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OBJECTIVEParaclinoid aneurysms represent approximately 5% of intracranial aneurysms (Drake et al. [1968]). Visual impairment, which occurs in 16%–40% of patients, is among the most common presentations of these aneurysms (Day [1990], Lai and Morgan [2013], Sahlein et al. [2015], and Silva et al. [2017]). Flow-diverting stents, such as the Pipeline Embolization Device (PED), are increasingly used to treat these aneurysms, in part because of their theoretical reduction of mass effect (Fiorella et al. [2009]). Limited data on paraclinoid aneurysms treated with a PED exist, and few studies have compared outcomes of patients after PED placement with those of patients after clipping or coiling.METHODSThe authors performed a retrospective analysis of 115 patients with an aneurysm of the cavernous to ophthalmic segments of the internal carotid artery treated with clipping, coiling, or PED deployment between January 2011 and March 2017. Postoperative complications were defined as new neurological deficit, aneurysm rupture, recanalization, or other any operative complication that required reintervention.RESULTSA total of 125 paraclinoid aneurysms in 115 patients were treated, including 70 with PED placement, 23 with coiling, and 32 with clipping. Eighteen (14%) aneurysms were ruptured. The mean aneurysm size was 8.2 mm, and the mean follow-up duration was 18.4 months. Most aneurysms were discovered incidentally, but visual impairment, which occurred in 21 (18%) patients, was the most common presenting symptom. Among these patients, 15 (71%) experienced improvement in their visual symptoms after treatment, including 14 (93%) of these 15 patients who were treated with PED deployment. Complete angiographic occlusion was achieved in 89% of the patients. Complications were seen in 17 (15%) patients, including 10 (16%) after PED placement, 2 (9%) after coiling, and 5 (17%) after clipping. Patients with incomplete aneurysm occlusion had a higher rate of procedural complications than those with complete occlusion (p = 0.02). The rate of postoperative visual improvement was significantly higher among patients treated with PED deployment than in those treated with coiling (p = 0.01). The significant predictors of procedural complications were incomplete occlusion (p = 0.03), hypertension, (p = 0.04), and diabetes (p = 0.03).CONCLUSIONSIn a large series in which patient outcomes after treatment of paraclinoid aneurysms were compared, the authors found a high rate of aneurysm occlusion and a comparable rate of procedural complications among patients treated with PED placement compared with the rates among those who underwent clipping or coiling. For patients who presented with visual symptoms, those treated with PED placement had the highest rate of visual improvement. The results of this study suggest that the PED is an effective and safe modality for treating paraclinoid aneurysms, especially for patients who present with visual symptoms.

BACKGROUNDPure arterial malformations (PAMs) are rare vascular anomalies that are commonly mistaken for other vascular malformations. Because of their purported benign natural history, PAMs are often conservatively managed. The authors report the case of a ruptured PAM leading to subarachnoid hemorrhage (SAH) with intraventricular extension that was treated endovascularly.OBSERVATIONSA 38-year-old man presented with a 1-day history of headaches and nausea. A computed tomography scan demonstrated diffuse SAH with intraventricular extension, and angiography revealed a right posterior inferior cerebellar artery–associated PAM. The PAM was treated with endovascular Onyx embolization.LESSONSTo the authors’ knowledge, only 2 other cases of SAH associated with PAM have been reported. In those 2 cases, surgical clipping was pursued for definitive treatment. Here, the authors report the first case of a ruptured PAM treated using an endovascular approach, showing its feasibility as a treatment option particularly in patients in whom open surgery is too high a risk.

Object The resection of glomus jugulare tumors can be challenging because of their inherent vascularity. Preoperative embolization has been advocated as a means of reducing operative times, blood loss, and surgical complications. However, the incidence of cranial neuropathy associated with the embolization of these tumors has not been established. The authors of this study describe their experience with cranial neuropathy following transarterial embolization of glomus jugulare tumors using ethylene vinyl alcohol (Onyx, eV3 Inc.). Methods The authors retrospectively reviewed all cases of glomus jugulare tumors that had been treated with preoperative embolization using Onyx at their institution in the period from 2006 to 2012. Patient demographics, clinical presentation, grade and amount of Onyx used, degree of angiographic devascularization, and procedural complications were recorded. Results Over a 6-year period, 11 patients with glomus jugulare tumors underwent preoperative embolization with Onyx. All embolization procedures were completed in one session. The overall mean percent of tumor devascularization was 90.7%. No evidence of nontarget embolization was seen on postembolization angiograms. There were 2 cases (18%) of permanent cranial neuropathy attributed to the embolization procedures (facial nerve paralysis and lower cranial nerve dysfunction). Conclusions Embolizing glomus jugulare tumors with Onyx can produce a dramatic reduction in tumor vascularity. However, the intimate anatomical relationship and overlapping blood supply between these tumors and cranial nerves may contribute to a high incidence of cranial neuropathy following Onyx embolization.

Object As endovascular techniques have become more advanced, preoperative embolization has become an increasingly used intervention in the management of meningiomas. To date, however, no consensus has been reached on the use of this technique. To clarify the role of preoperative embolization in the management of meningiomas, the authors conducted a systematic review of case reports, case series, and prospective studies to increase the current understanding of the management options for these common lesions and complications associated with preoperative embolization. Methods A PubMed search was performed to include all relevant studies in which the management of intracranial meningiomas with preoperative embolization was reported. Immediate complications of embolization were reported as major (sustained) or minor (transient) deficits, death, or no neurological deficits. Results A total of 36 studies comprising 459 patients were included in the review. Among patients receiving preoperative embolization for meningiomas, 4.6% (n = 21) sustained complications as a direct result of embolization. Of the 21 patients with embolization-induced complications, the incidence of major complications was 4.8% (n = 1) and the mortality rate was 9.5% (n = 2). Conclusions Preoperative embolization is associated with an added risk for morbidity and mortality. Preoperative embolization may be associated with significant complications, but careful selection of ideal cases for embolization may help reduce any added morbidity with this procedure. Although not analyzed in the authors' study, embolization may still reduce rates of surgical morbidity and mortality and therefore may still have a potential benefit for selected patients. Future prospective studies involving the use of preoperative embolization in certain cases of meningiomas may further elucidate its potential benefit and risks.

Object Retinoblastoma is the most common ocular neoplasm in children. Left untreated it spreads to the brain via the optic nerve. Traditional therapy is enucleation, and while this procedure is still the most common treatment worldwide, modern eye-preserving therapies can often spare the globe. However, patients with retinoblastoma often present in advanced stages of the disease when these globe-preserving strategies are often insufficient to prevent enucleation. In these challenging cases, direct infusion of chemotherapy into the ophthalmic artery has been attempted to achieve tumor control. The authors' aim in this study was to report on their initial experience with and clinical results for this approach. Methods The authors prospectively collected data on all cases of retinoblastoma treated with selective intraophthalmic melphalan at Bascom Palmer Eye Institute. All cases were classified as International Intraocular Retinoblastoma Classification (IIRC) Group D or Reese-Ellsworth Group Vb, had not responded to aggressive multimodal therapy consisting of chemotherapy and focal consolidating laser therapy, and were pending enucleation. Using digital subtraction angiography, a microcatheter was navigated under roadmap guidance into the ophthalmic artery, and melphalan was infused over 40 minutes. Early in the series, patients were treated with 3 or 5 mg of melphalan, but after low response rates occurred all eyes were treated with 7.5 mg of melphalan. All patients were examined with funduscopy while under anesthesia 3 weeks after treatment and every 3 months thereafter. Patients with persistent disease were retreated with repeat infusions of melphalan. Results Twenty-six procedures were performed to treat 17 tumors in 15 patients. Successful cannulation of the ophthalmic artery was achieved in all cases. The follow-up ranged from 3 to 12 months, with a mean of 8.6 months. Overall, 76% of the tumors responded to therapy and these cases were spared enucleation. The average number of treatments was 1.5 per tumor. Of the responders, 54% responded to a single dose of melphalan. Treatment with the higher dose of 7.5 mg up front was associated with a lower enucleation rate (0% vs 36%) as compared with the lower starting dose. Delayed vitreous hemorrhage occurred after 4 (15%) of 26 treatments, and these cases were treated with enucleation. Conclusions In this challenging group of advanced retinoblastomas refractory to aggressive multimodal therapy, virtually 100% of eyes are generally enucleated. In contrast, the authors' protocol of infusing melphalan directly into the ophthalmic artery led to a dramatic decrease in the enucleation rate to 23.5%. While it is now the treatment of choice for refractory retinoblastoma at their center, its role in less advanced disease remains to be elucidated.

Juvenile nasopharyngeal angiofibromas are vascular tumors that may make resection difficult and potentially dangerous. Preoperative embolization is frequently used to decrease surgical morbidity and blood loss. Embolization has typically been performed via a transarterial route using a variety of embolic materials. The authors present a case in which endoscopic assistance was used for direct transnasal tumor puncture and intratumoral embolization using the liquid embolic agent Onyx. In this case there was excellent infiltration of the parenchymal vasculature with complete angiographic obliteration. There were no complications related to the embolization. The tumor was resected with minimal blood loss. To the authors' knowledge, there have been no previous reports of this novel direct intratumoral embolization technique using endoscopic guidance.

The treatment of indirect carotid cavernous fistulas (CCFs) is challenging and primarily accomplished by endovascular means utilizing a variety of embolic agents. Transvenous access to the cavernous sinus is the preferred method of embolizaiton of indirect CCFs as they are frequently associated with numerous small-caliber meningeal branches. Although the inferior petrosal sinus is the simplest, shortest, and most commonly used venous route to the cavernous sinus, the superior ophthalmic vein, superior petrosal sinus, basilar plexus, and pterygoid plexus present other endovenous options. Occasionally, however, use of these venous routes may not be possible due to vessel tortuosity or sinus thrombosis and occlusion. The authors report a case of an indirect CCF that could not be treated endovascularly due to inability to access the cavernous sinus via a transfemoral transvenous approach. Angiography revealed a small, deeply located superior ophthalmic vein that was thought to be suboptimal for a direct cutdown. The cavernous sinus was cannulated directly via a transorbital approach using fluoroscopic guidance with a 3D skull reconstruction overlay. The fistula was subsequently obliterated using ethylene vinyl alcohol copolymer (Onyx). The technique and advantages of both 3D osseous reconstruction as well as Onyx embolization are discussed.

Endovascular obliteration of wide-necked aneurysms may be precluded by the inability to navigate across the aneurysm neck. The authors present a technique in which a Hyperform balloon is inflated within the aneurysm and used as a contact surface to “bounce” the remodeling balloon across the aneurysm neck. They have successfully used this technique in 3 patients to efficiently overcome vessel tortuosity, aneurysmal dead space, and balloon prolapse, allowing for obliteration of large, wide-necked aneurysms.

Object The authors assessed the safety and efficacy of embolization of head, neck, and spinal tumors with Onyx and determined the correlation between tumor embolization and intraoperative blood loss. Methods The authors prospectively collected all head, neck, and spinal tumors embolized with Onyx at their institution over a 28-month period. Information on tumor type, location, extent of tumor devascularization, endovascular and surgical complications, and intraoperative estimated blood loss (EBL) was evaluated. Results Forty-three patients with various head, neck, and spinal lesions underwent vascular tumor embolization with Onyx. Indications for embolization included uncontrolled tumor bleeding in 8 cases, elective preoperative devascularization in 34, and tumor-induced consumptive thrombocytopenia in 1 case. Embolization was performed via direct tumoral puncture in 14 cases and through the traditional transarterial route in the remaining lesions. Embolization was successful in ending uncontrolled tumor bleeding in all 8 cases and in reversing the consumptive coagulopathy in 1 case. Intraparenchymal penetration of embolic material was possible in all percutaneously embolized tumors and in 4 of the 20 tumors embolized preoperatively via the transarterial route. The mean percentage of devascularization in tumors with intraparenchymal penetration of Onyx was 90.3% compared with 83.7% in tumors without intraparenchymal penetration. The mean EBL with intraparenchymal penetration of Onyx was significantly lower than when there was no intraparenchymal penetration (459 vs 2698 ml; p = 0.0067). There were no neurological complications related to the embolization procedures. Conclusions Embolization of vascular tumors with Onyx can be performed safely but may not reach optimal effectiveness in reducing intraoperative EBL if the embolic material does not penetrate the tumor vasculature. In the authors' experience, the best method of intraparenchymal penetration is achieved with direct tumor puncture. Transarterial embolization may not result in tumor penetration, particularly when injected from a long distance through small caliber or slow flow vessels.

Object The authors conducted a study to determine the safety and efficacy of embolization of carotid-cavernous fistulas (CCFs) with the ethylene vinyl alcohol copolymer, Onyx. Methods They prospectively collected data in all patients with CCFs who underwent Onyx-based embolization at their institution over a 3-year period. The type of fistula, route of embolization, viscosity of Onyx, additional use of coils, extent of embolization, procedural complications, and clinical follow-up were recorded. Results A total of 12 patients (5 men and 7 women who were age 24–88 years) underwent embolization in which Onyx was used. There were 1 Barrow Type A, 1 Type B, 3 Type C, and 7 Type D fistulas. Embolization was performed via a transvenous route in 8 cases and a transarterial route in 4 cases. Onyx 34 was used in all but 2 cases: a direct Type A fistula embolized with Onyx 500 and an indirect Type C fistula embolized with Onyx 18. Adjuvant embolization with framing coils was performed in 7 cases. All procedures were completed in a single session. Immediate fistula obliteration was achieved in all cases. Clinical resolution of presenting symptoms occurred in 100% of the patients by 2 months. Neurological complications occurred in 3 patients. One patient developed a complete cranial nerve (CN) VII palsy that has not resolved. Two patients developed transient neuropathies—1 a Horner syndrome and partial CN VI palsy, and 1 a complete CN III and partial CN V palsy. Radiographic follow-up (mean 16 months, range 4–35 months) was available in 6 patients with complete resolution of the lesion in all. Conclusions Onyx is a liquid embolic agent that is effective in the treatment of CCFs but not without hazards. Postembolization cavernous sinus thrombosis and swelling may result in transient compressive cranial neuropathies. The inherent gradual polymerization properties of Onyx allow for casting of the cavernous sinus but may potentially result in deep penetration within arterial collaterals that can cause CN ischemia/infarction. Although not proven, the angiotoxic effects of dimethyl sulfoxide may also play a role in postembolization CN deficits.

Eagle syndrome is an uncommon entity but is well known in the otorhinolaryngology and oral surgery literature. This syndrome results from the compression of cranial nerves in the neck by an elongated styloid process causing unilateral cervical and facial pain. The styloid process can also cause compression of the cervical carotid arteries leading to the so-called carotid artery syndrome together with carotidynia or neurological symptoms due to flow reduction in these arteries. The authors discuss the case of a 70-year-old man who suffered from transient ischemic attacks on turning his head to the left, with immediate remission of symptoms when his head returned to the neutral position. The patient was studied with dynamic angiography, which clearly showed focal flow restriction. Once a diagnosis was made, the styloid process was removed surgically and the patient completely recovered from his symptoms. A postoperative angiogram demonstrated complete resolution of the pathology. Neurosurgeons might encounter patients with Eagle syndrome and should be aware of the symptoms and signs. Once the diagnosis is made, the treatment is clear and very effective.

Carotid body tumors (CBTs) are rare highly vascular lesions that frequently require preoperative embolization to minimize surgical morbidity secondary to blood loss. Embolization has typically been performed via a transarterial route. However, this frequently results in incomplete devascularization of the tumor due to the complex angioarchitecture of the feeding arteries. Direct intralesional embolization has been used to gain easier accesses to the tumor vasculature and thus increase the likelihood of complete embolization. Cyanoacrylate glue has been the most commonly used embolic agent. The authors present a case of CBT that underwent direct intralesional embolization using Onyx (ev3; ethylene vinyl alcohol copolymer). To their knowledge, there have been no previous reports of direct percutaneous embolization of a CBT with this agent.

✓Extracranial carotid artery (CA) aneurysms are rare in the pediatric population and are usually the result of connective tissue disorders, traumatic dissection, or infection. The authors present the case of a large calcified internal carotid artery pseudoaneurysm of obscure origins presenting with embolic stroke in a child. Aneurysm excision and CA reconstruction would have been extremely difficult due to the distal location of the lesion, and CA ligation was contraindicated due to a failed balloon test occlusion. Therefore, after anticoagulation therapy, the patient was treated endovascularly with a covered stent and complete exclusion of the aneurysm from the circulation. The patient recovered all neurological function and has remained in excellent condition. A follow-up angiogram performed at 6 months showed no recurrence or stenosis.