Experimental animal models for the study of moyamoya disease.

Moyamoya disease is a rare disorder of the cerebrovascular system affecting individuals in a bimodal age distribution and is characterized by progressive vascular stenosis of the bilateral supraclinoid internal carotid arteries with compensatory formation of collateral vessels at the base of the brain. Despite the disease's initial description in the literature in 1957, little progress has been made in the development of medical and surgical therapeutics due to, in no small part, the lack of effective experimental animal models. Currently, there is a poor understanding of the pathophysiological mechanisms behind the development of the moyamoya vasculopathies. Since the description of a genetic association between moyamoya disease, few studies have investigated the impact of genetic manipulation on the development of an animal model for experimentation. To date, no one model recapitulates the precise phenotype of the moyamoya vasculopathies, although development of an appropriate model would allow for an in-depth investigation into the pathological mechanisms underlying the disease. In this review, the authors discuss the immunological, mechanical, and genetic methods used to develop moyamoya experimental models, as well as future perspectives.

Endovascular embolization versus surgical clipping in a single surgeon series of basilar artery aneurysms: a complementary approach in the endovascular era.

BACKGROUND: Currently, most basilar artery aneurysms (BAAs) are treated endovascularly. Surgery remains an appropriate therapy for a subset of all intracranial aneurysms. Whether open microsurgery would be required or utilized, and to what extent, for BAAs treated by a surgeon who performs both endovascular and open procedures has not been reported. METHODS: Retrospective analysis of prospectively maintained, single-surgeon series of BAAs treated with endovascular or open surgery from the first 5 years of practice. RESULTS: Forty-two procedures were performed in 34 patients to treat BAAs-including aneurysms arising from basilar artery apex, trunk, and perforators. Unruptured BAAs accounted for 35/42 cases (83.3%), and the mean aneurysm diameter was 8.4 ± 5.4 mm. Endovascular coiling-including stent-assisted coiling-accounted for 26/42 (61.9%) treatments and led to complete obliteration in 76.9% of cases. Four patients in the endovascular cohort required re-treatment. Surgical clip reconstruction accounted for 16/42 (38.1%) treatments and led to complete obliteration in 88.5% of cases. Good neurologic outcome (mRS ≤ 2) was achieved in 88.5% and 75.0% of patients in endovascular and open surgical cohorts, respectively (p = 0.40). Univariate logistic regression analysis demonstrated that advanced age (OR 1.11[95% CI 1.01-1.23]) or peri-procedural adverse event (OR 85.0 [95% CI 6.5-118.9]), but not treatment modality (OR 0.39[95% CI 0.08-2.04]), was the predictor of poor neurologic outcome. CONCLUSIONS: Complementary implementation of both endovascular and open surgery facilitates individualized treatment planning of BAAs. By leveraging strengths of both techniques, equivalent clinical outcomes and technical proficiency may be achieved with both modalities.

How I do it: superficial temporal artery-middle cerebral artery bypass for flow augmentation and replacement.

BACKGROUND: The superficial temporal artery-middle cerebral artery (STA-MCA) bypass augments blood flow in patients with cerebral ischemia or replaces flow in patients with complex aneurysms or skull base tumors requiring vessel sacrifice. METHOD: We provide a description of the STA-MCA bypass with figures and video to illustrate the procedure. CONCLUSION: The STA-MCA end-to-side anastomosis is a foundational skill for the cerebrovascular surgeon and a building block for more complex bypasses.

Left temporal craniotomy and direct microcatheterization of the middle meningeal artery for treatment of a complex temporal dural arteriovenous fistula.

The preferred method for treating complex dural arteriovenous fistulae of the transverse and sigmoid sinuses is via endovascular, transarterial embolization using liquid embolysate. However, this treatment approach mandates access to distal dural feeding arteries that can be technically challenging by standard endovascular approaches. This video describes a left temporal craniotomy for direct stick microcatheterization of an endovascularly inaccessible distal posterior division of the middle meningeal artery for embolization of a complex left temporal dural arteriovenous fistula. The case was performed in the hybrid operative suite with biplane intraoperative angiography. Technical considerations, operative nuances, and outcomes are reviewed.The video can be found here: https://youtu.be/Dnd4yHgaKcQ.

Diagnosis and treatment of chordoma.

Chordoma is a primary bone cancer arising on the midline from the skull base to the sacrum. Diagnosis is often delayed because of insidious onset and nonspecific symptoms. Chordomas appear histologically low-grade but are highly invasive and often recur locally. Management centers primarily on radical en bloc surgical resection when possible. Radiation therapy using protons and/or photons is often necessary because complete resection is seldom possible due to critical location and invasion of the cancer cells into surrounding structures. No approved medical therapy exists. The high rate of recurrence is reflected by a median survival of 6 to 7 years. This article reviews the clinical management of chordoma and discusses ongoing research in the field.

Treatment of an infundibular aneurysm by posterior communicating origin disconnection, induced flow reversal and flow diversion after attempted surgical clipping: Case report.

Infundibular aneurysms are rare lesions that present unique challenges for both surgical and endovascular approaches. We present a case of a 58-year old man with an incidental 7.5 × 3 mm infundibular aneurysm that incorporated the origin of the posterior communicating artery (PCoA). The fusiform aneurysm incorporated the supraclinoid ICA to the terminus. An attempt at surgical clip reconstruction of the aneurysm to preserve the PCoA was not technically possible due to atherosclerosis at the aneurysm neck. The aneurysm was treated with disconnection of the PCoA origin, induced flow reversal and placement of a flow diverter, after confirmation of adequate collateral flow to the PCoA perforators from the posterior circulation. The case illustrates important concepts in the evaluation and management of infundibular aneurysms, including both reconstructive and deconstructive strategies.

Management strategies of unanticipated intracranial stenosis during mechanical thrombectomy for acute stroke: A survey of academic neurointerventionalists.

BACKGROUND: The optimal approach to the management of intracranial atherosclerotic disease (ICAD) at the time of mechanical thrombectomy (MT) for large vessel occlusion (LVO) remains controversial. The goal of this study is to characterize current practices concerning this challenging clinical situation in a survey of practicing neurointerventionalists. METHODS: An electronic questionnaire was sent to a cross-section of North American academic neurointerventionalists using publicly available contact information and departmental websites. Prior to analysis, responses were anonymized and categorized by region. RESULTS: A total of 136/360 responses were recorded from the U.S. and Canada. The mean number of years of practicing as a neurointerventionalist among the respondents was 10.5 (± 6.2 years). ICAD was perceived as a causative factor during MT for LVO in 5-10% of thrombectomy cases. The most common first-line treatment approach for significant ICAD, assuming a TICI 2b or better reperfusion, was medical therapy (77.9% of respondents), followed by angioplasty + stent placement (8.8% of respondents). There were no significant differences in the first line treatment of ICAD in LVO between geographical regions (p = 0.815). CONCLUSION: The approach to underlying ICAD in LVO varies widely; however, the majority of neurointerventionalists prefer medical therapy with DAPT as a first-line treatment approach. The current survey highlights the need for studies that better define the optimal timing and modality of treatment, along with an evidence-based framework for balancing the risks associated with these treatment approaches.

Arachnoid Granulation Causing Unilateral Pulsatile Tinnitus Treated With Dural Venous Sinus Stenting.

BACKGROUND: Large arachnoid granulations that protrude into dural venous sinuses and partially obstruct outflow are an underappreciated etiology of pulsatile tinnitus (PT). Endovascular dural venous sinus stenting is thought to diminish turbulent venous outflow and may relieve obstruction caused by arachnoid granulations. METHODS: Four patients at two institutions were evaluated for unilateral PT. Magnetic resonance imaging and digital subtraction angiography revealed moderate-to-severe stenoses from large arachnoid granulations within the implicated transverse sinus. All patients underwent venous manometry and endovascular sinus stenting. RESULTS: All patients experienced immediate and complete remission of their PT. Stenoses were relieved by a mean of 93% by Warfarin-Aspirin Symptomatic Intracranial Disease criteria. There were no procedural or periprocedural complications. All patients continued to report complete symptom resolution at a mean of 8-month follow-up. CONCLUSIONS: PT from arachnoid granulations are an underappreciated pathomechanism. Endovascular dural venous sinus stenting is an effective intervention for treating unilateral PT secondary to large arachnoid granulation.

Recurrence of a large intracranial fusiform aneurysm treated with overlapping Pipeline embolization devices: illustrative case.

BACKGROUND: Flow diversion, specifically with the Pipeline embolization device (PED), represents a paradigm shift in the treatment of intracranial aneurysms. Several studies have demonstrated its efficacy and at times superiority to conventional treatment modalities for aneurysms with a fusiform morphology, giant size, or wide neck. However, there may be a nonsignificant risk of recurrence after flow diversion of these historically difficult-to-treat aneurysms, relative to aneurysms with a more favorable morphology and size (i.e., saccular, narrow necked). To date, only three papers in the literature have demonstrated the recurrence of a completely occluded aneurysm on follow-up. OBSERVATIONS: The authors describe a patient with a giant middle cerebral artery fusiform aneurysm treated with multiple telescoping PEDs. On the 3-month follow-up angiogram, there was complete occlusion of the aneurysm. The patient was lost to follow-up and presented 4 years later with a recurrence of the aneurysm between PED segments, requiring retreatment. The patient represented 3 years posttreatment with the need for repeat treatment of the fusiform aneurysm due to separation of the existing PEDs along with stent reconstruction. At the 20-month follow-up after the third treatment, the initial aneurysm target was found to be occluded. LESSONS: This case illustrates the need for long-term follow-up, specifically for patients with giant wide-necked or fusiform aneurysms treated with overlapping PEDs.

Patient-reported outcome measures in cerebrovascular neurosurgery.

OBJECTIVE: The object of this study was to describe the use of patient-reported outcome measures (PROMs) in cerebrovascular neurosurgery and to outline a framework for incorporating them into future cerebrovascular research. METHODS: Following the standardized PRISMA guidelines, the authors performed a search of the PubMed and Embase databases in February 2023 using filters to investigate six specific cerebrovascular pathologies/procedures: subarachnoid hemorrhage (SAH), intracranial hemorrhage, ischemic stroke, arteriovenous malformation, chronic subdural hematoma, and carotid artery stenosis. PROMs in the identified articles were distinguished and classified as generic, symptom specific, or disease specific. RESULTS: A total of 259 studies including 51 PROMs were eligible for inclusion in the review. Most of the PROMs were generic or symptom specific. Only 5 PROMs were disease specific, and all of these pertained to stroke or SAH. CONCLUSIONS: There are only a limited number of disease-specific PROMs available for cerebrovascular pathologies and outcomes. Further validation of existing measures in independent cohorts, expanded incorporation of disease-specific PROMs in prospective trials, and the development of new PROMs specific to cerebrovascular conditions are critical to a better understanding of the impact of cerebrovascular diseases and novel therapies on patient lives.

Endoscopic endonasal compared with microscopic transsphenoidal and open transcranial resection of giant pituitary adenomas.

Giant (>4 cm) pituitary macroadenomas often require surgery to decompress the optic nerves. Compared with traditional open or transsphenoidal microscopic methods, the extended endoscopic endonasal transsphenoidal approach offers the potential for aggressive resection via a minimal access corridor. We conducted a systematic review of the literature to further our understanding of the role of endoscopy in the management of these challenging lesions. MEDLINE search of the modern literature (1995-2010) to identify surgical series for pediatric and adult pituitary adenomas >4 cm in maximal diameter. Patient and tumor characteristics, resection, morbidity and visual outcome were compared by approach. Chi-square and Fisher's exact tests with post-hoc Bonferroni analysis were used for statistical analyses. Sixteen studies (478 patients) were included. Compared with the open cohort, the endoscopic cohort had higher rates of gross total resection (47.2% vs. 9.6%; P < 0.003) and improved visual outcome (91.1% vs. 45.7%; P < 0.003). The microscopic transsphenoidal cohort had lower rate of total resection and worse visual outcomes than the endoscopic group. There were no instances of postoperative CSF leak reported in the endoscopic group. The transcranial group had a higher rate perioperative mortality compared to the transsphenoidal group (P = 0.004). In select cases, the endoscopic endonasal approach is safe and effective for the treatment of giant pituitary adenomas, with the potential for gross total resection and improved visual outcome. CSF leak, which is a major limitation of the endonasal approach, may be avoided using meticulous multi-layer closure and vascularised nasoseptal flaps.

Endoscopic skull base surgery: a comprehensive comparison with open transcranial approaches.

Little data exists comparing endoscopic endonasal approaches to pathology of the anterior skull base with more traditional transcranial and transsphenoidal microscopic approaches. In order to more fully characterize the role of endoscopy in the management of pathology of the anterior cranial base, we conducted a systematic review of case series and case reports documenting surgical and clinical outcomes. We found that for craniopharyngiomas, clival chordomas, esthesioneuroblastomas and giant pituitary adenomas, the endonasal endoscopic approach can result in equivalent or higher rates of gross total resection than open approaches. For meningiomas, however, open transcranial approaches are still able to achieve higher rates of total resection. CSF leak rates are higher for patients undergoing endoscopic surgery for meningiomas and craniopharyngiomas, but not for chordomas, esthesioneuroblastomas or giant pituitary adenomas. In certain patients, the endonasal endoscopic approach may be a safe and effective alternative for the treatment of a wide variety of skull base pathology, particularly those with small midline tumours.

Use of transcarotid artery revascularization for mechanical thrombectomy and treatment of symptomatic high-grade carotid artery stenosis associated with free-floating thrombus: illustrative case.

BACKGROUND: Patients with symptomatic high-grade stenosis of the internal carotid artery (ICA) associated with a free-floating thrombus (FFT) present a significant clinical challenge. In general, for patients with moderate to severe symptomatic ICA stenosis, carotid revascularization is recommended within 2 weeks of symptom onset; however, some physicians suggest that revascularization should be delayed in cases with FFT because some data suggest that early surgery with carotid endarterectomy or carotid stent poses a higher risk for stroke. Likewise, delayed revascularization with anticoagulation may increase risk of recurrent stroke. Few reports on the management of FTT included the use of a transcarotid artery revascularization (TCAR) approach for carotid revascularization with mechanical aspiration thrombectomy. OBSERVATIONS: This report described the use of TCAR for direct mechanical thrombectomy and carotid stent placement for a patient with 80% right ICA stenosis along with a large FFT extending into the bulb and the external carotid artery. LESSONS: The TCAR approach for mechanical thrombectomy and carotid stenting is a safe alternative for early revascularization with low periprocedural stroke risks.

PulseRider-assisted embolization of a distal anterior cerebral artery bifurcation aneurysm: illustrative case.

BACKGROUND: PulseRider is an endovascular device that can be a useful adjunctive device for wide-necked bifurcation aneurysms. However, its use in distal vessels such as the anterior cerebral artery (ACA) has not been widely reported. OBSERVATIONS: The authors reported the case of a 75-year-old woman who underwent coiling of a 6.9-mm distal ACA aneurysm with PulseRider assistance. Using a partially intraaneurysmal deployment technique, the wide-necked aneurysm was successfully embolized, resulting in Raymond-Roy class II occlusion without intra- or periprocedural complications. LESSONS: This case illustrates a novel approach to treatment for wide-necked distal ACA aneurysms, which can be challenging to treat via traditional endovascular means. PulseRider can be safely used to treat distal ACA aneurysms with minimal residual aneurysm.

The Lateral Supraorbital Craniotomy Approach for Anterior Circulation Aneurysms: A Modern Surgical Case Series in the Endovascular Era.

BACKGROUND: The lateral supraorbital (LSO) approach is a minimally invasive modification of the standard pterional approach to anterior circulation aneurysms. This study aimed to describe a dual-trained cerebrovascular neurosurgeon's first 18-month experience with the LSO approach, including decision-making criteria and lessons learned. METHODS: This retrospective case series analyzed 50 consecutive patients treated with LSO craniotomy for aneurysm clipping by a single surgeon. Aneurysms were separated into 3 categories by location: internal carotid artery, anterior communicating artery, and middle cerebral artery. Surgical characteristics were evaluated for differences by location and rupture status. RESULTS: Aneurysm clipping via LSO was performed on 57 aneurysms in 50 patients. Fixed retraction was employed less often in patients with internal carotid artery aneurysms than in patients with anterior communicating artery, middle cerebral artery, or multiple aneurysms (10% vs. 68.2%, 45.5%, and 42.9, P = 0.02). Of patients, 26 (52%) presented with subarachnoid hemorrhage; the majority of patients (92.3%) had Hunt and Hess grade I-III. No differences were noted in intraoperative rupture rates, fixed retractor use, operative duration, or estimated blood loss by rupture status. Adverse events included permanent frontalis nerve palsy in 1 patient (2%), temporalis atrophy in 1 patient, and transient aphasia in 1 patient. No postoperative hematomas or strokes were observed. CONCLUSIONS: The LSO approach can safely and effectively treat anterior circulation aneurysms and should be considered a viable minimally invasive option for aneurysm clipping. Further studies comparing the LSO approach with other cranial approaches are needed.

Socioeconomic factors associated with pediatric moyamoya disease hospitalizations: a nationwide cross-sectional study.

OBJECTIVE: Healthcare disparities are widely described in adults, but barriers affecting access to care for pediatric patients with moyamoya disease (MMD) are unknown. Understanding socioeconomic factors impacting hospital access and outcomes is necessary to address pediatric healthcare disparities. METHODS: In this cross-sectional observational study, the Kids' Inpatient Database was used to identify patients admitted with a primary diagnosis of MMD from 2003 to 2016. Patients ≤ 18 years with a primary diagnosis of MMD based on International Classification of Diseases (ICD) codes were included. Hospital admissions were queried for use of cerebral revascularization based on ICD procedure codes. RESULTS: Query of the KID yielded 1449 MMD hospitalizations. After multivariable regression, Hispanic ethnicity (OR 0.52 [95% CI 0.33-0.81], p = 0.004) was associated with lack of surgical revascularization. Private insurance (OR 1.56 [95% CI 1.15-2.13], p = 0.004), admissions at medium- and high-volume centers (OR 2.01 [95% CI 1.42-2.83], p < 0.001 and OR 2.84 [95% CI 1.95-4.14], p < 0.001, respectively), and elective hospitalization (OR 3.37 [95% CI 2.46-4.64], p < 0.001) were positively associated with revascularization. Compared with Caucasian race, Hispanic ethnicity was associated with increased mean (± SEM) length of stay by 2.01 ± 0.70 days (p = 0.004) and increased hospital charges by $24,333.61 ± $7918.20 (p = 0.002), despite the decreased utilization of surgical revascularization. Private insurance was associated with elective admission (OR 1.50 [95% CI 1.10-2.05], p = 0.01) and admission to high-volume centers (OR 1.90 [95% CI 1.26-2.88], p = 0.002). African American race was associated with the development of in-hospital complications (OR 2.52 [95% CI 1.38-4.59], p = 0.003). CONCLUSIONS: Among pediatric MMD hospitalizations, multiple socioeconomic factors were associated with access to care, whether surgical treatment is provided, and whether in-hospital complications occur. These results suggest that socioeconomic factors are important drivers of healthcare disparities in children with MMD and warrant further study.

Middle meningeal artery embolization for chronic subdural hematoma in cancer patients with refractory thrombocytopenia.

OBJECTIVE: Surgical evacuation of chronic subdural hematoma (SDH) in cancer patients is often contraindicated owing to refractory thrombocytopenia. Middle meningeal artery embolization (MMAE) recently emerged as a potential alternative to surgical evacuation for patients with chronic SDH. The goal of this study was to evaluate the safety and efficacy of MMAE for chronic SDH in cancer patients with refractory thrombocytopenia. METHODS: A multiinstitutional registry was reviewed for clinical and radiographic outcomes of cancer patients with transfusion-refractory thrombocytopenia and baseline platelet count < 75 K/µl, who underwent MMAE for chronic SDH. RESULTS: MMAE was performed on a total of 31 SDHs in 22 patients, with a mean ± SD (range) platelet count of 42.1 ± 18.3 (9-74) K/µl. At the longest follow-up, 24 SDHs (77%) had reduced in size, with 15 (48%) showing > 50% reduction. Two patients required surgical evacuation after MMAE. There was only 1 procedural complication; however, 16 patients (73%) ultimately died of cancer-related complications. Median survival was significantly longer in the 16 patients with improved SDH than the 6 patients with worsened SDH after MMAE (185 vs 24 days, p = 0.029). Length of procedure, technical success rate, SDH size reduction, and complication rate were not significantly differ between patients who underwent transfemoral and transradial approaches. CONCLUSIONS: Transfemoral or transradial MMAE is a potential therapeutic option for thrombocytopenic cancer patients with SDH. However, treatment benefit may be marginal for patients with high disease burden and limited life expectancy. A prospective trial is warranted to address these questions.

Herpes simplex encephalitis following spinal ependymoma resection: case report and literature review.

Herpes simplex encephalitis (HSE) is a rare complication of neurosurgical procedures but must be considered in early deterioration of the postoperative patient. This is the first report of HSE following spinal cord tumor resection. A 65-year-old woman had C2-C5 laminectomy for subtotal resection of intramedullary ependymoma. Six days postoperatively she developed fever, vomiting and rapid decline in mental status. Brain MRI revealed enhancement of left insular cortex. Polymerase chain reaction on cerebrospinal fluid (CSF) identified herpes simplex virus type 1 (HSV-1) as the causal agent. Twenty-one days of acyclovir led to improvement. Three subsequent admissions to neurological intensive care unit were required for deterioration in mental status, including pneumonia, hydrocephalus and deep vein thromboses. Ventriculoperitoneal shunt (VPS), tracheotomy, percutaneous intravenous central catheter (PICC) line and percutaneous endoscopic gastrostomy (PEG) were placed. She was discharged to skilled nursing home care. Acyclovir is effective therapy against HSV, though outcomes may be poor even in optimally treated cases. Empiric treatment must be started even in the absence of serologic evidence of HSV infection if suspicion for HSE is high.

The management of incidental low-grade gliomas using magnetic resonance imaging: systematic review and optimal treatment paradigm.

OBJECT: The discovery of incidental low-grade gliomas (LGGs) on MR imaging is rare, and currently there is no existing protocol for management of these lesions. Various studies have approached the dilemma of managing patients with incidental LGGs. While some advocate surgery and radiotherapy, others reserve surgery until there is radiological evidence of growth. For neurosurgeons and radiologists, determining the course of action after routine brain imaging poses not only a medical but also an ethical dilemma. The authors conducted a systematic review of case reports and case series in hopes of enhancing the current understanding of the management options for these rare lesions. METHODS: A PubMed search was performed to include all relevant MR imaging studies in which management of suspected incidental LGG was reported. Comparisons were made between the surgical treatment arm and the active surveillance arm in terms of outcome, mode of discovery, reasons for treatment, and histology. RESULTS: Nine studies with 72 patients were included in this study (56 in the surgical arm and 16 in the active surveillance arm). Within the surgical arm, 49% remained deficit free after treatment, 25% showed evidence of tumor progression, 13% underwent a second treatment, and 7% died. The active surveillance group resulted in no unanticipated adverse events, with serial imaging revealing no tumor growth in all cases. Lesion regression was reported in 31% of this group. The surgical arm's mortality rate was 7% compared with 0% in the active surveillance arm. CONCLUSIONS: Treatment decisions for incidental LGG should be individualized based on presenting symptoms and radiological evidence of growth. The asymptomatic patient may be monitored safely with serial MR imaging and occasionally PET scanning before treatment is initiated. In patients presenting with nonspecific symptoms or concurrent symptomatic lesions, treatment may be initiated earlier to reduce potential morbidity. All treatment decisions must be tempered by patient factors and expectations of anticipated benefit.