed with ACF at birth, and documentation from a previous neurologic consultation specifies the root cause of the asymmetry as developmental aplasia of the left depressor anguli oris muscle (DAOM). However, there is no record of electrodiagnostic testing or B-scan ultrasound imaging that would support this conclusion, and the patient's dysarthric speech may suggest lower motor neuron involvement. Botox chemodenervation of the right, unaffected side was recommended to deanimate the contralateral lower lip and achieve facial symmetry, in addition to potentially resolving some of the patient's speech difficulties. CONCLUSIONS: There are several approaches, both surgical and non-surgical, to the management and correction of CULLP. These include weakening the muscles of the contralateral side or increasing muscular tension on the ipsilateral side, referred to as deanimation and reanimation procedures, respectively.

Neurosurgical management of patients with neurocutaneous melanosis: a systematic review.

OBJECTIVE: Neurocutaneous melanocytosis (NCM), also referred to as neurocutaneous melanosis, is a rare neurocutaneous disorder characterized by excess melanocytic proliferation in the skin, leptomeninges, and cranial parenchyma. NCM most often presents in pediatric patients within the first 2 years of life and is associated with high mortality due to proliferation of melanocytes in the brain. Prognosis is poor, as patients typically die within 3 years of symptom onset. Due to the rarity of NCM, there are no specific guidelines for management. The aims of this systematic review were to investigate approaches toward diagnosis and examine modern neurosurgical management of NCM. METHODS: A systematic review was performed using the PubMed database between April and December 2021 to identify relevant articles using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Search criteria were created and checked independently among the authors. Inclusion criteria specified unique studies and case reports of NCM patients in which relevant neurosurgical management was considered and/or applied. Exclusion criteria included studies that did not report associated neurological diagnoses and neuroimaging findings, clinical reports without novel observations, and those unavailable in the English language. All articles that met the study inclusion criteria were included and analyzed. RESULTS: A total of 26 extracted articles met inclusion criteria and were used for quantitative analysis, yielding a cumulative of 74 patients with NCM. These included 21 case reports, 1 case series, 2 retrospective cohort studies, 1 prospective cohort study, and 1 review. The mean patient age was 16.66 years (range 0.25-67 years), and most were male (76%). Seizures were the most frequently reported symptom (55%, 41/74 cases). Neurological diagnoses associated with NCM included epilepsy (45%, 33/74 cases), hydrocephalus (24%, 18/74 cases), Dandy-Walker malformation (24%, 18/74 cases), and primary CNS melanocytic tumors (23%, 17/74 cases). The most common surgical technique was CSF shunting (43%, 24/56 operations), with tethered cord release (4%, 2/56 operations) being the least frequently performed. CONCLUSIONS: Current management of NCM includes CSF shunting to reduce intracranial pressure, surgery, chemotherapy, radiotherapy, immunotherapy, and palliative care. Neurosurgical intervention can aid in the diagnosis of NCM through tissue biopsy and resection of lesions with surgical decompression. Further evidence is required to establish the clinical outcomes of this rare entity and to describe the diverse spectrum of intracranial and intraspinal abnormalities present.

Spinal arteriovenous malformation in a pediatric patient with a history of congenital syphilis: a case report.

BACKGROUND: Spinal arteriovenous malformations in children are extremely rare and pose great risk for intraoperative hemorrhage. Congenital syphilis sometimes presents with vascular symptoms, however, there is little published on patients with a history of congenital syphilis presenting with spinal arteriovenous malformations. CASE PRESENTATION: A 15-month-old female with a history of congenital syphilis presented with urinary retention, fever, and subacute onset of paraplegia. MRI showed a lesion at T8-L1, angiogram was performed which confirmed the presence of a complex type IVc arteriovenous malformation and fistula from Artery of Adamkiewicz at L1-L2. It also showed peri medullary dilated veins and a pseudoaneurysm that compressed the spinal cord at T8-T10. Somatosensory evoked potentials and motor-evoked potentials were not recordable on the bilateral lower extremities prior to surgery. Once the patient was optimized for surgery, osteoplastic laminotomies from T6-T12 were performed. The dura was opened and the intradural, intramesenchymal hematoma was evacuated. There were two episodes of brisk arterial bleeding with hypotension during resection of the hematoma. The patient was taken to the angiography suite from the OR to successfully coil the large aneurysm. Intraoperative spinal cord monitoring remained undetectable in the bilateral lower extremities. The patient's paraplegia remained unchanged from preoperative presentation. CONCLUSION: Congenital syphilis may present with vascular changes that might impact surgical approaches and treatment outcomes in patients with spinal arteriovenous malformations. Preparation for massive transfusion and intraoperative monitoring are imperative in ensuring a safe perioperative experience.

Chiari I malformation with Klippel-Trenaunay syndrome: case report and review of the literature.

We present a rare case of an 8-year-old male with Klippel-Trenaunay syndrome (KTS) and a Chiari I malformation (CIM). Magnetic resonance imaging (MRI) to investigate facial asymmetry and speech delay at age two revealed CIM with cerebellar tonsils 1.3 cm below the foramen magnum without syringomyelia. The patient underwent a craniectomy and posterior fossa decompression with C1 laminectomy. While gene sequencing determined the patient was negative for the PIK3CA gene mutation, the patient's clinical history strongly suggests KTS. He has hemihypertrophy, leg length discrepancy, hemangiomas and pigmentary mosaicism along the upper and lower extremities, heart murmur, chronic low heart rate, recurrent hip pain, and mild scoliosis. Neurodevelopmental concerns include difficulty reading, attention deficit hyperactivity disorder (ADHD), anxiety, and difficulty running and going downstairs. His most recent MRI shows good decompression at the cervicomedullary junction, global cerebrospinal fluid (CSF) flow, and less peg-like cerebellar tonsils. Also noted were two intravertebral hemangiomas at T5 and T6. While the patient's speech has improved, there is still difficulty with the expressive language. He still has mild delays, runs slowly, and does not alternate feet when climbing stairs. The patient is being followed by multiple specialists including neurology, hematology-oncology, genetics, orthopedic surgery, and developmental pediatrics.

The utilization of sodium fluorescein in pediatric brain stem gliomas: a case report and review of the literature.

INTRODUCTION: A major challenge in the surgical resection of brainstem tumors is distinguishing tumor from normal tissue. One approach for addressing this problem is the use of fluorescent tracers such as sodium fluorescein (NaFl). NaFl disseminates through a disruption in the blood-brain barrier (BBB) and accumulates in the extracellular space of brain tumors. Intraoperative fluorescence microscopy can be performed to identify tumor tissue and avoid damage to adjacent, normal tissue. Here, we present the case of a 16-year-old male who underwent a left retrosigmoid craniotomy with splitting of the tentorium to remove a large exophytic brainstem tumor involving the cerebellar peduncle and with superior extension into the midbrain and thalamus. OBJECTIVES: The primary objective of this study was to investigate the effectiveness of sodium fluorescein as an intraoperative technique and evaluate its potential benefit for resection of tumors in eloquent regions in the pediatric population. To do so, we focused on a case study approach; however, we also performed a literature review and evaluated different intraoperative fluorescent techniques and their benefits for tumor resection. METHODS: We performed a literature search using PubMed and Google Scholar by the key words "sodium fluorescein," "brain stem tumor," and "central nervous system neoplasms." Twenty-nine articles including both pediatric and adult populations were selected for analysis and qualitative review. RESULTS: In this case study, sodium fluorescein helped the surgeons to identify and obtain a gross total resection of a large brainstem tumor. The marker was especially helpful for discerning the inferior pole of the tumor buried inconspicuously in cerebellar tissue. We evaluate different fluorescent tracers, 5-ALA and ICG, and discuss their application and benefits in tumor resection surgery. We present different cases that found sodium fluorescein to be helpful in achieving a gross total resection. CONCLUSION: The application of sodium fluorescein proved to be a safe and effective technique for the resection of brain stem tumors as shown in this case study. It helped to expose concealed areas and illuminate the tumor capsule. Further studies should test the clinical use of sodium fluorescein on brain stem tumor resection.

Treatment options and long-term outcomes in pediatric spinal cord vascular malformations: a case report and review of the literature.

A 16-month-old female was admitted for prolonged fever, gait ataxia, and neurogenic bowel and bladder. Neurological exam was significant for decreased sensory and motor functions in bilateral lower extremities. Initial MRI showed a thoracic spine hematoma and diagnostic angiogram revealed a large AVM and aneurysm. The patient underwent surgical resection of the hematoma and AVM, as well as clipping and later endovascular coiling of the aneurysm. Due to significant hemorrhage perioperatively, she developed spastic paraplegia improved by baclofen and onabotulinumtoxin A injections. The aims of this paper were to conduct a systematic review of the literature on pediatric spinal cord vascular malformations and analyze trends in treatment options and long-term neurological outcomes. PubMed searches were conducted using keywords "pediatric spinal vascular malformation" and "pediatric spinal AVM", yielding 34 results after abstract screening and cross-reference. Endovascular embolization was determined to have better long-term outcomes, with 10/19 (52.6%) patients with postoperative complications associated with open vascular surgeries. Open versus endovascular surgical decisions can be difficult with unique spinal AVM pathologies in pediatric patients. Important considerations such as size, location, neurological deficits, and risk of rupture are important factors to consider in treating these patients. We recommend endovascular treatment as a first-line approach due to lower risk of hemorrhage and postoperative deficits.

X-ray Imaging Analysis of Intrathecal Baclofen Pumps for Pediatric Emergency Medicine.

Intrathecal baclofen therapy has become increasingly popular because of its effectiveness in treating spasticity in pediatric patients. The device implanted into each patient is structurally similar. However, x-ray imaging can give the appearance of missing or extraneous objects on or near the intrathecal baclofen device. The composition of the material used in making the catheters is transparent on x-ray images. In addition, the connection port between the pump and catheter may resemble the shape of a needle. This has led to confusion among emergency room personnel, and unnecessary panic for other people involved. It is important that medical professionals understand many of the new designs of the pump-connector system to avoid further complications.

Understanding the reasons for delayed referral for intrathecal baclofen therapy in pediatric patients with severe spasticity.

OBJECTIVE: Intrathecal baclofen therapy (ITB) has been used in the treatment of spasticity and dystonia. In our pediatric movement disorder clinic, we noted a delay in referral of patients for consideration of ITB. Often, only after years of failed medical therapy, a baclofen pump is considered. This study attempts to investigate the prevalence, length and causes of the delay. METHODS: A retrospective, outcome analysis was performed. We conducted a survey of 30 pediatric patients who received baclofen pumps between the ages of 5 and 23. Patients were divided into two groups (before and after ITB approval by the US Food and Drug Administration in 1996) (FDA/Center for Drug Evaluation and Research, 2014; Ridley and Rawlins, J Neurosci Nurs 38:72-82, 2006; Medical Advisory Secretariat, Ont Health Technol Assess Ser 5:1-93, 2005). Information was collected regarding their onset of spasticity, attempted treatments, pump referral, satisfaction, and resulting change in the quality of life. RESULTS: There was a delay in referral in most cases investigated. Average time to baclofen pump implantation, after initial onset of spasticity, was 5.14 years (group A) and 11.7 years (group B). Out of the subjects who reported diminished effects or no effect of pharmacological treatment, 93 % of these respondents reported that ITB had a dramatic long-lasting effect on their spasticity. Of 30 patients, 28 reported effectiveness of ITB, and 26 of 30 subjects reported an improved quality and ease of life. CONCLUSION: Despite the limitations of this subjective retrospective analysis of outcomes and delay in referral, the opinions of the parents and caregivers should be considered. Earlier referral for ITB therapy may better treat severe spasticity in pediatric patients.

The Accreditation Council for Graduate Medical Education 20-Year Trends in Diversity, Equity, and Inclusion in the United States: How Does Neurological Surgery Compare?

BACKGROUND: Within the current medical workforce, diversity is limited among surgical specialties. However, diversity allows physicians to provide culturally competent care. This paper discusses the trends in racial, ethnic, and gender representation within different surgical subspecialties with an emphasis on neurosurgery over a 20-year time frame. METHODS: A retrospective review of data collected by the Accreditation Council for Graduate Medical Education over the past twenty years, as reported in Journal of the American Medical Association, was conducted. Residents from 5 surgical specialties were evaluated based on gender, race, and ethnic identifications from 2002 to 2022. One-way analysis of variance was performed to compare the levels and retention rates of racial, ethnic, and gender diversity within these specialties. RESULTS: Analysis of resident demographics of the 5 surgical specialties reveals an overall trend of increasing diversity over the study period. Over the past 20 years, neurosurgery had an overall increase in Asian (+5.1%), Hispanic (+3.0%), and female (+11.4%) residents, with a decrease in White residents by 2.1% and Black residents by 1.1%. Among the surgical specialties analyzed, otolaryngology had the greatest overall increase in minority residents. Notably, there has been an overall increase in female residents across all 5 surgical specialties, with the highest in otolaryngology (+20.3%) which was significantly more than neurosurgery (P < 0.001). CONCLUSIONS: This chronological analysis spanning 20 years demonstrates that neurosurgery, like other specialties, has seen a growth in several racial and ethnic categories. Relative differences are notable in neurosurgery, including Black, Asian, Hispanic, and White ethnic categories, with growth in females, but at a significantly lesser pace than seen in otolaryngology and plastic surgery.

Utilization of Locum Tenens in Neurosurgery.

INTRODUCTION: Despite its rising popularity, little has been described about locum tenens employment (locums) in neurosurgery. This study provides the first nationwide overview of the locums neurosurgery experience. METHODS: An anonymous online survey examined practice characteristics of respondents, extent of and satisfaction with locums, motivations for pursuing locums, case volumes, agencies used, compensation, and positive/negative aspects of experiences. Responses were collected between November 2020 and February 2021. RESULTS: Response rate for the 1852 neurosurgeons who opened the survey request was 4.9%; 36 of 91 respondents had previously worked locums and were commonly motivated by compensation or transitioning to new jobs or retirement. In our response group, 92% of locums respondents had taken more than one position and 47% had taken more than 10. Neurosurgeons performing <200 cases/year were significantly more likely to have also worked locums than those performing >200 cases/year (41.6% locums, 12.7% non-locums, P = 0.001). Responses showed that 69% of locums respondents earned $2000-$2999/day and 16% earned >$3500/day. Nearly 78% of locums respondents were satisfied with their experience(s) and 86% would take another future locums position. Being in practice for >15 years was significantly associated with satisfaction with locums (P = 0.03). Reported flaws included unfamiliarity with hospitals, limited continuity of care, credentialing burdens, and inadequate travel compensation. CONCLUSIONS: Locums is utilized by neurosurgeons across multiple practice types and may serve to complement workloads or "fill in gaps" between longer-term employment. Overall, locums neurosurgeons are well compensated, and the majority are satisfied with their experience(s). Inevitably, flaws still exist with locums employment, which may be the focus of organized efforts aiming to improve the experience.

Mechanisms of Peer Review and Their Potential Impact on Neurosurgeons: A Pilot Survey.

BACKGROUND: Physician peer review is a universal practice in U.S. hospitals. While there are many commonalities in peer review procedures, many of them established by law, there is also much institutional variation, which should be well understood by practicing neurosurgeons. METHODS: A 13-question pilot survey was conducted of a sample of 5 hospital systems with whom members of the Council of State Neurosurgical Societies Medico-Legal Committee are affiliated. Survey questions were constructed to qualitatively assess 3 features of hospital peer review: 1) committee composition and process, 2) committee outcomes, and 3) legal protections and ramifications. RESULTS: The most common paradigm for a physician peer review committee was an interdisciplinary group with representatives from most major medical and surgical subspecialties. Referrals for peer review inquiry could be made by any hospital employee and were largely anonymous. Most institutions included a precommittee screening process conducted by the physician peer review committee leadership. The most common outcomes of an inquiry were resolution with no further action or ongoing focused professional practice evaluation. Hospital privileges were only rarely reported to be revoked or terminated. Members of the physician peer review committee were consistently protected from retaliatory litigation related to peer review participation. Most hospitals had a multilayered decision process and availability of appeal to minimize potential for punitive investigations. CONCLUSIONS: According to a recent study, only 62% of hospitals consider their peer review process to be highly or significantly standardized. This pilot survey provides commentary of potential areas of commonality and variation among hospital peer review practices.

Trends in Racial and Ethnic Representation Among Neurosurgery Applicants and Residents: A Comparative Analysis of Procedural Specialties.

BACKGROUND: Diversity, equity, and inclusion within the healthcare workforce are conducive to providing culturally competent care. However, few existing studies have assessed the level of racial and ethnic diversity among resident physicians and residency applicants. Our objective was to provide a comparative analysis of the trends in racial and ethnic representation within different subspecialties in medicine. METHODS: Using data from the American Association of Medical Colleges and the Journal of the American Medical Association, we evaluated the racial and ethnic identification of residency applicants and current residents in 9 procedural-focused specialties from 2005 to 2019 and performed a descriptive analysis to compare the different levels of racial and ethnic diversity in these specialties. RESULTS: Among the specialties analyzed during the study period, neurosurgery had the greatest magnitude of differences between Black/African-American residency applicants and current residents. The percentage of Black/African-American applicants was 92% greater than that of Black/African-American residents (10% of applicants vs. 5.2% of residents). In contrast, the percentage of White neurosurgery residents was 17.6% greater than that of White neurosurgery applicants (53.9% of applicants vs. 63.4% of residents). Similar trends were noted in all the specialties evaluated. Obstetrics and gynecology demonstrated the least disparity between Black/African-American applicants and residents (13.7% of applicants vs. 10.2% of residents; 35.4% difference). Hispanic and Asian representation varied widely between specialties. CONCLUSIONS: Among the surveyed specialties, neurosurgery demonstrated the greatest disparity between the percentage of Black/African-American residency applicants and current residents. To further drive progress in this domain, we advocate for a series of initiatives designed to increase underrepresented minority participation in neurosurgery practice and scholarship.

Telehealth in Neurosurgery: 2021 Council of State Neurosurgical Societies National Survey Results.

OBJECTIVE: Telehealth was rapidly adopted during the COVID-19 pandemic. A survey was distributed to neurosurgeons in the United States (U.S.) to understand its use within neurosurgery, what barriers exist, unique issues related to neurosurgery, and opportunities for improvement. METHODS: A survey was distributed via email and used the SurveyMonkey platform. The survey was sent to 3828 practicing neurosurgeons within the U.S., 404 responses were collected between October 30, 2021 and December 4, 2021. RESULTS: During the pandemic, telehealth was used multiple times per week by 60.65% and used daily by an additional 12.78% of respondents. A supermajority (89.84%) of respondents felt that evaluating patients across state lines with telemedicine is beneficial. Most respondents (95.81%) believed that telehealth improves patient access to care. The major criticism of telehealth was the inability to perform a neurological exam. CONCLUSIONS: Telehealth has been widely implemented within the field of neurosurgery during the COVID-19 pandemic and has increased access to care. It has allowed patients to be evaluated remotely, including across state lines. While certain aspects of the neurological exam are suited for video evaluation, sensation and reflexes cannot be adequately assessed. Neurosurgeons believe that telehealth adds value to their ability to deliver care.

Sodium Fluorescein-Guided Gross Total Resection of Pediatric Exophytic Brainstem Glioma: 2-Dimensional Operative Video.

Brainstem gliomas comprise 10% to 20% of pediatric intracranial tumors.1 Gross total resection is associated with the greatest long-term survival.1 However, due to the eloquence of surrounding brain tissue, an aggressive resection is not always achievable and can be associated with significant risk.2 Sodium fluorescein can be used to help differentiate between tumor and normal tissue.3,4 In this operative video, we demonstrate the step-by-step technique to utilize fluorescence in surgically resecting an exophytic glioma arising from the midbrain in a 16-yr-old male. Technical nuances are highlighted in this operative video, including the use of the YELLOW 560 (Zeiss) filter to differentiate fluorescent tumor from normal tissue as well as strategic splitting of the tentorium to expose the supratentorial component of the tumor. A decrease in motor potentials of the right leg during the case did not translate into neurological worsening postoperatively; at 3-mo follow-up, the patient attests to better strength and coordination on his affected side. In summary, sodium fluorescein can be used as a critical adjunctive tool for successful surgery in the case of a brainstem tumor. Alternatives to this procedure included gross total resection with 5-aminolevulinic acid, subtotal resection with chemotherapy, and active monitoring, but were not chosen due to their limitations in this clinical case.5-7 Appropriate patient consent was obtained to perform this procedure and present this clinical case and surgical video for academic purposes.