Delayed Migration of Onyx Embolic Agent after Preoperative Embolization of an Arteriovenous Malformation in a Pediatric Patient: A Case Report and Review of the Literature.

INTRODUCTION: Brain arteriovenous malformations (AVMs) are increasingly being treated with Onyx liquid embolic agent (Onyx, Medtronic, Inc.). The phenomenon of delayed Onyx migration is not well documented in the literature. Moreover, the clinical presentation associated with Onyx migration is not well understood. CASE PRESENTATION: A pediatric patient with a history of neonatal seizures was referred to our institution upon experiencing daily headaches with photophobia, phonophobia, and sleep disturbance. Cerebral angiography revealed an AVM of the medial left cerebellar hemisphere. Preoperative embolization with Onyx liquid embolic achieved 25% closure of the AVM nidus. Upon developing worsening headaches the following day, new perinidal parenchymal edema was revealed on MRI, and urgent angiography demonstrated delayed migration of Onyx into the venous drainage. The patient underwent emergency resection of the AVM due to the risk of hemorrhage resulting from venous outflow obstruction. CONCLUSION: Our report and literature review demonstrate that while the delayed, unexpected migration of Onyx embolic material has been alluded to in a handful of papers, this phenomenon is not well documented. Future research is needed to understand the frequency of delayed Onyx migration from brain AVMs and the possible clinical presentations to look for. The sudden development of headaches and other signs of perilesional edema, in particular, should prompt repeat angiographic examination due to the possibility of delayed liquid embolic migration.

Repair of the superior sagittal sinus following penetrating intracranial injury caused by nail gun accident: case report and technical note.

We report a 45-year-old man who suffered a penetrating nail gun injury resulting in damage to the lateral edge of the superior sagittal sinus. The injury was successfully treated via a parasagittal craniotomy that enabled removal of the nail under direct vision, allowing for rapid suturing of the sagittal sinus. Two neurosurgeons worked together; one carefully withdrew the tip of the nail back into the sinus itself while the second rapidly sutured the hole in the inner superior sagittal sinus leaflet. Postoperatively, the patient made a rapid recovery without neurological deficit.

Contralateral approach for the treatment of a distal supraclinoid aneurysm: a technical case report.

BACKGROUND: When treating intracranial aneurysms with open microsurgery, rare cases arise in which an ipsilateral approach leads to poor visualization, lack of proximal control, or potential damage to nearby vital structures due to the anatomy of the aneurysm. CASE DESCRIPTION: We describe a patient with a small, unruptured aneurysm arising from the medial aspect of the distal supraclinoid internal carotid artery (ICA), just below the ICA bifurcation. A contralateral surgical approach was chosen because our view of the aneurysm from an ipsilateral approach would have been obstructed by the ICA. The contralateral approach provided excellent exposure of the aneurysm and allowed for precise clip placement without complications. CONCLUSIONS: Contralateral approaches may be a good option for some small medially pointing aneurysm of large proximal cerebral arteries.

Technical note: the use of frameless stereotactic guidance in the treatment of peripheral intracranial aneurysms.

Frameless stereotactic guidance (FSG) has previously been reported to have advantages over intraoperative computed tomography (CT) and frame-based imaging guidance methods in the targeting of intracranial lesions. We report our experience using FSG to minimize brain dissection during microsurgical repair of peripheral aneurysms. We used FSG as a surgical adjunct in the management of 91 peripheral aneurysms. It was used to localise and avoid larger bridging veins, enabling us to minimise unnecessary brain dissection by coming directly down on the aneurysm dome in unruptured lesions or targeting the parent artery just proximal to the aneurysm in ruptured cases. We treated 72 aneurysms located on the distal ACA (79%), 7 on the PCA (7.7%), 6 on the MCA distal to the MCA bifurcation (6.6%), and 6 on the SCA (6.6%). There were no complications related to FSG use. However, we noted a tendency to create an overly limited corridor to the aneurysm, which did not allow sufficient proximal or distal control of the parent artery. In these cases, we had to widen our exposure by further opening the interhemispheric fissure to obtain more proximal control once the aneurysm was reached. Subsequently, we learned to avoid this problem by creating a slightly wider corridor during the initial exposure. Using FSG as a surgical adjunct for peripheral intracranial aneurysms allowed us to safely limit craniotomy size and brain dissection while more confidently exposing these unusually situated lesions, facilitating aneurysm clipping in our series.

Non-islet cell tumor-induced hypoglycemia in the setting of metastatic intracranial hemangiopericytoma: case report and review of the literature.

Intracranial hemangiopericytoma, particularly in the setting of extracranial metastatic disease, can cause severe hypoglycemia; but there is limited description of this phenomenon in the neurosurgical literature. We report the case of a male patient with recurrent meningeal hemangiopericytoma that had metastasized to multiple extracranial locations, who presented with severe, symptomatic hypoglycemia and abnormal levels of insulin, pro-insulin, insulin-like growth factors I and II, and C-peptide. This case highlights the association between hemangiopericytoma and abnormal levels of peptide hormones involved in glucose homeostasis, which may serve as a warning sign for hemangiopericytoma and/or metastatic disease.

Intraventricular haemorrhage due to re-ruptured arteriovenous malformation cleared with tissue plasminogen activator administered through a pre-existing ventriculoperitoneal shunt.

In the context in intraventricular haemorrhage (IVH), intrathecal thrombolytic agents administered in conjunction with extraventricular drainage have been demonstrated to clear larger volumes of blood and reduce mortality rates. However, patients with arteriovenous malformations (AVM) have been mostly excluded from clinical trials. We describe a patient with hydrocephalus secondary to a ruptured AVM who was treated via external ventriculostomy, which was subsequently converted to a ventriculoperitoneal shunt (VPS). Eight months later, the AVM re-ruptured, causing IVH and rendering the patient comatose. Taking into consideration the patient's poor outlook, a single dose of intraventricular tissue plasminogen activator (t-PA) was administered through the shunt reservoir. The shunt maintained its function and the patient's condition ultimately improved. This impressive case demonstrates the utility of t-PA administered through an existing VPS in the setting of IVH due to ruptured AVM, highlighting its lifesaving potential in the appropriate patient and overall decrease in the cost of care by mitigating the need for shunt revision.

Preventing cerebral spinal fluid leakage following endoscopy through a burr hole using a novel watertight closure: technical note.

BACKGROUND: Cerebrospinal fluid (CSF) leakage is a common complication after neuroendoscopic surgery through a burr hole and can lead to further complications including infection. METHODS: We describe the use of a dural substitute larger than the burr hole itself, placed over the burr hole and then secured underneath a burr hole cover by microscrews running through the graft itself into the underlying skull. RESULTS: This simple technical modification contributes to achieving a watertight seal to aid in preventing CSF leakage in this setting. CONCLUSIONS: Our technical modification of endoscopy through a burr hole may help to prevent postoperative CSF leak and secondary CSF infections.

Case report and literature review of BRAF-V600 inhibitors for treatment of papillary craniopharyngiomas: A potential treatment paradigm shift.

WHAT IS KNOWN AND OBJECTIVE: The BRAF-V600E genetic mutation offers a potential targeted therapy for the treatment of papillary craniopharyngiomas. CASE SUMMARY: A 35-year-old man underwent a craniotomy and subtotal resection of a large BRAF-V600E-positive papillary craniopharyngioma before referral to our institution. Our treatment included the BRAF-V600 inhibitor dabrafenib mesylate (75 mg, twice/day) and trametinib dimethyl sulfoxide (2 mg/day). The residual tumour decreased in size by 95% over 21 months without negative side effects. WHAT IS NEW AND CONCLUSION: We reviewed the literature on BRAF-V600E inhibition as a non-invasive method of treating papillary craniopharyngiomas harbouring the BRAF-V600E mutation.

Delayed sudden hearing recovery after treatment of a large vertebral artery aneurysm causing hearing loss and imbalance: a case report.

PURPOSE: Cerebral aneurysms that compress cranial nerve VIII can cause hearing loss and imbalance. Hearing function that does not recover after aneurysm occlusion can signal neurological damage with the potential for permanent deafness. CASE DESCRIPTION: A 72-year-old woman presented with gradually worsening left-sided hearing loss and imbalance over a period of 10 years. She was found to have a lesion of the cerebellopontine angle, which proved to be a large fusiform vertebral artery aneurysm with mass effect on cranial nerve VIII. The patient underwent surgical clip occlusion of the vertebral artery distal to the posterior inferior cerebellar artery and proximal to the aneurysm, which no longer filled on catheter angiography. Postoperatively, the patient experienced delayed complete loss of ipsilateral hearing on the third post-operative day. Otherwise, she made a good recovery with improvement in her balance issues. At that time, we suspected that delayed occlusion of a perforating vessel had probably caused irreversible hearing loss. Ten months later, the patient awoke with significant subjective recovery of her hearing. Audiometry confirmed substantial improvement in her hearing likely due to the aneurysm shrinking away from and decompressing the cranial nerve. CONCLUSION: This case highlights the continued usefulness of vascular occlusion in the management of selected cases of intracranial aneurysms and also that neurological function may recover suddenly, even in very delayed fashion, following treatment.

Procedural complications in patients undergoing microsurgical treatment of unruptured intracranial aneurysms: a single-center experience with 1923 aneurysms.

BACKGROUND: With the growing use of endovascular therapy (EVT) to manage unruptured intracranial aneurysms (IAs), detailed information regarding periprocedural complication rates of microsurgical clipping and EVT becomes increasingly important in determining the optimal treatment for individual cases. We report the complication rates associated with open microsurgery in a large series of unruptured IAs and highlight the importance of maintaining surgical skill in the EVT era. METHODS: We reviewed all cases of unruptured IAs treated with open microsurgery by a single neurosurgeon between July 1997 and June 2019. We analyzed surgical complications, deaths, and patient-reported outcomes. RESULTS: A total of 1923 unruptured IAs in 1750 patients (mean age 44 [range: 6-84], 62.0% [1085/1750] female) were treated surgically during the study period. Of the aneurysms treated, 84.9% (1632/1923) were small, 11.1% (213/1923) were large, and 4.1% (78/1923) were giant. Aneurysm locations included the middle cerebral artery (44.2% [850/1923]), internal carotid artery (29.1% [560/1923]), anterior cerebral artery (21.0% [404/1923]), and vertebrobasilar system (5.7% [109/1923]). The overall mortality rate was 0.3% (5/1750). Surgical complications occurred in 7.4% (129/1750) of patients, but only 0.4% (7/1750) experienced permanent disability. The majority of patients were able to return to their preoperative lifestyles with no modifications (95.9% [1678/1750]). CONCLUSIONS: At a high-volume, multidisciplinary center, open microsurgery in carefully selected patients with unruptured IAs yields favorable clinical outcomes with low complication rates. The improvement of EVT techniques and the ability to refer cases for EVT when a high complication rate with open microsurgery was expected have contributed to an overall decrease in surgical complication rates. These results may serve as a useful point of reference for physicians involved in treatment decision-making for patients with unruptured IAs.

Traumatic epidural hematoma treated with endovascular coil embolization.

BACKGROUND: Traumatic cerebrovascular injury may result in epidural hematoma (EDH) from laceration of the middle meningeal artery (MMA), which is a potentially life-threatening emergency. Treatment ranges from surgical evacuation to conservative management based on a variety of clinical and imaging factors. CASE DESCRIPTION: A 14-year-old male presented to our institution after falling from his bicycle with traumatic subarachnoid hemorrhage and a right frontotemporal EDH. The patient did not meet criteria for surgical evacuation and endovascular embolization of the right MMA was performed. Rapid resolution of the EDH was observed. CONCLUSION: This case corroborates the sparse existing literature for the potential role of endovascular embolization to treat acute EDH in carefully selected patients who do not meet or have borderline indications for surgical management.

Neoplastic cerebral aneurysm from triple-negative breast cancer: A case report.

BACKGROUND: We present a rare case of a ruptured neoplastic aneurysms (NCA) caused by metastatic spread of triple-negative breast cancer (TNBC) in a female patient in her 60s. The patient had a medical history of TNBC and presented to the emergency department after experiencing 3 days of persistent headache. CASE DESCRIPTION: Head computed tomography (CT) revealed a small volume subarachnoid hemorrhage and digital subtraction angiography revealed a 3.9 x 3.5 x 4.2 mm aneurysm or pseudoaneurysm involving the left middle cerebral artery. The aneurysm was successfully clipped and resected, and histopathological examination confirmed triple-negative invasive ductal breast carcinoma within the aneurysm. Six weeks after surgery, she underwent stereotactic radiosurgery and began treatment with chemotherapy. Four months later, the patient presented once again with acute severe headache, and magnetic resonance imaging revealed multiple small lesions within the brain parenchyma, compatible with new metastatic deposits. The patient was subsequently treated with whole-brain radiation therapy and chemotherapy. Over the ensuing 4 months, CT revealed progression of malignancy in the chest, abdomen, and pelvis. Chemotherapy and radiation therapy were terminated, and the patient unfortunately succumbed to her disease 6 months later. CONCLUSION: In patients with NCA with poor prognosis due to aggressive brain metastases, treatments that improve quality of life and survival time should be favored.

CT cisternography to visualize epidermoid tumors for stereotactic radiosurgery treatment planning.

The visualization of intracranial epidermoid tumors is often limited by difficulties associated with distinguishing the tumor from the surrounding cerebrospinal fluid using traditional computed tomography (CT) or magnetic resonance imaging (MRI) modalities. This report describes our experience using CT cisternography to visualize intracranial epidermoid tumors in three illustrative cases. CT cisternography of the epidermoid tumor provides more clarity and precision compared to traditional neuroimaging modalities. We demonstrate the feasibility of using CT cisternography to produce high-resolution images with well-defined tumor margins that can be used effectively for precise SRS treatment planning.

Bilateral Vestibular Schwannomas in a Patient with Ring Chromosome 22: Case Report and Review of the Literature.

INTRODUCTION: Ring chromosome 22 (r[22]) can lead to the development of intracranial tumors such as meningiomas, neurofibromas, and schwannomas similar to neurofibromatosis 2 (NF2). CASE PRESENTATION: An 18-year-old female with r(22) and a history of global development delay and cognitive impairment presented with sudden hearing loss. MRI revealed bilateral vestibular schwannomas. Given documented audiologic decline in the patient's hearing, the larger tumor was treated with CyberKnife fractionated stereotactic radiosurgery, and the smaller tumor is being monitored. CONCLUSION: This case provides further evidence that patients with r(22) can develop clinical features of NF2, including the development of bilateral vestibular schwannomas, and should be monitored for hearing disturbances starting in puberty as a warning sign for these tumors.

Cognitive deficits among patients surviving aneurysmal subarachnoid hemorrhage. A contemporary systematic review.

BACKGROUND: Subarachnoid hemorrhage (SAH) is associated with high rates of morbidity, including neurological and cognitive deficits that may be difficult to identify and quantify. This review provides an update on the cognitive deficits that may result from spontaneous aneurysmal SAH (aSAH) and identifies factors that may help predict and manage these deficits at discharge and thereafter. MATERIALS AND METHODS: We conducted a systematic review of PubMed and Google Scholar to identify studies published between 2010 and 2019 that assessed cognitive deficits at discharge and during follow-up in patients with aSAH. Full-text articles were assessed for information regarding cognitive testing and factors that may be associated with functional outcomes in this population. RESULTS: We reviewed 65 studies published since 2010 that described the cognitive deficits associated with non-traumatic aSAH. Such deficits may impact functional outcomes, quality of life, and return to work and may result in cognitive impairments, such as memory difficulties, speech problems, and psychiatric disorders. CONCLUSIONS: Patients with aSAH, even those that appear normal at the time of hospital discharge, may harbor cognitive deficits that are difficult to detect, yet can interfere with daily functioning. Further research is needed to provide additional information and to identify stronger correlations to be used in the identification, treatment, and amelioration of long-term cognitive deficits in aSAH patients, including those who are discharged with good clinical outcomes scores.

Adenosine-induced transient asystole to control intraoperative rupture of intracranial aneurysms: institutional experience and systematic review of the literature.

BACKGROUND: Intraoperative rupture of an intracranial aneurysm is a life-threatening situation that carries a high risk of morbidity and mortality. Since 2000, adenosine has been used successfully to induce transient hypotension and/or asystole to control bleeding and facilitate surgical clipping of aneurysms that rupture intraoperatively. Given the paucity of reports describing this method in a limited number of patients, we performed a systematic review of the literature detailing the use and outcomes of this technique. METHODS: The authors performed a systematic review and identified all studies in which adenosine was used in the setting of an intracranial aneurysm that ruptured intraoperatively. We then determined overall morbidity and mortality rates, adding an additional six of our own patients. RESULTS: Data was analyzed for a total of 29 patients, including 23 previously reported patients from the literature and 6 additional cases from our own experience (mean age 54.8 years, 58.6% female). Most patients (82.8%, 24/29) presented with subarachnoid hemorrhage (SAH). Overall mean dose of adenosine was 51.8 mg. Successful clipping was achieved in 100% of patients. Transient or permanent morbidity was reported in 5/29 (17.2%) and the overall mortality rate was 31% (9/29), which occurred primarily due to an initial severe SAH and its resultant complications. CONCLUSIONS: Adenosine-induced circulatory arrest appears to safely control intraoperative bleeding and facilitate the clipping of ruptured intracranial aneurysms based on the limited published literature available. Further studies comparing patient outcomes using this technique to traditional approaches are required to validate the safety and efficacy of adenosine in this high-risk setting.

Primary intracranial angiomatoid fibrous histiocytoma: a case report and literature review.

Angiomatoid fibrous histiocytoma (AFH) is a tumour primarily occurring in the extremities which can very rarely occur as an isolated intracranial lesion. We report a case of a 22-year-old woman presenting with generalized seizure and visual field deficit due to an occipital mass, which immunohistochemistry showed to be an AFH.

Non-myxoma neoplastic cerebral aneurysms: A systematic review.

Neoplastic cerebral aneurysms (NCAs) are highly rare lesions characterized by invasion of cancerous cells within the wall of an artery leading to aneurysm formation. While NCAs caused by myxomas are well characterized in the clinical literature, rarer etiologies have also been reported and are typically associated worse clinical outcomes. We performed the first PRISMA-compliant systematic literature review of true, non-myxoma neoplastic cerebral aneurysms using the PubMed/MEDLINE, Embase, Scopus, and Google Scholar databases. Data of interest included age, sex, aneurysm size, number of aneurysms, aneurysm location, neoplasm type, aneurysm treatments, cancer treatments, risk of rupture, intracerebral hemorrhage prevalence, subarachnoid hemorrhage prevalence, and survival at 90 and 180 days. A total of 50 studies met our inclusion criteria. The mean age of the patient population was 37.4 years (SD: ±16.8) and had an overall female preponderance (39/50, 78%). Of these NCA cases, 29/50 (58.0%) were choriocarcinomas, 10/50 (20.0%) were related to lung cancer, and 11/50 (22.0%) had other origins of variable pathologies. 90-day survival rates were 60.0% (15/25) for choriocarcinomas, 28.6% (2/7) for the lung cancer group, and 14.3% (1/7) for the other origins group. 180-day survival rates were 52.0% (13/25) for the choriocarcinoma group, 14.3% (1/7) for the lung cancer group, and 0% (0/7) for the other origins group. Prognosis of NCA patients ultimately depends on the course of disease progression and cancer management. Further research is needed to better understand optimal treatment modalities for patients with NCAs.

Delayed Onset of an Intradural Epidermoid Tumor in the Lumbar Region Seven Years After Spinal Anesthesia for Childbirth: A Case Report.

Epidural or spinal anesthesia is commonly administered in births in the US, and the potential risks for epidermoid tumors are not well-characterized. We present the case of a 29-year-old female patient who developed an intradural epidermoid tumor in the lumbar spine, discovered seven years after spinal anesthesia for childbirth. MRI revealed a 4 cm tumor filling the entire spinal canal. Pathology confirmed the mass to be an epidermoid. Complete surgical resection of the intradural lesion was accomplished with full symptomatic relief. This case supports the relationship between delayed development of epidermoid tumors and spinal puncture in adult populations.

A Systematic Review of Intracranial Aneurysms in the Pregnant Patient - A Clinical Conundrum.

Hemodynamic and hormonal changes during pregnancy can increase rates of formation, growth, and rupture of intracranial aneurysms (IA), and the increased incidence of subarachnoid hemorrhage (SA) in pregnant patients represents a risk to both mother and fetus. Despite this, management and treatment guidelines have not been defined for this patient population. In most instances, treatment decisions are made on a case-by-case basis with varying degrees of input from subspecialists. Important considerations, such as aneurysm location, morphology, size, growth pattern, and patient characteristics have not been presented in a management algorithm in the context of the pregnant patient. Given limited and controversial literature describing management of IAs in pregnant patients, we performed a systematic literature review. We then describe our multidisciplinary team approach and provide analysis of existing literature to provide guidelines for the management of the pregnant patient with an IA. A systematic review was undertaken to identify studies describing the management of IAs in the pregnant patient using the PubMed database. Overall maternal and fetal morbidity and mortality rates were determined. Data was analyzed for 1537 patients, including 1115 (73%) pregnant with ruptured intracranial aneurysms. In most cases, these aneurysms were managed conservatively (77%; 781/1013); however, when treatment was provided, surgical clipping was the most common modality (15%; 149/1013). Overall maternal outcomes were reported for 934 cases with morbidity and mortality rates of 5% (42/934) and 21% (194/934), respectively. Overall fetal outcomes were reported for 114 cases with morbidity and mortality rates of 10% (12/119) and 8% (9/119), respectively. Pregnancy-associated physiological changes likely elevate the risk of intracranial aneurysm formation, growth, and rupture. Treatment for aneurysms and SAs is safe and effective during pregnancy when risks are properly mitigated. Due to the complexity of care, such patients should be treated using a collaborative, interdisciplinary approach by a multidisciplinary team.