An Uncommon Culprit: Dural Arteriovenous Fistula Presenting as Otodynia and Dizziness.

Dural arteriovenous fistulas (DAVFs) are rare vascular abnormalities that can present with diverse neurological symptoms. We report a case of a woman in her early 60s who presented with pain in the left ear and dizziness. Neurological evaluation and imaging studies revealed a DAVF in the left cerebellopontine angle. This case underscores the importance of considering DAVF as a potential etiology in patients presenting with atypical otological symptoms.

Use of the Scepter Dual-Lumen Balloon Catheter for Transarterial Onyx Embolization of Cranial Dural Arteriovenous Fistulas.

BACKGROUND: Dual-lumen balloon microcatheters allow for controlled anterograde flow of Onyx while providing proximal flow arrest, thereby obviating the need for a second microcatheter or Onyx plug formation. We sought to assess the safety and efficiency of the Scepter dual-lumen balloon microcatheter in trans arterial Onyx embolization of intracranial dural arteriovenous fistulas (DAVFs). METHODS: We conducted a retrospective study of 36 patients with cranial DAVFs in which a Scepter balloon microcatheter was used between 2016 and 2023. RESULTS: Our study comprised 36 patients, mostly male (n = 23, 63.8%) with a mean age of 60.8 years. Most DAVFs were in the occipital lobe (n = 24, 66.7%), and 50% had external carotid artery supply from the occipital artery. Eighteen (50%) of DAVFs were Cognard type III and IV, respectively. About one third (33.3%, n = 12) of the DAVFs drained into the transverse sigmoid junction, and 27.7% (n = 10) had direct cortical venous drainage into supratentorial or posterior fossa veins. Complete occlusion was obtained in 22 (61.1%) patients while partial occlusion was observed in 14 (38.9%) patients. One patient (2.8%) developed a retroperitoneal hematoma. At final follow-up, complete occlusion was observed in 21 (77.8%) and partial occlusion was observed in 8 (22.2%). Recurrence was observed in 4/30 (13.3%) patients, and retreatment was required in 6 (18.75%) cases. CONCLUSIONS: At midterm follow-up, our study showed low morbidity and modest complete occlusion rates using the Scepter for transarterial Onyx embolization of high-grade DAVFs.

Cerebellar mutism syndrome caused by bilateral cerebellar hemorrhage in adults: a case report and review of the literature.

Cerebellar mutism syndrome (CMS) is a frequent complication of surgical intervention on posterior fossa in children. It has been only occasionally reported in adults and its features have not been fully characterized. In children and in young adults, medulloblastoma is the main reason for neurosurgery. A single case of postsurgical CMS is presented in an adult patient with a cerebellar hemorrhage and a systematic review of the published individual cases of CMS in adults was done. Literature review of individual cases found 30 patients, 18/30 (60%) males, from 20 to 71 years at diagnosis. All but one case was post-surgical, but in one of the post-surgical cases iatrogenic basilar artery occlusion was proposed as cause for CMS. The causes were: primary tumors of the posterior fossa in 16/22 (72.7%) metastasis in 3/30 (10%), ischemia in 3/30 (10%) cerebellar hemorrhage in 3/30 (10%), and benign lesions in 2/30 (6.7%) patients. 8/30 patients (26.7%) were reported as having persistent or incomplete resolution of CMS within 12 months. CMS is a rare occurrence in adults and spontaneous cerebellar hemorrhage has been reported in 3/30 (10%) adult patients. The generally accepted hypothesis is that CMS results from bilateral damage to the dentate nucleus or the dentate-rubro-thalamic tract, leading to cerebro-cerebellar diaschisis. Several causes might contribute in adults. The prognosis of CMS is slightly worse in adults than in children, but two thirds of cases show a complete resolution within 6 months.

Estimation of the rupture point of the craniovertebral junction intradural arteriovenous fistula with vessel wall magnetic resonance image and its pathological findings: A case report.

Background: Arteriovenous fistulas (AVFs) of the craniocervical junction (CCJ) and intradural AVFs are often associated with aneurysms and varics, and it is sometimes difficult to identify the ruptured point on radiological images. We report a case in which vessel wall magnetic resonance image (VW-MRI) was useful for identifying the ruptured point at the CCJ AVF. Case Description: A 70-year-old man presented with a sudden onset of headache. He had Glasgow Coma Scale E4V5M6, world federation of neurosurgical societies (WFNS) Grade I. Fisher group 3 subarachnoid hemorrhage and hydrocephalus were found on head computed tomography. Cerebral angiography showed a spinal AVF at the C1 level of the cervical spine. Magnetic resonance image-enhanced motion sensitized driven equilibrium (MSDE-method showed an enhancing effect in part of the AVF draining vein, but the vascular architecture of this lesion was indeterminate. We performed continuous ventricular drainage for acute hydrocephalus and antihypertensive treatment. Cerebral angiography was performed 30days after the onset of the disease, and was revealed an aneurysmal structure in a portion of the AVF draining vein, which VW-MRI initially enhanced. On the 38th day after onset, he underwent direct surgery to occlude the AV fistula and dissect the aneurysmal structure. Histopathology showed that the aneurysmal structure was varices with lymphocytic infiltration, and hemosiderin deposition was observed near the varices. Conclusion: Recently, VW-MRI has been reported to show an association between the enhancement of varices in dural AVF and rupture cases. VW-MRI, especially the enhanced MSDE method, may be useful in estimating the ruptured point in arteriovenous shunt disease.

Dural arteriovenous fistula and sinus thrombosis presenting as parkinsonism and dementia: a case report with literature review.

Introduction: Dural arteriovenous fistula (DAVF) is an uncommon malformation involving an abnormal connection between dural arteries, or the pachymeningeal branches of cerebral arteries, and dural veins. Its exact pathogenesis remains elusive. Known potential triggers for DAVF include cerebral venous sinus thrombosis (CVST), trauma, ear infections, and cranial surgeries. Due to its rarity and diverse clinical presentations, diagnosing DAVF can be a challenge. Case description: We present a case of DAVF associated with CVST, manifesting as rapidly advancing parkinsonism accompanied by dementia over a month. Brain magnetic resonance imaging (MRI) revealed bilateral symmetric T2 hyperintensities in the basal ganglia and brain stem. Cerebral angiography further confirmed a fistula between the torcular herophili and the transverse-sigmoid sinuses. Despite strong recommendations for transvenous embolization of the fistula, the patient declined the procedure. The anticoagulant therapy and symptomatic treatments administered did not yield any improvement in the patient's condition. Additionally, we reviewed 27 DAVF-derived parkinsonism and dementia cases. Conclusion: DAVF must be considered in the differential diagnosis of cases of rapidly progressive parkinsonism with concurrent dementia. Given its potential for treatment and reversibility, timely diagnosis and intervention for DAVF are paramount.

Case report: Association between PTEN-gene variant and an aggressive case of multiple dAVFs.

Introduction: Mutations of the phosphatase and tensin homolog (PTEN) gene have been associated with a spectrum of disorders called PTEN hamartoma tumor syndrome, which predisposes the individual to develop various types of tumors and vascular anomalies. Its phenotypic spectrum includes Cowden syndrome (CS), Bannayan-Riley-Ruvalcaba syndrome (BRRS), Proteus syndrome, autism spectrum disorders (ASD), some sporadic cancers, Lhermitte-Duclos disease (LDD), and various types of associated vascular anomalies. Clinical presentation: A previously healthy 27-year-old woman was experiencing visual scintillating scotomas and mild chronic headaches for the past 2 years. The initial computed tomographic (CT) and magnetic resonance imaging (MRI) scans did not reveal any abnormalities, but the possibility of pseudotumor cerebri was considered. Furthermore, a cerebral angiogram showed a posterior fossa dural arteriovenous fistula (dAVF), which was initially treated through embolization. However, in spite of proper treatment, this patient experienced multiple recurrent dAVFs in different locations, requiring multiple embolizations and surgeries. Despite exhibiting altered cerebral perfusion and hemodynamics, the patient did not display any significant symptoms until she experienced a sudden stroke resulting from deep venous thrombosis, which was not associated with any medical procedures or medication use. A comprehensive analysis was performed due to the aggressive nature of the dAVFs. Surprisingly, exome sequencing of a blood sample revealed a PTEN gene variant in chromosome 10, indicative of Cowden syndrome. However, no tumors or other vascular lesions were detected in other systems that would constitute Cowden syndrome. Conclusion: The rapid formation of multiple and complex dAVFs, coupled with not meeting the criteria for any other PTEN-related syndrome, unequivocally leads to the presentation of a novel phenotype of the PTEN germline variant.

Dural Arteriovenous Fistula in Neuro-Behçet's Disease: Association or Chance?

Behçet's disease (BD) is a multisystemic vasculitis disorder. Neuro-Behçet's disease (NBD) is a set of neurologic symptoms imputable to an underlying Behçet vasculitis. Among the wide range of vascular abnormalities secondary to BD, a dural arteriovenous fistula (dAVF) is not classically described. Whether a dAVF is associated with BD or dAVF is a chance occurrence is still a matter of debate. Herein, we describe an NBD case of a 48-year-old male, presenting with headache and fever, where a dAVF was seen on imaging. He was treated with prednisolone and colchicine, followed by the surgical resection for dAVF. Then, we discuss the possible association between BD and dAVF based on the latest literature.

Comparison between endovascular and surgical treatment of spinal dural arteriovenous fistulas: a single-center cohort and systematic review.

OBJECTIVE: With recent advancements in minimally invasive techniques, endovascular embolization has gained popularity as a first-line treatment option for spinal dural arteriovenous fistulas (sDAVFs). The authors present their institution's case series of sDAVFs treated endovascularly and surgically, and they performed a systemic review to assess the outcomes of both modalities of treatment. METHODS: The authors conducted a retrospective observational study of 24 consecutive patients with sDAVFs treated between 2013 and 2023. The primary outcome was the rate of occlusion, which was compared between the surgically and endovascularly treated sDAVFs. They also conducted a systemic review of all the literature comparing outcomes of endovascular and surgical treatment of sDAVFs. RESULTS: A total of 24 patients with 24 sDAVFs were studied. The mean patient age was 63.8 ± 15.5 years, and the majority of patients were male (n = 19, 79.2%). Of the 24 patients, 8 (33.3%) received endovascular treatment, 15 (62.5%) received surgical treatment, and 1 (4.2%) patient received both. Complete occlusion at first follow-up was higher in the surgical cohort but did not achieve statistical significance (66.7% vs 25%, p = 0.52). Recurrence was higher in the endovascular cohort (37.5% vs 13.3%, p = 0.3), while the rate of postprocedural complications was higher in the surgical cohort (13.3% vs 0%, p = 0.52); however, neither of these differences was statistically significant. CONCLUSIONS: Endovascular embolization in the management of sDAVFs is an alternative treatment to surgery, whose long-term efficacy is still under investigation. These findings suggest overall comparable outcomes between endovascular and open surgical treatment of sDAVFs. Future studies are needed to determine the role of endovascular embolization in the overall management of sDAVFs.

Long-term treatment outcomes and natural course of low-grade intracranial dural arteriovenous fistulas.

OBJECTIVE: In contrast to high-grade dural arteriovenous fistula (dAVF), low-grade dAVF is mainly associated with tinnitus and carries a low risk of morbidity and mortality. It remains unclear whether the benefits of active interventions outweigh the associated risk of complications in low-grade dAVF. METHODS: The authors conducted a retrospective single-center study that included all consecutive patients diagnosed with an intracranial low-grade dAVF (Cognard type I and IIa) during 2012-2022 with DSA. The authors analyzed symptom relief, symptomatic angiographic cure, treatment-related complications, risk for intracerebral hemorrhage (ICH), and mortality. All patients were followed up until the end of 2022. RESULTS: A total of 81 patients were diagnosed with a low-grade dAVF. Of these, 48 patients (59%) underwent treatment (all primary endovascular treatments), and 33 patients (41%) did not undergo treatment. Nine patients (19%) underwent retreatments. Angiographic follow-up was performed after median (IQR) 7.7 (6.1-24.1) months by means of DSA (mean 15.0, median 6.4 months, range 4.5-83.4 months) or MRA (mean 29.3, median 24.7 months, range 5.9-62.1 months). Symptom control was achieved in 98% of treated patients after final treatment. On final angiographic follow-up, 73% of patients had a completely occluded dAVF. There were 2 treatment-related complications resulting in 1 transient (2%) and 1 permanent (2%) neurological complication. One patient showed recurrence and progression of a completely occluded low-grade dAVF to an asymptomatic high-grade dAVF. No cases of ICH- or dAVF-related mortality were found in either treated patients (median [IQR] follow-up 5.1 [2.0-6.8] years) or untreated patients (median [IQR] follow-up 5.7 [3.2-9.0] years). CONCLUSIONS: Treatment of low-grade dAVF provides a high rate of symptom relief with small risks for complications with neurological sequela. The risks of ICH and mortality in patients with untreated low-grade dAVF are minimal. Symptoms may not reveal high-grade recurrence, and radiological follow-up may be warranted in selected patients with treated low-grade dAVF. An optimal radiographic follow-up regimen should be developed by a future prospective multicenter registry.

Retrograde Flow Into the Internal Jugular Vein in a Hemodialysis Patient Mimicking Dural Arteriovenous Fistula: A Case Report.

Arterial spin labeling (ASL) and three-dimensional (3D) time-of-flight (TOF) magnetic resonance angiography (MRA) are sensitive tools to detect dural arteriovenous fistula (DAVF), but hyperintensity in these images is also caused by jugular venous reflux. We present a case of a patient with renal failure on hemodialysis with retrograde flow into the internal jugular vein (IJV) mimicking DAVF. A 74-year-old man with a radial arteriovenous fistula for hemodialysis experienced transient dizziness. The TOF MRA and ASL revealed high signal intensity, suggesting the presence of a DAVF in the left transverse and sigmoid sinuses and the IJV. Digital subtraction angiography (DSA) revealed no evidence of a DAVF but showed retrograde flow into the IJV via his radial shunt. In hemodialysis patients, a high-flow shunt can cause fast retrograde flow into the dural sinuses and might lead to intracranial hypertension. The ASL images are useful for early detection and careful observation.

Hormone Concentration Measurement in Intracranial Dural Arteriovenous Fistulae.

OBJECTIVE: Intracranial dural arteriovenous fistulae (DAVFs) represent a subset of cerebral vascular malformations associated with significant morbidity and mortality. In Japan, DAVF exhibits sex-based differences in anatomical distribution, with female predominance in the cavernous sinus (CS) and male predominance in the transverse sinus (TS). Nevertheless, the pathophysiology of DAVF is not fully understood, and hormonal influences are hypothesized to play a role in its development. This study aimed to investigate changes in the concentrations of sex steroid hormones between intracranial and peripheral sampling sites in patients with CS- and TS-DAVF. METHODS: We recruited 19 patients with CS-DAVF (n = 12) and TS-DAVF (n = 7) in this study. Blood hormone measurements were obtained from peripheral and jugular bulb samples during endovascular intervention. Hormone concentrations were analyzed using enzyme-linked immunosorbent assay kits, and statistical analyses were performed. RESULTS: Our study revealed a higher prevalence of CS-DAVF in females and TS-DAVF in males, which is consistent with previous studies. Estradiol concentration was significantly lower in the jugular bulb compared with in the periphery in both patients with CS- and TS-DAVF. This decrease in estradiol was observed irrespective of the patient's sex and independent of follicle-stimulating hormone levels. CONCLUSIONS: These findings indicate a local decrease in estradiol levels within the intracranial vasculature of patients with DAVF. This suggests a potential multifactorial role of estradiol in the pathomechanism of DAVFs, warranting further investigation to understand its influence on DAVF formation and potential targeted therapies, thereby enhancing patient outcomes.

Cerebral dural arteriovenous fistulas in patients with PTEN-related hamartoma tumor syndrome.

Central nervous system (CNS) dural arteriovenous fistulas (DAVF) have been reported in PTEN-related hamartoma tumor syndrome (PHTS). However, PHTS-associated DAVF remain an underexplored field of the PHTS clinical landscape. Here, we studied cases with a PTEN pathogenic variant identified between 2007 and 2020 in our laboratory (n = 58), and for whom brain imaging was available. Two patients had DAVF (2/58, 3.4%), both presenting at advanced stages: a 34-year-old man with a left lateral sinus DAVF at immediate risk of hemorrhage, and a 21-year-old woman with acute intracranial hypertension due to a torcular DAVF. Interestingly, not all patients had 3D TOF/MRA, the optimal sequences to detect DAVF. Early diagnosis of DAVF can be lifesaving, and is easier to treat compared to developed, proliferative, or complex lesions. As a result, one should consider brain MRI with 3D TOF/MRA in PHTS patients at genetic diagnosis, with subsequent surveillance on a case-by-case basis.

An isolated Sinus Intracranial Dural Arteriovenous Fistula with Unusual Drainage Pattern.

Isolated sinus dural arteriovenous fistulas (DAVFs) involve a dural sinus with occlusion on both sides of the diseased sinus segment. Because of venous drainage refluxing from the isolated sinus into the cortical veins, all isolated sinus DAVFs are Borden type III or Cognard type Ⅲ/Ⅳ. Venous drainage typically involves temporo-occipital cortical veins or the superior petrosal sinus and tributaries of the petrosal vein. However, drainage veins involving the perimedullary venous system are extremely rare. Here, we present a case of Cognard type V isolated sinus DAVF successfully treated with balloon catheter and Onyx.

Onyx Cast Migration to the Right Ventricle Following Trans-arterial Embolization of a Cranial Dural Arteriovenous Fistula.

We report a case of a middle-aged man who presented to the cardiology clinic with an incidental finding of a hyperdense lesion in the right ventricle (RV). He is an ex-smoker and had a low-dose CT chest as part of a screening program for early lung malignancy. His medical history included a cerebellar hemorrhage in 2021 due to a ruptured dural arteriovenous fistula (dAVF). He was treated as an emergency with trans-arterial embolization using Onyx liquid embolic material (Medtronic, Fridley, MN). The high-flow dAVF embolization was straightforward, with Onyx filling the arteriovenous (AV) shunt and draining the vein. The patient made a good recovery, and routine cerebral digital subtraction angiography (DSA) at three months confirmed the occlusion of the dAVF. Cardiac migration of liquid embolic material used to treat AV shunts is uncommon and probably underreported as it can be asymptomatic, as in this case. Cardiac embolization should be suspected in patients with dense material in the RV and prior treatment with trans-arterial embolization.

Spinal Dural Arteriovenous Fistulas: A Retrospective Analysis of Prognostic Factors and Long-Term Clinical Outcomes in the Light of the Recent Diagnostic and Technical Refinements.

Spinal dural arteriovenous fistulas (dAVFs) are abnormal connections between the meningeal branches of segmental arteries and a radiculomedullary veins that result in a progressive myelopathy thanks to perimedullary coronal venous plexus congestion. Usually, dAVFs show nonspecific symptoms, thus leading to late clinical suspicion and a difficult MRI diagnosis.Several authors have tried to identify prognostic factors before treatment, but published studies results are often inconsistent and sometimes contradictory.In this study, we reviewed our recent experience of 30 dAVF patients where we collected all demographic, clinical and angioarchitectural features as well as radiological and treatment-related characteristics. The thoracic spine was the most common location, constituting 53.3% of cases, followed by the lumbar roots, comprising 30%. About 83% of patients showed motor deficits, urinary disturbances were present in 70%, and bowel symptoms in 50%.We treated 86.7% of patients with microsurgery and 13.3% with endovascular occlusion with a mean interval between clinical onset and intervention of 10.8 ± 14.2 months.A significant clinical improvement was observed at follow-up in 80% of patients, with a significant reduction in mean G-score, U-score and F-score at a mean follow-up of 105.89 ± 191.9 months.However, none among the principal demographic, clinical and radiological characteristics showed significant prognostic value to the clinical improvement observed at follow-up.

Exploring Spinal Subarachnoid Hemorrhage: A Neurosurgical Case Series.

Spinal subarachnoid hemorrhage (SSAH) is a rare condition that can cause spinal cord or nerve root compression and permanent neurologic damage. The reported etiologies include trauma, vascular malformations or aneurysms, coagulopathies, neoplasms, autoimmune disease, and spontaneous hemorrhage. If there is evidence of neurologic deterioration, it is commonly managed as a surgical emergency, but cases of conservative management have also been reported. In this case series, we present three patients who suffered from SSAH. The first was a spontaneous cervical SSAH that occurred following cardiac catheterization, the second was a spontaneous thoracolumbar SSAH in a patient with a known history of coagulopathy, and the third was a thoracolumbar SSAH that was caused by a dural arteriovenous fistula (dAVF). All three patients exhibited neurologic deficits and thus underwent emergent decompression and hematoma evacuation. The patient with the dAVF also required open ligation of the fistula. Following surgical intervention, all three patients regained at least partial neurologic function, but one patient developed symptomatic arachnoid cysts that required further intervention. The presented case series highlights the importance and time-sensitivity of surgical decompression in patients experiencing neurologic deficits from SSAH. These cases underscore the urgency of timely neurosurgical intervention to mitigate neurologic impairment and add insights to the existing literature on this rare condition.

Intracranial Non-Sinus-Type Dural Arteriovenous Fistulas Could Be Curable by Transarterial Embolization or Transvenous Embolization with Liquid Embolic Material.

Objective: Recently, the occlusion rate of transarterial embolization (TAE) for intracranial non-sinus-type dural arteriovenous fistulas (NSDAVFs) has improved after ONYX was introduced. Additionally, when TAE for NSDAVF is unsuccessful, transvenous embolization (TVE) has become available as an alternative treatment. We investigated the factor for the favorable occlusion rate of endovascular treatment for NSDAVF at our institutions. Methods: Two hundred and twenty-seven patients with intracranial dural arteriovenous fistulas (DAVFs) were treated at our institutions between September 2014 and October 2022. The patients diagnosed with NSDAVF in all DAVFs who underwent endovascular treatment were included. The clinical characteristics, angiographical outcomes, and clinical outcomes of patients who underwent endovascular treatment were evaluated. Results: Thirty-eight patients had intracranial NSDAVF (tentorial: 23 cases, parasagittal-convexity: 7, anterior cranial fossa: 6, middle cranial fossa: 2). Our participants' mean age was 64.8 ± 11.3 years, and 31 (81.6%) of them were males. Patients' symptoms were as follows: asymptomatic (24), hemorrhage (10), tinnitus (3), and trigeminal neuralgia (1). TAE and TVE were performed on 35 and 3 patients, respectively. The rate of immediate angiographical occlusion was 84.2% (32/38). The follow-up angiographical occlusion rate in 6 months was 88.5% (31/35). Complications occurred in three cases. There was no morbidity or mortality after 30 days. Conclusion: TAE using the combination of the new microcatheter and microguidewire and TVE in the case of difficult or failed TAE for NSDAVF could achieve high success rates and safety.

Endovascular Treatment of Dural Arteriovenous Fistulas in a Medium-Sized Scandinavian Neurovascular Center.

BACKGROUND: Endovascular treatment is the primary treatment modality for dural arteriovenous fistulas (DAVFs). We performed a retrospective analysis of DAVFs treated in our hospital to determine if high-quality endovascular treatment can be provided in a medium-volume vascular center. METHODS: From 2007 to 2021, 69 DAVF treatments were undertaken in our hospital. Of these DAVFs, 55 were endovascular, 11 were open surgical procedures, and 3 were Gamma Knife treatments. Of the endovascular treatments, 10 (18.2%) were in ruptured DAVFs. The most common location of endovascularly treated DAVFs was at the transverse/sigmoid sinus (32.7%) and at the cavernous sinus (25.5%). Of the endovascularly treated DAVFs, 38.2% were low-grade fistulas (Cognard I/IIa), whereas 61.8% were high-grade fistulas (Cognard ≥IIb). 58.2% of fistulas were treated transarterially. DAVFs located in the cavernous sinus were treated using coils alone, whereas most other DAVFs were treated with liquid embolics alone or in combination with coils. RESULTS: Complete or near-complete cure was achieved in 74.5% of treatments, whereas 18.2% of treatments resulted in downgrading of the fistula. Twelve patients were retreated once (9 endovascularly) and 1 patient was retreated twice. After retreatment, complete or near-complete cure was achieved in 86.4% of patients. 72.7% of treatments were performed without any remnant or retreatment. There were no procedure-related deaths. One patient experienced a complication resulting in permanent neurologic deficits. Seven other complications (12.7%) were recorded, all asymptomatic or causing only temporary symptoms. CONCLUSIONS: Based on our findings, we conclude that high-quality treatment of DAVFs can be provided in a medium-volume vascular center.