Low-profile mechanical thrombectomy devices for large vessel occlusion in pediatric ischemic stroke.

BACKGROUND: Mechanical thrombectomy (MT) is a well-established treatment modality for large vessel occlusion (LVO) in adults, but there are limited data in the pediatric population. The rarity of the condition makes prospective trial design difficult, and therefore evaluation of MT devices and outcomes is sparse. In pediatric LVO cases, some newer devices may be appropriate for use in revascularization procedures. Furthermore, illustrative specific device-access combinations have rarely been presented in young patients under the age of 5 years. METHODS: This was a single institution experience in intracranial MT procedures for pediatric acute ischemic stroke (AIS). A given procedure was included if there was an attempt at MT between 2015 and 2023. RESULTS: Sixteen endovascular procedures were performed in 15 patients with AIS, with a mean age of 7.4 years; eight patients were <5 years of age. MT was attempted in 15/16 (93.7%) procedures, including 11 (73.3%) in the anterior circulation and four (26.7%) in the posterior circulation; one case recanalized after medical treatment. The most common MT techniques were combined aspiration with a stentriever (n=10, 66.7%) and aspiration alone (n=3, 20%). Traditional stentrievers (4-6 mm) were used in seven cases and low-profile stentrievers in four cases. Improved reperfusion was achieved in all low-profile stentriever cases. Reperfusion of modified Thrombolysis in Cerebral Infaction ≥2b was obtained in 73.3% of cases, including 72.7% of those in which a stentriever was used. CONCLUSIONS: Low-profile thrombectomy devices may achieve similar revascularization results to other approaches in challenging clinical scenarios observed in younger children.

Infantile dural arteriovenous fistula: a reconsideration.

BACKGROUND: Infantile-type dural arteriovenous shunts (IDAVS) are rare and heterogeneous vascular lesions, complicating their classification and management. The current tripartite classification of pediatric dural arteriovenous shunts (DAVS) into dural sinus malformation, infantile-type, and adult-type, does not stand up to scrutiny, given the variable presentations of the latter two types in children. We estimate the prevalence of IDAVS and evaluate the long term outcomes after endovascular treatment (EVT). METHODS: A retrospective review of a pediatric cerebrovascular database between 2006 and 2023 was conducted. Clinical and radiographic data were analyzed to evaluate the presentation and long term outcomes of IDAVS. RESULTS: IDAVS were identified in 8 (0.5%) of 1463 patients, with mean age at diagnosis of 34.7 months; male infants comprised 62.5%. The most common clinical presentations included macrocephaly (37.5%), seizures (25%), and dilated scalp veins (25%). EVT was performed in 87.5% of cases, averaging 5.8 procedures per patient. Radiographic obliteration was observed in 28.6%. Good clinical outcomes (modified Rankin Scale score of ≤2) were achieved in 85.7%. Our findings showcased discrepancies and limitations in the current classification of pediatric DAVS, prompting a re-evaluation. CONCLUSION: IDAVS accounted for a small proportion of pediatric cerebrovascular pathologies, with markedly heterogeneous presentations. Stepwise selective embolization was associated with favorable outcomes, and is recommended over an aggressive approach with the goal of complete angiographic obliteration. Our proposed revised classification system bifurcates pediatric DAVS into dural sinus malformations and all other DAVS that are manifest in children, thereby enhancing diagnostic clarity and therapeutic approaches.

Lower Cranial Nerve Schwannomas: Cohort Study and Systematic Review.

BACKGROUND AND OBJECTIVES: Schwannomas originating from the lower cranial nerves (LCNS) are rare and pose a significant surgical challenge. Resection is the mainstay treatment; however, risk of treatment morbidity is considerable, and the available literature regarding differential treatment outcomes in this vulnerable population is sparse. METHODS: A single-institution cohort study and systematic literature review of LCNS were performed. RESULTS: Fifty-eight patients were included: 34 underwent surgical resection and 24 underwent stereotactic radiosurgery (SRS). The median age at diagnosis was 48 years (range 17-74). Presenting symptoms were dysphagia (63%), dysarthria/hypophonia (47%), imbalance (33%), and hearing loss/tinnitus (30%). Tumor size was associated with surgical resection, as compared with initial SRS (4.1 cm vs 1.5 cm, P = .0001). Gross total resection was obtained in 52%, with tumor remnants predominantly localized to the jugular foramen (62%). Post-treatment worsening of symptoms occurred in 68% of surgical and 29% of SRS patients ( P = .003). Postoperative symptoms were mostly commonly hypophonia/hoarseness (63%) and dysphagia (59%). Seven patients (29%) had new neurological issues after SRS treatment, but symptoms were overall milder. The median follow-up was 60 months (range 12-252); 98% demonstrated meaningful clinical improvement. Eighteen surgical patients (53%) underwent adjuvant radiation at a median of 5 months after resection (range 2-32). At follow-up, tumor control was 97% in the surgical cohort and 96% among SRS patients. CONCLUSION: Although LCNS resection is potentially morbid, most postoperative deficits are transient, and patients achieve excellent tumor control-particularly when paired with adjuvant SRS. For minimally symptomatic patients undergoing surgical intervention, we advise maximally safe resection with intracapsular dissection to preserve nerve integrity where possible. For residual or as a primary treatment modality, SRS is associated with low morbidity and high rates of long-term tumor control.

Revascularization of a Complex Case of Vertebrobasilar Insufficiency with Occipital Artery-Anterior Inferior Cerebellar Artery Bypass.

Atherosclerotic disease is the most common etiology in causing posterior circulation strokes and can be found within the intracranial vertebrobasilar system.1 Endovascular and surgical approaches to treat this disease have been defined with both advantages and disadvantages.2,3 We present a case of surgical revascularization of a complex case of vertebrobasilar insufficiency with occipital artery (OA)-anterior inferior cerebellar artery (AICA) bypass (Video 1). A 56-year-old gentleman presented with severe, disabling, and progressive symptoms of vertebrobasilar insufficiency refractory to maximal medical management. Symptom onset was 2 years prior after suffering a left lateral medullary stroke due to a left vertebral artery (VA) occlusion. Angiography showed left VA occlusion after the origin of the posterior inferior cerebellar artery (PICA) and a hypoplastic right VA mostly ending in a PICA with a small and critically stenosed branch to the basilar artery. An initial attempt to revascularize the chronic totally occluded left VA using a combination of anterograde and retrograde (via PComm) approaches was unsuccessful. The decision was made to proceed with an OA-AICA bypass. The end-to-side anastomosis was conducted in the right cerebellopontine cistern and was uncomplicated. Postoperative angiography demonstrated a patent bypass with brisk OA-to-AICA flow with retrograde filling of the basilar artery and its branches. No perioperative strokes. The patient remained free of recurrent symptoms of vertebrobasilar insufficiency at 6 months' follow-up.

Transvenous embolization of a cerebrospinal fluid-venous fistula for the treatment of spontaneous intracranial hypotension.

Cerebrospinal fluid-venous fistula is an increasingly recognized cause of spontaneous intracranial hypotension.1 The site of the leak is between the dural sleeve around a spinal nerve root and the surrounding foraminal veins. In appropriately investigated patients, transvenous embolization of the draining foraminal and paraspinal veins has been shown to be an effective way of treating the disease, with low periprocedural morbidity, improvement in symptoms and radiological appearances.2 Video 1 shows the technique employed in a typical case using Onyx (Medtronic, Minnesota, USA) to embolize a CSF-venous fistula at the right T10 neural foramen. neurintsurg;14/9/948/V1F1V1Video 1Video showing the technique for trans-venous embolization of a right T10 CSF-venous fistula for the treatment of spontaneous intracranial hypotension. The first section covers patient selection and work up, before then focusing on the technical aspects of navigating through the azygos system to the target foramen and completely occluding the pathway for CSF outflow.

Repeat Catheter Angiography in Patients with Aneurysmal-Pattern Angiographically Negative Subarachnoid Hemorrhage.

BACKGROUND: A significant proportion of patients with subarachnoid hemorrhage have a normal cerebral angiogram. Patients with angiographically negative subarachnoid hemorrhage (anSAH) with either perimesencephalic- (panSAH) or aneurysmal-pattern hemorrhage (aanSAH, also known as diffuse anSAH) have an excellent prognosis, but only if underlying vascular abnormalities are completely excluded. The rate of occult aneurysms in patients with aanSAH varies widely across studies. The purpose of this study was to quantify the value of repeat DSA in these patients. METHODS: We reviewed the records of all patients initially diagnosed with aanSAH after a screening DSA at a single tertiary neurovascular referral center from January 2006-April 2018. Patients with panSAH and traumatic SAH were excluded. We also performed a systematic review and meta-analysis of positive second DSAs in previously published case series of patients with aanSAH who underwent two serial DSAs. For meta-analysis, PubMed Central, MEDLINE and Cochrane Library databases were searched for pertinent studies up to November 2019. The rate of aneurysm detection on repeat angiography was extracted from each study. Pooled rates for positive second angiogram were calculated as untransformed proportions in a binary random-effects model meta-analysis. Inter-study heterogeneity was calculated using an I2 statistic. RESULTS: Three of 27 patients (11.1%) with aanSAH and at least two DSAs were subsequently found to have a cerebral aneurysm in our institutional dataset. Twenty-six studies in our systematic review met inclusion criteria, and the pooled rate of positive second angiogram was 10.4% (95% CI 7.3%-13.5%, P < 0.001). Substantial inter-study heterogeneity was observed in the meta-analysis (I2 = 61.7%, P < 0.001). CONCLUSIONS: One in 10 patients with aanSAH has an occult ruptured aneurysm. A second-look DSA should be strongly considered in these cases.

Venous Causes of Pulsatile Tinnitus: Clinical Presentation, Clinical and Radiographic Evaluation, Pathogenesis, and Endovascular Treatments: A Literature Review.

Tinnitus is an abnormal perception of a sound without external stimulation. Venous pulsatile tinnitus (VPT) is a specific form of tinnitus characterized by an objective and often subjective bruit that occurs as a result of localized venous abnormalities. Clinical evaluation relies on sound quality, duration, and precipitating factors. Idiopathic intracranial hypertension (IIH) and transverse sinus stenosis (TSS) are among the most common causes of VPT. Other causes include sigmoid sinus wall abnormalities (SSWAs), jugular vein anomalies (JVAs), and emissary veins anomalies. These anomalies can be detected on magnetic resonance imaging, magnetic resonance angiography/magnetic resonance venography, and high-resolution temporal bone computed tomography. The pathogenesis behind the VPT includes turbulent blood flow as a result of luminal stenosis or abnormal dilation, amplification of internal sound due to temporal bone defects, and abnormal position of the venous sinus system structures adjacent to the bony structures of the auditive apparatus. Based on these theories, different interventional treatment modalities can be applied to treat the underlying causes. Endovascular treatments have shown high efficacy and safety among those treatments which include stenting of the lateral sinus stenosis in IIH and TSS, coiling of the SSWA and JVA, and embolization of emissary veins anomalies. Further studies are needed to understand the natural history of these anomalies and the efficacy of treatments of VPT, which-unlike other types of tinnitus-can be cured with proper treatment.

A Comprehensive Study of Spindle Cell Oncocytoma of the Pituitary Gland: Series of 6 Cases and Meta-Analysis of 85 Cases.

OBJECTIVE: To discuss optimal treatment strategy for spindle cell oncocytoma (SCO) of the pituitary gland. METHODS: Institutional cases were retrospectively reviewed. A systematic literature search and subsequent quantitative synthesis were performed for further analysis. The detailed features were summarized and the tumor control rate (TCR) was calculated. RESULTS: Eighty-five patients (6 institutional and 79 literature) were included. The annual incidence was approximately 0.01-0.03/100,000. The mean age was 56 years. Vision loss was present in 60%. Seventy-three percent showed hormonal abnormalities. On magnetic resonance imaging, tumor was avidly enhancing, and the normal gland was commonly displaced anterosuperiorly. Evidence of hypervascularity was seen in 77%. Gross total resection (GTR) was achieved in only 24% because of its hypervascular, fibrous, and adhesive nature. The mean postoperative follow-up was 3.3 years for institutional cases and 2.3 years for the integrated cohort. The TCR was significantly better after GTR (5-year TCR, 75%; P = 0.012) and marginally better after non-GTR + upfront radiotherapy (5-year TCR, 76%; P = 0.103) than after non-GTR alone (5-year TCR, 24%). The TCRs for those with low Ki-67 index (≤5%) were marginally better than those with higher Ki-67 index (5-year rate, 57% vs. 23%; P = 0.110). CONCLUSIONS: Frequent endocrine-related symptoms, hypervascular signs, and anterosuperior displacement of the gland support preoperative diagnosis of SCO. GTR seems to have better long-term tumor control, whereas the fibrous, hypervascular, and adhesive nature of SCO makes it difficult to achieve GTR. In patients with non-GTR, radiotherapy may help decrease tumor progression.

Long-term clinical and mid-term radiographic follow-up of pure arterial malformations.

BACKGROUND: Pure arterial malformations (PAMs) are rare vascular lesions characterized by dilated, tortuous arterial loops without venous shunting. The natural history of PAMs remains unclear. We report the mid-term radiological and longer-term clinical outcomes of the largest series of patients with PAM. METHODS: Retrospective review at a tertiary academic referral center for patients with a PAM. Follow-up clinical and radiological data were collected and analyzed for clinical symptoms and radiographic changes. RESULTS: Twenty-five patients met the inclusion criteria. The mean age at presentation was 30.9 ± 14 years. Nineteen (76%) patients were female, and six (23.1%) were male. Eleven (44%) patients had ≥1 symptom at presentation, of which only 3 (12%) could be linked to PAM. The most common symptom was headache (n=8). PAMs involved a single vessel in 16 (64%) cases and ≥2 vessels in 9 cases (36%). Fifteen (60%) lesions were isolated to the anterior circulation compared to 6 (24%) in the posterior circulation. The most frequently involved vessel was the supraclinoid internal cerebral artery (36%). An associated saccular aneurysm was present in 32% of patients. Ten lesions were partially calcified. The mean radiographic and clinical follow-up was 21.9 ± 26.5 months and 44.6 ± 34.8 months, respectively. None of the patients developed new symptoms related to their lesion or radiographic progression over the duration of follow-up. CONCLUSIONS: PAMs are found most frequently in young, asymptomatic females. PAMs have a benign natural history and are best managed conservatively with serial imaging.

Carotid artery revascularization: endarterectomy versus endovascular therapy.

Carotid endarterectomy is considered the gold standard for primary and secondary stroke prevention in patients with asymptomatic and symptomatic carotid artery stenosis. The role of CEA has been defined by multiple randomized multicenter trials and CEA is the most studied surgical procedure. In recent years, with advances in endovascular techniques, carotid angioplasty, and stenting (CAS) has been proposed as an alternative to CEA especially in high-risk patients. In this article, we review some of the most important trials on the invasive treatment of carotid artery stenosis and summarized the most recent treatment recommendations based on current evidence. The data overwhelmingly supports revascularization of patients with symptomatic stenosis between 70-90%, with a clear preference for CEA over CAS to be done within 14 days of symptom onset is possible. However, CAS is an acceptable alternative to CEA in certain symptomatic patients such as those with severe medical comorbidities, high riding plaques, contralateral occlusion, restenosis after prior CEA, and radiation-induced stenosis. Treatment of asymptomatic patients remains controversial because of advanced of modern medical therapy and large trials are underway to define the role of invasive revascularization in these patients.

Safety of immediate use of nonsteroidal antiinflammatory drugs after pediatric craniotomy for tumor.

OBJECTIVE: Postoperative pain can limit the recovery of children undergoing craniotomy for tumor resection, and pain management is highly variable between institutions and practitioners. Nonsteroidal antiinflammatory drugs (NSAIDs) are effective in treating postoperative pain following craniotomy, but their use has been limited by concerns about postoperative hemorrhage. The risk of postoperative hemorrhage is not insignificant in patients undergoing craniotomy for tumor resection. No study has specifically addressed the safety of NSAIDs in the immediate postoperative setting following craniotomy for tumor resection in pediatric patients. METHODS: The authors performed a retrospective cohort study in patients younger than 18 years of age who underwent craniotomy for tumor resection at a single tertiary referral center between 2009 and 2019. The study outcomes were 1) postoperative hemorrhage requiring return to the operating room for decompression, evacuation, or CSF diversion for hemorrhage-associated hydrocephalus; and 2) more-than-minimal hemorrhage on routine postoperative imaging. Patients receiving any NSAID in the hospital formulary on the same day as surgery (postoperative day zero [POD0]) were designated as such. RESULTS: Two hundred seventy-six children underwent 308 craniotomies for tumor resection over the study period. One hundred fifty-four patients (50.0%) received at least one dose of an NSAID on POD0. Six patients (1.9%) required a return to the operating room for a hemorrhagic complication, including 3 who received an NSAID on POD0 (OR 1.00, 95% CI 0.20-5.03). Seventeen patients (6.3% of patients imaged) had more-than-minimal hemorrhage on routine postoperative imaging, 9 of whom received an NSAID on POD0 (OR 1.08, 95% CI 0.40-2.89). CONCLUSIONS: Use of NSAIDs on POD0 was not associated with either an increased risk of hemorrhage requiring a return to the operating room or asymptomatic hemorrhage on routine postoperative imaging. The overall incidence of clinically significant postoperative intracranial hemorrhage is low. These data support the use of NSAIDs as a safe measure for pain control in the postoperative setting for children undergoing craniotomy for tumor resection.

Extraordinary case presentations in pediatric pituitary adenoma: report of 6 cases.

Pediatric pituitary adenomas (PPAs) are rare neoplasms with a propensity for unusual presentations and an aggressive clinical course. Here, the authors describe 6 highly atypical PPAs to highlight this tendency and discuss unexpected management challenges.A 14-year-old girl presented with acute hemiparesis and aphasia. MRI revealed a pituitary macroadenoma causing internal carotid artery invasion/obliteration without acute apoplexy, which was treated via emergent transsphenoidal resection (TSR). Another 14-year-old girl developed precocious galactorrhea due to macroprolactinoma, which was medically managed. Several years later, she re-presented with acute, severe, bitemporal hemianopia during her third trimester of pregnancy, requiring emergent induction of labor followed by TSR. A 13-year-old boy was incidentally diagnosed with a prolactinoma after routine orthodontic radiographs captured a subtly abnormal sella. An 18-year-old male self-diagnosed pituitary gigantism through a school report on pituitary disease. A 17-year-old boy was diagnosed with Cushing disease by his basketball coach, a former endocrinologist. A 12-year-old girl with growth arrest and weight gain was diagnosed with Cushing disease, which was initially treated via TSR but subsequently recurred and ultimately required 12 operations, 5 radiation treatments involving 3 modalities, bilateral adrenalectomy, and chemotherapy. Despite these efforts, she ultimately died from pituitary carcinoma.

Pediatric ganglioglioma of the brainstem and cervicomedullary junction: a retrospective cohort study.

OBJECTIVE: Ganglioglioma is a low-grade central nervous system neoplasm with a pediatric predominance, accounting for 10% of all brain tumors in children. Gangliogliomas of the cervicomedullary junction (GGCMJs) and brainstem (GGBSs) present a host of management challenges, including a significant risk of surgical morbidity. At present, understanding of the prognostic factors-including BRAF V600E status-is incomplete. Here, the authors report a single-institution GGCMJ and GGBS experience and review the pertinent literature. METHODS: A prospectively maintained neurosurgical database at a large tertiary care academic referral center was retrospectively queried for cases of GGCMJ pathologically confirmed in the period from 1995 to 2015; appropriate cases were defined by diagnosis codes and keywords. Secondary supplemental chart review was conducted to confirm or capture relevant data. The primary study outcome was treatment failure as defined by evidence of radiographic recurrence or progression and/or clinical or functional decline. A review of the literature was conducted as well. RESULTS: Five neurosurgically managed GGBS patients were identified, and the neoplasms in 4 were classified as GGCMJ. All 5 patients were younger than 18 years old (median 15 years, range 4-16 years) and 3 (60%) were female. One patient underwent gross-total resection, 2 underwent aggressive subtotal resection (STR), and 2 underwent stereotactic biopsy only. All patients who had undergone STR or biopsy required repeat resection for tumor control or progression. Progressive disease was treated with radiotherapy in 2 patients, chemotherapy in 2, and chemoradiotherapy alone in 1. Immunostaining for BRAF V600E was positive in 3 patients (60%). All 5 patients experienced at least one major complication, including wound infection, foot drop, hemiparesis, quadriparesis, cranial neuropathy, C2-3 subluxation, syringomyelia, hydrocephalus, aspiration, and coma. Overall mortality was 20%, with 1 death observed over 11 years of follow-up. CONCLUSIONS: GGBS and GGCMJ are rare, benign posterior fossa tumors that carry significant perioperative morbidity. Contemporary management strategies are heterogeneous and include combinations of resection, radiotherapy, and chemotherapy. The BRAF V600E mutation is frequently observed in GGBS and GGCMJ and appears to have both prognostic and therapeutic significance with targeted biological agents.

Beyond the Learning Curve: Comparison of Microscopic and Endoscopic Incidences of Internal Carotid Injury in a Series of Highly Experienced Operators.

BACKGROUND: As the endoscopic endonasal approach (EEA) has gained popularity as an alternative to microsurgery (MS) for transsphenoidal resection (TSR), numerous studies have attempted to assess the differential risk of internal carotid artery (ICA) injury between the techniques, yet results have been equivocal and contradictory. The aim of this study was to evaluate ICA injury in MS versus EEA among highly experienced neurosurgeons. METHODS: We performed a systematic literature review of publications from 2002-2017 reporting ICA injury outcomes in ≥250 cases using MS or EEA. RESULTS: Seventeen series reporting 11,149 patients were included: 3 MS series, 13 EEA series, and 1 series with adequate samples for each. ICA injury incidences were 0.0%-1.6% in cohorts of 275-3000. MS series documented 5 ICA injuries in 2672 operations, for an overall incidence of 0.2% (range, 0.0%-0.4%), and EEA series reported 30 ICA injuries in 8477 operations, for a 0.4% injury rate (range, 0.0%-1.6%); the difference was nonsignificant (P = 0.25). Increased operative experience was associated with decreased incidence of ICA injury, a finding preserved in the overall study cohort and within discretely examined MS and EEA subgroups (overall r2 = 0.08, MS r2 = 0.23, EEA r2 = 0.07). CONCLUSIONS: ICA injury is the most serious complication of TSR of pituitary neoplasms. Operator inexperience may be a more important risk factor than choice of surgical technique, given the comparably low rates of injury obtained by highly experienced surgeons independent of technique. This emphasizes the need for consolidated care in pituitary centers of excellence, improvement of high-fidelity simulators, and skull base mentorship between senior and junior staff.

Single-fraction stereotactic radiosurgery for spindle cell oncocytoma: preliminary experience and systematic review of the literature.

PURPOSE: Spindle cell oncocytoma (SCO) is a rare benign pituitary tumor. No patient series regarding stereotactic radiosurgery (SRS) for SCO has been published. We report the clinical outcomes of SCO treated with single-fraction SRS, as well as a systematic review of the literature. METHODS: Retrospective cohort series and systematic literature review. RESULTS: Five patients (four male, one female) having single-fraction SRS for persistent or recurrent SCO between 2002 and 2018. Median age was 56 (range 54-79) years. Pre-SRS treatments included transsphenoidal resection (TSR) (n = 3), multiple TSR (n = 1), and TSR, radiotherapy, and craniotomy (n = 1). Median target volume was 4.7 (range 1.8-8.4) cm3, with a median tumor margin dose of 17 (range 14-20) Gy. Median follow-up was 24 (range 10-69) months. All radiation-naïve patients achieved tumor control after SRS; tumor progression was noted 24 months after SRS in one patient who failed prior radiotherapy. No radiation-induced complications were observed after SRS. Systematic literature review of 43 cases in addition to the five cases presented here showed that tumor progression/recurrence was more frequent after STR compared to GTR (P < 0.001). Ten previous cases of radiotherapy for SCO have been reported, but most did not detail radiation volumes, doses, or outcomes. CONCLUSIONS: SCO are uncommon sellar lesions with a propensity for progression or recurrence. Based on the clinically aggressive course of these tumors, adjuvant SRS after STR or at the time of tumor recurrence should be considered. Further case accumulation and follow-up is required to better understand the long-term treatment outcomes after single-fraction SRS for these rare tumors.

Skull Base Enchondroma and Chondrosarcoma in Ollier Disease and Maffucci Syndrome.

BACKGROUND: Maffucci syndrome (MS) and Ollier disease (OD) are rare diseases characterized by multiple benign enchondromas. The incidence of skull base (SB) enchondromas and the risk of malignant transformation remain unknown. Most SB lesions are asymptomatic, and surgical resection carries significant morbidity. Observation may be a reasonable strategy. We report our experience with observation of probable SB enchondromas in MS/OD patients. METHODS: Retrospective review of OD/MS patients with cranial imaging between 1995 and 2018 at 1 institution. RESULTS: 14 patients were included: 3 with MS (21.4%) and 11 with OD (78.6%). The median age was 28 years (range, 11-74 years) and 57.1% were female. Extracranial chondrosarcoma was reported in 3 (21.4%) patients. Seven (50%) patients with SB enchondroma or chondrosarcoma were identified on initial imaging. In patients with SB lesions, the indications for imaging were headache (n=3), seizure (n=1), and diplopia (n=1); 2 cases were incidental findings. The most commonly involved structures were petroclival fissure (86%) and clivus (71%). Treatment included observation (6/7) and resection (1/7). Follow-up imaging was available for all SB lesions, with a mean interval of 50.7 months (range, 5-225 months) and was negative for progression in all patients. CONCLUSIONS: Primary SB lesions in OD/MS patients frequently present in the petroclival junction. Cranial screening and close observation should be considered in MS/OD patients, given the increased risk of intra-axial intracranial tumors, de novo chondrosarcomas, or malignant degeneration of previously known lesions. In asymptomatic patients, observation appears to be a safe strategy in this cohort. Further case accumulation and follow-up are required to better understand the long-term outcomes.

Spontaneous Regression of a Retroodontoid Transverse Ligament Cyst: A Case Report.

BACKGROUND AND IMPORTANCE: Transverse ligament cysts (TLC) are rare, surgically complex lesions arising posterior to the odontoid process of C2. Direct compression of the cervicomedullary junction is a devastating consequence of untreated lesions. We report the first case of spontaneous TLC regression without surgical intervention. CLINICAL PRESENTATION: A 75-yr-old woman presented to an outside hospital with acute episodes of left face and upper extremity numbness. Magnetic resonance imaging (MRI) demonstrated a well-circumscribed 5.8 mm cystic mass at the atlantoaxial junction, posterior to the odontoid process, most consistent with a TLC. She presented to our institution 1 yr later with symptoms of progressive occipital neuralgia seeking surgical treatment. No evidence of cervical myelopathy was identified on clinical examination. Repeat MRI showed near doubling of the cyst, with no brainstem edema. The patient elected for surveillance of the cyst with a transforaminal steroid injection at C1-C2 for her occipital neuralgia. One year later, symptoms of occipital neuralgia had resolved and she remained neurologically intact; MRI of the cervical spine showed near complete involution of the cyst. CONCLUSION: Symptomatic TLCs are often managed with surgical decompression and, in selected cases, fusion with good functional outcome. However, these interventions carry high risk of postoperative morbidity, particularly in the elderly. Conservative surveillance is rarely reported as a viable option. We present the first case of spontaneous TLC regression in the absence surgery or neck bracing. In select patients without acute myelopathy, clinical and radiographic surveillance may be considered for the management of TLCs.

Nervus intermedius and the surgical management of geniculate neuralgia.

OBJECTIVE: Geniculate neuralgia (GN) is an uncommon craniofacial pain syndrome attributable to nervus intermedius (NI) dysfunction. Diagnosis and treatment can be challenging, due to the complex nature of ear sensory innervation, resulting in clinical overlap with trigeminal neuralgia (TN) and glossopharyngeal neuralgia (GPN). METHODS: A retrospective review of a prospective neurosurgical database at our institution was performed, 2000-2017, with a corresponding systematic literature review. Pain outcomes were dichotomized as unfavorable for unchanged/worsened symptoms versus favorable if improved/resolved. Eight formalin-fixed brains were examined to describe NI at the brainstem. RESULTS: Eleven patients were surgically treated for GN-9 primary, 2 reoperations. The median age was 48, 7 patients were female, and the median follow-up was 11 months (range 3-143). Seven had ≥ 2 probable cranial neuralgias. NI was sectioned in 9 and treated via microvascular decompression (MVD) in 2. Five patients underwent simultaneous treatment for TN (4 MVD; 1 rhizotomy) and 5 for GPN (3 MVD; 2 rhizotomy). Eleven reported symptomatic improvement (100%); 8 initially reported complete resolution (73%). Pain outcomes at last contact were favorable in 8 (73%)-all among the 9 primary operations (89% vs 0%, p = 0.054). Six prior series reported outcomes in 111 patients. CONCLUSIONS: GN is rare, and diagnosis is confounded by symptomatic overlap with TN/GPN. Directed treatment of all possible neuralgias improved pain control in almost all primary operations. Repeat surgery seems a risk factor for an unfavorable outcome. NI is adherent to superomedial VIII at the brainstem; the intermediate/cisternal portion is optimal for visualization and sectioning.

New-onset seizure during and after brain tumor excision: a risk assessment analysis.

OBJECTIVE Prophylactic use of antiepileptic drugs (AEDs) in seizure-naïve brain tumor patients remains a topic of debate. This study aimed to characterize a subset of patients at highest risk for new-onset perioperative seizures (i.e., intraoperative and postoperative seizures occurring within 30 days of surgery) who may benefit from prophylactic AEDs. METHODS The authors conducted a retrospective case-control study of all adults who had undergone tumor resection or biopsy at the authors' institution between January 1, 2004, and June 31, 2015. All patients with a history of preoperative seizures, posterior fossa tumors, pituitary tumors, and parasellar tumors were excluded. A control group was matched to the seizure patients according to age (± 0 years). Demographic data, clinical status, operative data, and postoperative course data were collected and analyzed. RESULTS Among 1693 patients who underwent tumor resection or biopsy, 549 (32.4%) had never had a preoperative seizure. Of these 549 patients, 25 (4.6%) suffered a perioperative seizure (Group 1). A total of 524 patients (95.4%) who remained seizure free were matched to Group 1 according to age (± 0 years), resulting in 132 control patients (Group 2), at an approximate ratio of 1:5. There were no differences between the patient groups in terms of age, sex, race, relationship status, and neurological deficits on presentation. Histological subtype (infiltrating glioma vs meningioma vs other, p = 0.041), intradural tumor location (p < 0.001), intraoperative cortical stimulation (p = 0.004), and extent of resection (less than gross total, p = 0.002) were associated with the occurrence of perioperative seizures. CONCLUSIONS While most seizure-naïve brain tumor patients do not benefit from perioperative seizure prophylaxis, such treatment should be considered in high-risk patients with supratentorial intradural tumors, in patients undergoing intraoperative cortical stimulation, and in patients in whom subtotal resection is likely.