MR-guided focused ultrasound in pediatric neurosurgery: current insights, technical challenges, and lessons learned from 45 treatments at Children's National Hospital.

OBJECTIVE: MR-guided focused ultrasound (MRgFUS) is an evolving technology with numerous present and potential applications in pediatric neurosurgery. The aim of this study was to describe the use of MRgFUS, technical challenges, complications, and lessons learned at a single children's hospital. METHODS: A retrospective analysis was performed of a prospectively collected database of all pediatric patients undergoing investigational use of MRgFUS for treatment of various neurosurgical pathologies at Children's National Hospital. Treatment details, clinical workflow, and standard operating procedures are described. Patient demographics, procedure duration, and complications were obtained through a chart review of anesthesia and operative reports. RESULTS: In total, 45 MRgFUS procedures were performed on 14 patients for treatment of diffuse intrinsic pontine glioma (n = 12), low-grade glioma (n = 1), or secondary dystonia (n = 1) between January 2022 and April 2024. The mean age at treatment was 9 (range 5-22) years, and 64% of the patients were male. With increased experience, the total anesthesia time, sonication time, and change in core body temperature during treatment all significantly decreased. Complications affected 4.4% of patients, including 1 case of scalp edema and 1 patient with a postprocedure epidural hematoma. Device malfunction requiring abortion of the procedure occurred in 1 case (2.2%). Technical challenges related to transducer malfunction and sonication errors occurred in 6.7% and 11.1% of cases, respectively, all overcome by subsequent user modifications. CONCLUSIONS: The authors describe the largest series on MRgFUS technical aspects in pediatric neurosurgery at a single institution, comprising 45 total treatments. This study emphasizes potential technical challenges and provides valuable insights into the nuances of its application in pediatric patients.

The role of focused ultrasound for pediatric brain tumors: current insights and future implications on treatment strategies.

INTRODUCTION: Focused ultrasound (FUS) is an innovative and emerging technology for the treatment of adult and pediatric brain tumors and illustrates the intersection of various specialized fields, including neurosurgery, neuro-oncology, radiation oncology, and biomedical engineering. OBJECTIVE: The authors provide a comprehensive overview of the application and implications of FUS in treating pediatric brain tumors, with a special focus on pediatric low-grade gliomas (pLGGs) and the evolving landscape of this technology and its clinical utility. METHODS: The fundamental principles of FUS include its ability to induce thermal ablation or enhance drug delivery through transient blood-brain barrier (BBB) disruption, emphasizing the adaptability of high-intensity focused ultrasound (HIFU) and low-intensity focused ultrasound (LIFU) applications. RESULTS: Several ongoing clinical trials explore the potential of FUS in offering alternative therapeutic strategies for pathologies where conventional treatments fall short, specifically centrally-located benign CNS tumors and diffuse intrinsic pontine glioma (DIPG). A case illustration involving the use of HIFU for pilocytic astrocytoma is presented. CONCLUSION: Discussions regarding future applications of FUS for the treatment of gliomas include improved drug delivery, immunomodulation, radiosensitization, and other technological advancements.

Evolution of Surgical Management of Pineal Region Tumors in the Pediatric Population: A 17-Year Experience at a Single Institution.

INTRODUCTION: Pineal region tumors have historically been challenging to treat. Advances in surgical techniques have led to significant changes in care and outcomes for these patients, and this is well demonstrated by our single institution's experience over a 17-year-period in which the evolution of diagnosis, treatment, and outcomes of pineal tumors in pediatric patients will be outlined. METHODS: We retrospectively collected data on all pediatric patients with pineal region lesions treated with surgery at Children's National Hospital (CNH) from 2005 to 2021. Variables analyzed included presenting symptoms, presence of hydrocephalus, diagnostic and surgical approach, pathology, and adverse events, among others. IRB approval was obtained (IRB: STUDY00000009), and consent was waived due to minimal risk to patients included. RESULTS: A total of 43 pediatric patients with pineal region tumors were treated during a 17-year period. Most tumors in our series were germinomas (n = 13, 29.5%) followed by pineoblastomas (n = 10, 22.7%). Twenty seven of the 43 patients (62.8%) in our series received a biopsy to establish diagnosis, and 44.4% went on to have surgery for resection. The most common open approach was posterior interhemispheric (PIH, transcallosal) - used for 59.3% of the patients. Gross total resection was achieved in 50%; recurrence occurred in 20.9% and mortality in 11% over a median follow-up of 47 months. Endoscopic third ventriculostomy (ETV) was employed to treat hydrocephalus in 26 of the 38 patients (68.4%) and was significantly more likely to be performed from 2011 to 2021. Most (73%) of the patients who received an ETV also underwent a concurrent endoscopic biopsy. No difference was found in recurrence rate or mortality in patients who underwent resection compared to those who did not, but complications were more frequent with resection. There was disagreement between frozen and final pathology in 18.4% of biopsies. CONCLUSION: This series describes the evolution of surgical approaches and outcomes over a 17-year-period at a single institution. Complication rates were higher with open resection, reinforcing the safety of pursuing endoscopic biopsy as an initial approach. The most significant changes occurred in the preferential use of ETVs over ventriculoperitoneal shunts. Though there has been a significant evolution in our understanding of and treatment for these tumors, in our series, the outcomes for these patients have not significantly changed over that time.

Clinical outcomes and complications of eyelid versus eyebrow approaches to supraorbital craniotomy: systematic review and indirect meta-analysis.

OBJECTIVE: Eyebrow supraorbital craniotomy is a versatile keyhole technique for treating intracranial pathologies. The eyelid supraorbital approach, an alternative approach to an eyebrow supraorbital craniotomy, has not been widely adopted among most neurosurgeons. The purpose of this systematic review and meta-analysis was to perform a pooled analysis of the complications of eyebrow or eyelid approaches for the treatment of aneurysms, meningiomas, and orbital tumors. METHODS: A systematic review of the literature in the PubMed, Embase, and Cochrane Review databases was conducted for identifying relevant literature using keywords such as "supraorbital," "eyelid," "eyebrow," "tumor," and "aneurysm." Eyebrow supraorbital craniotomies with or without orbitotomies and eyelid supraorbital craniotomies with orbitotomies for the treatment of orbital tumors, intracranial meningiomas, and aneurysms were selected. The primary outcomes were overall complications, cosmetic complications, and residual aneurysms and tumors. Secondary outcomes included five complication domains: orbital, wound-related, scalp or facial, neurological, and other complications. RESULTS: One hundred three articles were included in the synthesis. The pooled numbers of patients in the eyebrow and eyelid groups were 4689 and 358, respectively. No differences were found in overall complications or cosmetic complications between the eyebrow and eyelid groups. The proportion of residuals in the eyelid group (11.21%, effect size [ES] 0.26, 95% CI 0.12-0.41) was significantly higher (p < 0.05) than that in the eyebrow group (6.17%, ES 0.10, 95% CI 0.08-0.13). A subgroup analysis demonstrated significantly higher incidences of orbital, wound-related, and scalp or facial complications in the eyelid group (p < 0.05), but higher other complications in the eyebrow group. Performing an orbitotomy substantially increased the complication risk. CONCLUSIONS: This is the first meta-analysis that quantitatively compared complications of eyebrow versus eyelid approaches to supraorbital craniotomy. This study found similar overall complication rates but higher rates of selected complication domains in the eyelid group. The literature is limited by a high degree of variability in the reported outcomes.

Isolated subependymal giant cell astrocytoma (SEGA) in the absence of clinical tuberous sclerosis: two case reports and literature review.

PURPOSE: Subependymal giant cell astrocytoma (SEGA) is a WHO grade I pediatric glioma arising in 5-15% of patients with tuberous sclerosis (TSC). Rare cases of isolated SEGA without TSC have been described. The etiology, genetic mechanisms, natural history, and response to treatment of these lesions are currently unknown. We describe two such cases of isolated SEGA with follow-up. METHODS: Retrospective review was performed at a single institution to describe the clinical course of pathology-confirmed SEGA in patients with germline testing negative for TSC mutations. RESULTS: Two cases of isolated SEGA were identified. Genetic analysis of the tumor specimen was available for one, which revealed an 18 base pair deletion in TSC1. Both cases were managed with surgical resection, one with preoperative embolization. In spite of a gross total resection, one patient experienced recurrence after three years. Treatment with an mTOR inhibitor led to a significant interval reduction of the mass on follow-up MRI. The patient tolerated the medication well for 6 years and is now off of treatment for 2 years with a stable lesion. CONCLUSION: Cases of SEGA outside of the context of TSC are exceedingly rare, with only 48 cases previously described. The genetic mechanisms and treatment response of these lesions are poorly understood. To date, these lesions appear to respond well to mTOR inhibitors and may behave similarly to SEGAs associated with TSC. However, given that experience is extremely limited, these cases should be followed long term to better understand their natural history and treatment response.

Minimally Invasive Surgical Decompression without Fusion for the Treatment of Lumbar Synovial Cysts: Feasibility and Long-Term Outcomes.

BACKGROUND: Lumbar synovial cysts (LSCs) can cause painful radiculopathy and sensory and/or motor deficits. Historically, first-line surgical treatment has been decompression with fusion. Recently, minimally invasive laminectomy without fusion has shown equal or superior results to traditional decompression and fusion methods. OBJECTIVE: This study investigates the long-term efficacy of minimally invasive laminectomy without fusion in the treatment of LSC as it relates to the rate of subsequent fusion surgery. METHODS: A retrospective review was performed over a 10-year period of patients undergoing minimally invasive laminectomy for symptomatic LSCs. The primary end point was the rate of revision surgery requiring fusion. RESULTS: Eighty-five patients with symptomatic LSCs underwent minimally invasive laminectomy alone January 2010-August 2020 at our institution. The most common location was L4-5 (72%). Preoperative imaging identified spondylolisthesis (grade 1) in 43 patients (57%), none of which was unstable on available dynamic radiographs. Average procedure duration was 93 minutes, with 78% of patients discharged home on the same day of surgery. Over 46 months of mean follow-up, 17 patients (20%) required 19 revision operations. Of those operations, 16 were spinal fusions (17.6%). Median time to fusion surgery was 36 months. There were no identifiable risk factors on multivariate regression analysis that predicted the need for fusion. CONCLUSIONS: Minimally invasive laminectomy is an effective first-line treatment for symptomatic LSCs and avoids the need for fusion in most treated patients. Of our patients, 18% required a fusion over 46 months, suggesting that further studies are required to guide patient selection.

Cirq robotic arm-assisted transpedicular instrumentation with intraoperative navigation: technical note and case series with 714 thoracolumbar screws.

The Cirq is a surgeon-controlled robotic arm that provides a new technique for accurately placing transpedicular screws. This report aims to present a technical report and our experience with this new robotic arm combined with intraoperative navigation. Technique and workflow using the Cirq robotic arm with intraoperative navigation is described. A retrospective review was conducted of all patients undergoing elective open thoracic/lumbar fusion surgery by a single surgeon in the first year of using the novel Cirq robotic arm. Descriptive analysis of patient and operative variables was performed. A total of 84 patients underwent placement of a total 714 transpedicular screws using the Cirq robotic arm. Most (69.1%) underwent 3-6 level fusion procedures. Mean operative time was 198 min total and 28 min when adjusted per screw. There was a learning curve with operative time per screw decreasing from 32 to 25 min from the first to second half of cases (p = 0.057). There were no intraoperative screw revisions and 2.4% (2/84) required instrumentation revision and return to the operating room. The Cirq robotic arm is seamlessly incorporated into the workflow of a transpedicular fusion. Our experience with over 700 pedicle screw placements using the Cirq robot demonstrates efficacy and safety although further comparative studies are needed.

Pituitary Apoplexy Case Series: Outcomes After Endoscopic Endonasal Transsphenoidal Surgery at a Single Tertiary Center.

BACKGROUND: The management of pituitary apoplexy, caused by acute hemorrhage and/or infarction of a pituitary adenoma, is debated. OBJECTIVE: To analyze clinical characteristics of patients undergoing endoscopic endonasal approaches (EEAs) for pituitary apoplexy. METHODS: A retrospective review of patients at our institution from 2012 to 2018 undergoing EEA for pituitary apoplexy diagnosed clinically and with imaging/pathologic findings. Analysis included demographics, symptoms, neuroendocrine deficits, neuroimaging, complications, symptom resolution, and follow-up details. RESULTS: Fifty patients (mean age, 53 years) were included. Preoperative symptoms included headache (86%), vision loss (62%), and cranial nerve paresis (40%). Mean tumor diameter was 2.7 cm and extrasellar extension was observed in 96% of tumors. Twenty-eight tumors were hemorrhagic (76%), 24 were necrotic (65%), and 13 (35%) had both features. Magnetic resonance imaging showed gross total resection in 58% of patients. Headache and vision loss improved in 87% and 86% of presenting patients. Cranial nerve paresis resolved in 72% of patients, partially improved in 11%, and remained unchanged in 17%. There were no deaths or carotid artery injuries. Surgical complications included postoperative cerebrospinal fluid leak (n = 4, 8%), epistaxis (n = 2, 4%), postoperative abscess (n = 1, 2%), and transient postoperative vision loss requiring reoperation (n = 1, 4%). Endocrinopathies improved in 21% of patients and panhypopituitarism persisted in 48% and developed in 6% of patients. Mean follow-up time was 26 months; 2 patients experienced recurrence. CONCLUSIONS: EEA for pituitary apoplexy is effective in rapidly improving headache and visual symptoms. Although neuro-ophthalmic deficits often improve over time, panhypopituitarism persists in most patients after surgical resection.

Dual responsive neurostimulation implants for epilepsy.

Closed-loop brain-responsive neurostimulation via the RNS System is a treatment option for adults with medically refractory focal epilepsy. Using a novel technique, 2 RNS Systems (2 neurostimulators and 4 leads) were successfully implanted in a single patient with bilateral parietal epileptogenic zones. In patients with multiple epileptogenic zones, this technique allows for additional treatment options. Implantation can be done successfully, without telemetry interference, using proper surgical planning and neurostimulator positioning.Trajectories for the depth leads were planned using neuronavigation with CT and MR imaging. Stereotactic frames were used for coordinate targeting. Each neurostimulator was positioned with maximal spacing to avoid telemetry interference while minimizing patient discomfort. A separate J-shaped incision was used for each neurostimulator to allow for compartmentalization in case of infection. In order to minimize surgical time and risk of infection, the neurostimulators were implanted in 2 separate surgeries, approximately 3 weeks apart.The neurostimulators and leads were successfully implanted without adverse surgical outcomes. The patient recovered uneventfully, and the early therapy settings over several months resulted in preliminary decreases in aura and seizure frequency. Stimulation by one of the neurostimulators did not result in stimulation artifacts detected by the contralateral neurostimulator.

Outcomes Following Transsphenoidal Pituitary Surgery in the Elderly: A Retrospective Single-Center Review.

BACKGROUND: Transsphenoidal surgery (TSS) for pituitary adenomas (PAs) is performed on elderly patients with increasing frequency. More research is necessary to assess the risk factors that are associated with TSS in an aging population. OBJECTIVE: To perform a retrospective study on postoperative safety outcomes following TSS in the elderly population stratified by decade of life. METHODS: A retrospective chart review of the USC Pituitary Database was conducted to identify patients ≥70 yr, who underwent TSS for PAs between 1995 and 2017. Surgical outcomes were analyzed in elderly (≥70 yr) vs nonelderly (<70 yr) patients. Elderly patients were additionally stratified according to age 70 to 79 vs ≥80 yr. RESULTS: The cohorts included 115 elderly patients (70-79 yr: 94 patients; ≥80 yr: 21 patients) and 770 nonelderly patients. Elderly patients presented with more vision loss (62% vs 38%, P < .0001), dizziness (17% vs 6%, P = .0001) and altered mental status (7.3% vs 3%, P = .0451). Overall rates of surgical, medical, and endocrine complications were similar; however, elderly patients had significantly higher rates of postoperative hyponatremia (9.3% vs 4.7%, P = .0401) and lower rates of transient diabetes insipidus (0.9% vs 7.9%, P = .0038). Patients ≥ 80 yr had significantly more surgical complications (26.3%) compared to the 70 to 79 group (7.87%; P = .021) and <70 group (12.5%; P = .04). CONCLUSION: Patients >70 yr are appropriate surgical candidates for TSS given the similar safety outcomes as younger patients. Given the higher propensity for surgical complications; however, a higher level of operative selectivity should be maintained in octogenarian patients.

Variability and Lack of Prognostic Value Associated With Atypical Pituitary Adenoma Diagnosis: A Systematic Review and Critical Assessment of the Diagnostic Criteria.

BACKGROUND: Atypical pituitary adenomas (APAs) are a subset of pituitary adenomas (PAs) characterized by the 2004 World Health Organization (WHO) guidelines to have higher risk histopathological features than typical PAs. In July 2017, the WHO published an update to their classification of pituitary tumors and abandoned the APA terminology. OBJECTIVE: To assess the prevalence and outcomes of patients diagnosed with APA through a literature review. Focus was placed on variation in the application of the previous WHO criteria and on rates of recurrence. METHODS: A systematic review of PubMed (2004-July 2017) was performed to identify studies reporting prevalence and clinical characteristics/outcomes of APA. Eight studies were analyzed for prevalence. Six studies reporting histopathological details were analyzed in depth. RESULTS: Of the 7105 included patients, 373 (5.2%) met criteria for APA (prevalence range: 3%-15%). Only 2 of 8 studies utilized identical grading criteria, demonstrating a lack of standardized application. Most APAs (84%) were macroadenomas, with 52% invasive on magnetic resonance imaging. Nonfunctional PAs were most common (37%), followed by prolactinomas (23%) and Growth Hormone adenomas (21%). Recurrence/progression occurred in 21% of APA patients (follow-up range 37-75 mo). Only 2 of 8 studies reported an association between APA diagnosis and recurrence/progression. CONCLUSION: Based on diagnostic variability and lack of association with clinical outcomes, refinement of criteria for APA was necessary. The WHO update eliminates the ambiguity in APA diagnosis in favor of criteria that emphasize clinical behavior (invasion, recurrence, and resistance to treatment) and molecular markers. Our review supports abandonment of the previous APA designation due to limited prognostic utility.