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Dural arteriovenous fistula represents a rare cause of secondary Trigeminal Neuralgia. To date, there have been 18 reported cases of successful treatment of trigeminal neuralgia with endovascular embolization. Here we describe a unique case of a 51-year-old man with right-sided petrous ridge dural arteriovenous fistula causing ipsilateral V1 pain and cranial autonomic dysfunction. Cerebral angiography confirmed a Cognard type 3 right-sided tentorial dAVF supplied by an enlarged meningo-hypophpyseal vessel from the extradural ICA and by the middle meningeal artery. The fistula drains towards the midline via deep cerebral veins, the internal cerebral vein and the straight sinus with likely variceal compression of the right trigeminal nerve root entry zone. Transarterial Onyx embolization resulted in complete obliteration of the dural arteriovenous fistula with immediate resolution of facial pain and autonomic symptoms. After 24 months, the patient remains symptom-free and has no radiographic or clinical evidence of fistula recurrence. To date, there have been no other cases reported of trigeminal neuralgia with cranial autonomic symptoms or SUNCT syndrome due to a dural arteriovenous fistula. The presence of cranial autonomic symptoms with trigeminal neuralgia or a diagnosis of SUNCT should not deter endovascular treatments of dural arteriovenous fistulas or treatment of other compressive vascular lesions.

A dural arteriovenous fistula (DAVF) is a dural-based shunt between meningeal arteries and meningeal veins, sinuses and/or cortical veins; they have been classified and named according to the location and the flow pattern. Petrous apex DAVFs are located where the petrosal vein penetrates the dura mater into the superior petrosal sinus; there are only few cases reported in the literature, they can show an aggressive behaviour (subarachnoid haemorrhage, severe brainstem oedema) with a high mortality rate. The described case is, to the best of our knowledge, the first case of a DAVF presenting with symptoms mimicking idiopathic normal pressure hydrocephalus. After worsening of gait impairment, memory loss and urinary incontinence an urgent CT of the brain showed hydrocephalus and a hyperdense mass in the pineal region mimicking a pineal tumour; an emergent digital subtraction angiogram showed a left petrous apex Borden type III DAVF. A transvenous embolisation was performed obtaining a complete obliteration., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

In recent years, with the current access in techniques, studies have significantly advanced the knowledge on meningeal immunity, revealing that the central nervous system (CNS) border acts as an immune landscape. The latest concept of meningeal immune system is a tertiary structure, which is a comprehensive overview of the meningeal immune system from macro to micro. We comprehensively reviewed recent advances in meningeal immunity, particularly the new understanding of the dural sinus and meningeal lymphatics. Moreover, based on the clues from the meningeal immunity, new insights were proposed into the dural arteriovenous fistula (DAVF) pathology, aiming to provide novel ideas for DAVF understanding., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Tu, Peng, Song, Ma and Zhang.)

Background and Purpose: Type I and IIa dural arteriovenous fistulas (DAVFs) have a low hemorrhagic risk, but are often the cause of debilitating tinnitus that requires treatment. While Onyx® and PHIL™ (Precipitating hydrophobic injectable liquid) transarterial embolization represent the first endovascular option, there are occasional cases where performing angioplasty and stenting of the affected sinus may lead to satisfactory results., Material and Methods: We retrospectively analyzed four consecutive cases of patients with DAVF-induced pulsatile tinnitus secondary to type I and II DAVFs who were treated with angioplasty and stenting of the sinus only. All the patients had clinical and radiological long-term follow-up., Results: We noticed a significant radiological and clinical improvement in all the cases. Of the patients two were completely cured at follow-up with eradication of the neurological symptoms as well as the fistula, one was retreated with Onyx® for a very small residual shunt despite having no more tinnitus, and one showed improvement in venous drainage (from type IIa+b to type I fistula) without resolution of the fistula., Conclusion: In cases of type I and II DAVFs associated with sinus stenosis, angioplasty and stenting alone seem to be safe and effective. This treatment probably compresses the venules within the sinus walls, promoting thrombosis of the shunts thus solving the underlying cause of the fistula.

Objective: Spinal dural arteriovenous fistula (DAVF) occurs at any spinal level, but the clinical characteristics of lumbosacral DAVF have not been well documented. The purpose of this study was to evaluate clinical characteristics of lumbosacral DAVF and compare these features with those in thoracic DAVF., Methods: Twenty-five consecutive patients with 16 thoracic and 9 lumbosacral DAVFs were included (mean age, 63.9 years; 20 men). All patients presented with progressive myelopathy. Preoperative and postoperative neurologic deficits were compared between thoracic and lumbosacral DAVF groups. Using magnetic resonance imaging, the extent of T2 high-intensity areas and signal flow voids were documented. Follow-up after surgical interventions ranged from 6 to 96 months (mean, 38.1 months)., Results: Preoperatively, patients suffering lumbosacral DAVF tended to be more severely disabled compared with thoracic DAVF patients. Lumbosacral DAVF patients exhibited diminished patellar (P = 0.04) and Achilles tendon reflexes (P < 0.01), while most thoracic DAVF patients exhibited hyperreflexia. In magnetic resonance imaging, signal flow voids around the spinal cord were evident in only 4 of 9 lumbosacral DAVF patients (P = 0.012). Rather, a serpentine signal flow void of the filum terminale was a hallmark of lumbosacral DAVFs to distinguish them from thoracic DAVFs. In the lumbosacral DAVF group, postoperative improvements were significantly better in micturition function (P = 0.02)., Conclusions: In lumbosacral DAVF, postoperative micturition function recovery was superior to thoracic DAVF. Intradural lumbar signal flow void is indicative of lumbosacral DAVF. For appropriate management, it is important to recognize these differences between lumbosacral and thoracic DAVF., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Purpose: Computed tomography angiography (CTA) and magnetic resonance imaging/angiography (MRI/MRA) are used for the diagnosis of intracranial dural arteriovenous fistulas (DAVFs). The purpose of this study was to compare the diagnostic accuracy of CTA and magnetic resonance imaging/angiography (MRI/MRA) for detection of cortical venous reflux (CVR) in intracranial DAVFs., Methods: The records of patients with angiography-confirmed intracranial DAVFs who also received CTA and MRI/MRA from January 2008 to July 2016 were reviewed. CTA and MRI/MRA were reviewed for signs of CVR, and the diagnostic accuracy of individual signs was evaluated by receiver operating curve (ROC) analysis., Results: A total 108 patients were included in this study. CTA signs of CVR included abnormal dilatation, early enhancement, and the presence of a medullary or pial vein. MRI/MRA signs of CVR included abnormal dilatation, early enhancement, flow-related enhancement, flow void, and medullary or pial venous collaterals. The sensitivity of individual CTA signs ranged from 62 to 96%, and specificities from 79 to 94%. The sensitivities of individual MRI/MRA signs ranged from 58 to 83%, and specificities from 77 to 93%. The area under ROC curve (AUC) of CTA and MRI/MRA were 0.91 and 0.87, respectively (P = 0.04 in direct comparison). In subgroup analysis, CTA had better diagnostic accuracy for higher grade disease (P = 0.05) and non-aggressive manifestation (P = 0.04)., Conclusions: Both CTA and MRI/MRA have good diagnostic accuracy for detection of CVR in patients with intracranial DAVFs. There is modest evidence that CTA is better than MRI/MRA.

Caroticocavernous fistula or cavernous sinus dural arteriovenous fistula (CS-DAVF) has presented various treatment challenges over many years. This paper outlines these challenges in a review of the literature, and attempts to address them by analyzing the anatomical and hemodynamic characteristics of 32 consecutive patients with CS-DAVF treated between 2007 and 2016, in doing so proposing novel strategies for safe access and treatment of CS-DAVF., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Acquired arteriovenous malformations, such as is the case with dural arteriovenous fistulae (DAVF), are the consequence of a pathological new arterial ingrowth into venous spaces that reaches directly the venous lumen, without interposition of a capillary network, thereby creating an AV-shunt.The following concise text will provide elements in regards to diagnosis, indication for treatment discussion and choice of endovascular treatment (EVT) method.

We present a case of a patient with rapid loss of motor strength in his lower extremities. He became bedridden with bowel and bladder incontinence, and developed saddle anesthesia. MRI of the lumbar spine showed edema in the conus medullaris and multiple flow voids within the spinal canal. A spinal angiogram showed a dorsal Type I spinal AVF. This was treated successfully with Onyx 18 (eV3, Irvine, CA). The patient showed rapid post-procedure improvement, and at discharge from the hospital to a rehabilitation center he was fully ambulatory. At 3-year follow-up, the patient was found to ambulate without difficulty. He also had improved saddle anesthesia, and he was voiding spontaneously. There was no evidence of flow voids on repeat MRI of the lumbar spine. The video can be found here: http://youtu.be/SDYNIGNQIW8 .

Background and Purpose: Cavernous sinuses and draining dural sinuses or veins are often visualized on 3D TOF MRA images in patients with dural arteriovenous fistulas involving the CS. Flow signals may be seen in the jugular vein and dural sinuses at the skull base on MRA images in healthy participants, however, because of reverse flow. Our purpose was to investigate the prevalence of flow signals in the pterygoid plexus and CS on 3T MRA images in a cohort of participants without DAVFs., Materials and Methods: Two radiologists evaluated the flow signals of the PP and CS on 3T MRA images obtained from 406 consecutive participants by using a 5-point scale. In addition, the findings on 3T MRA images were compared with those on digital subtraction angiography images in an additional 171 participants who underwent both examinations., Results: The radiologists identified 110 participants (27.1%; 108 left, 10 right, 8 bilateral) with evidence of flow signals in the PP alone (n = 67) or in both the PP and CS (n = 43). Flow signals were significantly more common in the left PP than in the right PP. In 171 patients who underwent both MRA and DSA, the MRA images showed flow signals in the PP with or without CS in 60 patients; no DAVFs were identified on DSA in any of these patients., Conclusions: Flow signals are frequently seen in the left PP on 3T MRA images in healthy participants. This finding may be the result of flow reversal and should not be considered to indicate occult DAVF.

Purpose: The cavernous sinus (CS) region is a common region of dural arteriovenous fistula (DAVF). Over time, treatment strategies are gradually changing. In this study, we present our center's experience in managing CS-DAVF over the past 20 years., Methods: Medical records of patients diagnosed with CS-DAVF between 2002 and 2021 were collected for analysis. Patients meeting the predefined inclusion and exclusion criteria were included. This study summarized and analyzed their clinical characteristics, CS-DAVF angioarchitecture, treatment strategies, and outcomes., Results: A total of 141 patients (mean age 55 years, 46 males) were included in this study. Ocular/orbital symptoms were the most frequently reported initial symptoms, with 84 (59.6%) patients experiencing these symptoms first. Presentation with ocular/orbital symptoms as the first symptom was associated with thrombosis of the inferior petrosal sinus (p = 0.032). Presentation with headache/dizziness and tinnitus/intracranial murmur as the first symptom was associated with sphenoparietal sinus/superficial middle cerebral vein drainage (p = 0.011). Among the patients, 131 (92.9%) patients received endovascular treatment, with 114 (87.0%) undergoing transvenous embolization. Onyx (92.4%) and coil (74.8%) were the most used embolic materials. 17 (13.0%) of the patients who received endovascular treatment suffered intraoperative or postoperative complications, and 11 (64.7%) patients fully recovered within 6 months after discharge., Conclusion: Ocular/orbital symptoms were the most common first symptom of CS-DAVF. The mode of venous drainage played a significant role in determining the first symptoms. Transvenous embolization using Onyx or a combination of Onyx and coils was the primary treatment modality., (© 2023. The Author(s) under exclusive licence to Belgian Neurological Society.)

Background: Intracranial dural arteriovenous fistulas (dAVFs) often present with pulsatile tinnitus, orbital congestion, and headache. Occasionally, they present with focal neurologic deficits, a dementia-like syndrome, hemorrhage, or ischemic infarction., Methods: This study is based on the case of a 71-year-old gentleman who presented with 6 months of progressive forgetfulness, inattention, and hypersomnolence. Four weeks prior to presentation, he developed symptoms of left-sided pain, numbness, and worsening weakness. Neurologic examination demonstrated hypersomnolence, a score of 30/38 on the Kokmen Short Test of Mental Status, and left hemiparesis. MRI brain revealed bilateral thalamic T2 hyperintensities with associated enhancement. MR venogram (MRV) showed a vascular malformation in the posterior fossa and occlusion of the straight sinus. Conventional cerebral angiogram confirmed a tentorial dAVF. The dAVF was definitively treated with transarterial embolization, followed by clip ligation of the arterialized draining vein. Twelve weeks later, there was clinical resolution of left hemiparesis and improvement in cognitive status. MRI revealed complete resolution of the thalamic hyperintensities. MRV demonstrated recanalization of the straight sinus., Results: Intracranial dAVFs are uncommon but potentially life-threatening acquired vascular malformations. The initiating factor is venous hypertension, causing retrograde flow, venous congestion, ischemia, and sometimes infarction. The spectrum of clinical manifestations in dAVFs reflects the degree of venous congestion present. If retrograde venous flow is surgically obliterated, then venous hypertension may be reversible. Bilateral thalamic venous congestion can present as a thalamic dementia., Conclusion: We conclude that intracranial dAVFs with thalamic venous congestion should be considered in the diagnostic differential for patients who present with subacute cognitive decline and T2 hyperintense thalamic signal change.

Objectives: DSA is currently the criterion standard for the assessment of dural arteriovenous fistulas (dAVF). Recently, evolving MRA techniques have emerged as a non-invasive alternative. The aim of this study is to assess the value of 3 T MRI in detecting and describing dAVF and to determine whether MRI can replace DSA as diagnostic procedure., Methods: A total of 19 patients with dAVF and 19 without dAVF underwent the same MRI protocol, including 3D time-of-flight MRA and time-resolved contrast-enhanced MRA. The images were evaluated retrospectively by three independent readers with different levels of experience blinded to clinical information. The readers assessed the presence, the site, the venous drainage and the feeders of dAVF. Sensitivity, specificity, accuracy, intertechnique and interobserver agreements were calculated., Results: DAVF can be detected with high sensitivity, specificity and accuracy by experienced and also by less experienced readers. However, MRI has limitations when used for grading and evaluation of the angioarchitecture of the dAVF. Different experience, the limited resolution of MRI and its inability to selectively display arteries were the reasons for these limitations., Conclusions: With MRI dAVF can be detected reliably. Nevertheless, at present MRI can not fully replace DSA, especially for treatment planning.

We describe the case of a patient who presented with ocular symptoms (chemosis, proptosis, increased intra-ocular pressure, impaired visual acuity) eight months after the transvenous coil occlusion of an ipsilateral sigmoid sinus dural arteriovenous fistula (dAVF). Digital substraction angiography revealed a partial occlusion of the left sigmoid sinus with coils. Since the connection from the sigmoid sinus to the internal jugular vein was obliterated by coils without interrupting the arteriovenous shunt, the venous drainage was redirected into the inferior petrosal sinus, the cavernous sinus and the superior ophthalmic vein. The transjugular access to the inferior petrosal sinus was obstructed by a large coil mass in the jugular bulb. Several attempts to remove these coils with an Alligator retrieval device and a goose neck snare failed. The coil mass was withdrawn via a direct access to the internal jugular vein using flexible cysto-urethroscopy grasping forceps, an urological device designed for the removal of kidney stones. After establishing anterograde drainage, the now accessible inferior petrosal sinus was occluded with fibered coils and the dAVF was completely obliterated. The ocular symptoms resolved within ten days.

Objective: The precise mechanisms responsible for the development and growth of dural arteriovenous fistula (DAVF) remain unclear, but it has been hypothesized that vascular endothelial growth factor (VEGF) might be involved in the pathogenesis. The aim of this study was to examine the expression of VEGF in the rat DAVF model., Methods: Forty-five Sprague-Dawley rats were used in two experiments. In Experiment 1 (n = 20, including sham-operated controls), VEGF expression was analysed by Western blots in three different rat DAVF models: model I: common carotid artery-external jugular vein (CCA-EJV) anastomosis (n = 5); model II: sagittal sinus thrombosis and bipolar coagulation of the vein draining the transverse sinus (n = 5); model III: CCA-EJV anastomosis and bipolar coagulation of the vein draining the transverse sinus and sagittal sinus thrombosis to induce venous hypertension (n = 5). Based on the results of Experiment 1, Western blots were performed at weekly intervals 1, 2 and 3 weeks in Experiment 2 following induction of venous hypertension in model III (n = 5 at each time point and n = 5 sham controls); in addition, VEGF expression was immunohistochemically examined in the dura and the brain near the occluded sinus in five model III animals after 1 week., Results: In Experiment 1, Western blot analysis showed barely detectable bands with molecular weights of 45 kD, corresponding to VEGF, in the sham group, but the highest level of VEGF was induced in model III, followed by models I and II (model III>model I>model II). In Experiment 2, the expression of VEGF peaked 1 week after induction of venous hypertension in model III, decreasing in a linear fashion over 2 and 3 weeks (week 1>weeks 2 and 3). The expression of immunoreactive VEGF was restricted in the connective tissue and the endothelial layer of the dura matter, cerebral cortical tissue and neurons of the basal ganglia., Conclusion: Our results strongly suggest a possible contribution of an angiogenic factor to the growth of DAVF. Venous ischemia by venous hypertension might be a mechanism for inducing up-regulation of angiogenic factor expression.

A 75-year-old woman with a dural arteriovenous fistula (DAVF) presented with progressive cognitive impairment including amnesia and alexia. Neuroradiological studies showed a relatively confined DAVF lesion in the left temporal lobe. The patient did not have a history of trauma and did not complain of headache or tinnitus. Amnesia and alexia dramatically improved upon treatment of the DAVF, and this was associated with attenuation of an abnormal MRI signal in the left temporal lobe. The results suggest that gradually impaired cerebral circulation due to focal venous hypertensive encephalopathy localized to the left temporal lobe and resulting from a DAVF could be involved in slowly progressive amnesia and alexia. The case also shows that an intracranial DAVF may present as a variety of neurological symptoms, depending on its localization, size and clinical stage.

Background: Transarterial embolization (TAE) is generally the endovascular treatment of choice for tentorial dural arteriovenous fistula (dAVF). Although flow control of the feeder vessel has been reported to achieve complete shunt blockade, flow control in the absence of ischemia tolerance of internal carotid artery as a feeder has not been reported. We present a case in which treatment by Onyx TAE with intermittent flow control of the meningohypophyseal trunk as the feeder was successful for a tentorial dAVF presenting with myelopathy without tolerance of ischemia., Methods: The intermittent flow control is presented for a tentorial dAVF presenting with myelopathy without tolerance for ischemia. An inflation of the balloon in the internal carotid artery was set for 5 minutes, and the Onyx injection was repeated at intervals of at least 2 minutes. Injections and pauses were repeated to allow Onyx to reach the shunt pouch., Results: The patient underwent successful TAE with intermittent flow control for a tentorial dAVF presenting with myelopathy. The disappearance of the shunt was confirmed with gait disturbance resolution postoperatively., Conclusions: Intermittent flow control of the meningohypophyseal trunk using a balloon may be safe and effective for cases showing no tolerance for ischemia., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Dural arteriovenous fistula (DAVF) represents a pathological group of intracranial shunts arising from the dural artery to venous sinus and veins. Childhood-onset DAVF is generally considered to be poor in prognosis, whereas only limited information is available for the onset and long-term outcomes. We herein report a Japanese girl with trisomy 21, large ventricular septal defects, and pulmonary vein stenosis, for which a transcatheter stent had been placed after birth. At age 6 years, she developed bacterial meningitis due to S. pneumoniae, leading to the diagnosis of venous sinus thrombosis and multiple intracranial shunts. Cerebral angiography identified multiple shunts arising from the middle meningeal arteries to the superior sagittal sinus and a concurrent reflux to cortical vein. Endovascular embolization successfully occluded the shunts without neurovascular complications over 24 months. This report first demonstrates the favorable outcome of DAVF in a pediatric patient with trisomy 21 after the catheter intervention. For children at a risk for intracranial thrombosis, preemptive neurovascular evaluation and transcatheter intervention provide a chance of early diagnosis of DAVF to improve their survival and neurologic outcome., (Copyright © 2024 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: The Onyx™ Liquid Embolic System is a non-adhesive liquid embolic agent, which has been proved by the US FDA for embolization of lesions in the peripheral and neurovasculature since 2005. We reported a case of ischemic optic neuropathy after using Onyx-18 to embolize the anterior ethmoid arteries that feeding dural arteriovenous fistulas (DAVF)., Case Presentation: A 57-year-old Asian male presented with anterior cranial fossa DAVF underwent embolotherapy by delivering Onyx-18 through a microcatheter into the anterior ethmoid arteries under angiography guidance. The interventional procedure was successful and no clear evidence was found pointing to untargeted occlusive embolus. But after the surgery the patient experienced delayed painless vision loss in the right eye (RE). The fundoscopy showed unilateral papilledema with pale optic disc in RE, accompanied by significant edema and thickening in the retinal nerve fiber layer (RNFL) of macula. The fundus fluorescence angiography showed that most of the optic disc in RE had postponed or absent fluorescence filling. Visual evoked potential (VEP) confirmed that the amplitude of the P100 component was decreased in RE without significant prolongation of the latency. The patient was diagnosed with anterior ischemic optic neuropathy, but immediate pulse steroid therapy failed to rescue his vision., Conclusion: Preoperative evaluation of the patient's hemodynamic status and fundus examination are essential for assessing the risk of ischemic ocular complications, and the non-adhesive liquid embolic agent Onyx-18 should be used cautiously during endovascular embolization of intracranial artery., (© 2024. The Author(s).)

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., (Copyright © 2024. Published by Elsevier Inc.)

Superior petrosal sinus (SPS) dural arteriovenous fistulas (DAVFs) are a commonly encountered type of tentorial DAVF that occasionally requires microsurgical intervention. This study aims to analyze the tributaries of the superior petrosal vein (SPV) observed intraoperatively, their association with clinical symptoms, and venous drainage patterns. This retrospective study reviewed 34 consecutive patients with SPS DAVFs who underwent microsurgical treatment at a single institution between 2014 and 2022. The cohort had a mean age of 52.8 ± 11.8 years, with 85.3% (29/34) being male. Predominant symptoms included venous hypertensive myelopathy (VHM) (55.9%) and intraparenchymal or subarachnoid hemorrhage (23.5%). Standard retrosigmoid approach was used to ligate 94.1% (32/34) of the lesions, resulting in immediate complete fistula occlusion for all patients. Supratentorial venous drainage patterns were associated with a higher incidence of intracranial hemorrhage and venous varix (P = 0.047). Infratentorial drainage patterns were more frequently linked with VHM-related symptoms (P<0.001). Patients presenting VHM-related symptoms showed a higher prevalence of drainage through the vein of the cerebellopontine fissure (VCPF) (P = 0.01), while those with intracranial hemorrhage symptoms exhibited a higher prevalence of pontotrigeminal vein (PTV) drainage (P = 0.033) in their DAVFs. Endovascular management of SPS DAVFs carries inherent risks. Surgical treatment via standard retrosigmoid craniotomy offers favorable clinical outcomes with high rates of cure. In cases featuring infratentorial venous drainage, the predominant arterialized tributary of the SPV was the VCPF, commonly associated with VHM-related symptoms. Conversely, in cases with supratentorial venous drainage, the predominant arterialized SPV tributary was the PTV, often associated with intracranial hemorrhage symptoms., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., (© 2024 The Authors. Clinical Genetics published by John Wiley & Sons Ltd.)

Purpose: To report a case of bilateral thalamic infarction (BTI) presenting as progressive thalamic dementia due to a midline tentorial dAVF (TdAVF) and to provide a systematic review of the literature., Methods: We performed a systematic literature review of previously reported cases of bi-thalamic signal changes due to dAVF considering population characteristics, clinical presentation, imaging findings, treatments, and outcomes., Results: We found 29 papers from 1985 until 2021 describing 35 cases of BTI dAVF-related. We analysed 36 cases comprehensive of our case report. The mean age was 58.7 years (range 38-79), 91.6% were males (n=33). Most cases presented with a subacute syndrome. In 86.1% (n=31) of cases a TdAVF was found; 58.3% (n=21) were type 2 Borden-Shucart fistulas, the remaining were mostly type 3. In 80.5% (n=29), a thrombosed sinus was identified. 33.3% of cases (n=12) had bi-thalamic haemorrhages. Endovascular treatment was performed in 83.3% of cases (n=30). A total of 75% (n=27) of cases had a good recovery., Conclusions: BTIs due to dAVFs may present with subacute symptoms overlapping with several differential diagnoses. Prompt identification at MRI, before venous drainage failure and bleeding, is crucial for a good prognosis., (© 2023. Fondazione Società Italiana di Neurologia.)

Dural arteriovenous fistula (DAVF) is a rare condition affecting approximately 1.5% of 1,000,000 individuals annually. It frequently occurs in the transsigmoid and cavernous sinuses. An isolated sigmoid sinus is extremely rare and is treated by performing transfemoral transvenous embolization along the opposite transverse sinus. A 69-year-old woman presented with asymptomatic Borden type III/Cognard type III DAVF involving an isolated sigmoid sinus. She underwent a staged operation in which a navigation system was used to expose the sigmoid sinus in the operating room before transferring the patient to the angio suite for transvenous embolization. Various modalities have been used to treat DAVF, including surgical disconnection, transarterial embolization, transvenous embolization, and stereotactic radiosurgery. However, treating DAVF cases where the affected sinus is isolated can be challenging because an easily accessible surgical route may not be available. In this case, direct sinus cannulation and transvenous embolization were the most effective treatments. [ABSTRACT FROM AUTHOR]

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., (Copyright © 2024 Elsevier Inc. All rights reserved.)

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., (Copyright © 2023. Published by Elsevier Inc.)

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.

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.

Background: Cognitive decline is a non-hemorrhagic, major complication of intracranial dural arteriovenous fistula (DAVF), thought to be primarily related to venous hypertension. However, imaging features to predict cognitive decline are scanty in the literature., Purpose: To evaluate functional connectivity (FC) changes of resting-state networks (RSNs) in DAVF before and after treatment and its relation to cognitive impairment., Study Type: Prospective., Subjects: DAVF subjects were screened for inclusion. Pre-embolization (N = 33, mean age 45.9 years, 29 males), 1 month post-embolization (N = 20, mean age 42.7 years, 19 males), and healthy controls (HC, N = 33, mean age 45.09 years, 27 males)., Field Strength/sequence: 3.0 T, resting-state functional magnetic resonance imaging (MRI), three-dimensional (3D) T1, T 2 fast spin echo (FSE), diffusion weighted imaging (DWI), susceptibility weighted imaging (SWI), fluid-attenuated inversion recovery, and time of flight., Assessment: Data quality assessment was performed. FC analysis was done using group independent component analysis (ICA) and seed to voxel analysis. Neuropsychology (NP) scores of patients were compared with HC and correlated with FC changes., Statistical Tests: Voxel-wise parametric T-statistics for F-test was executed in FC analysis (p-FDR corrected <0.05). NP scores between DAVF group and HC group were compared using one-way analysis of variance with post hoc Bonferroni correction (P < 0.05)., Results: Both RSNs analysis methods showed reduced FC at the precuneus-posterior cingulate cortex (PC-PCC) of default mode network (DMN), anterior cingulate cortex (ACC) of the salience network (SN), and possible compensatory increased connectivity at the frontoparietal (FPN) and dorsal attention (DAN) networks. DAVF with low NP scores showed reduced FC at DMN and SN and minimal to absent connectivity at FPN and DAN. At post-embolization 1-month follow-up, improvement in FC at PC-PCC of DMN and ACC of SN were noted., Data Conclusion: RS-fMRI in DAVF displayed FC changes that may be related to cognitive decline and its subsequent reversibility after treatment. FC changes at DMN, SN, FPN, and DAN were linked to cognitive decline and the corresponding NP scores., Level of Evidence: 2 TECHNICAL EFFICACY: Stage 2., (© 2021 International Society for Magnetic Resonance in Medicine.)

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., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose: The functional changes concerning memory deficits in dural arteriovenous fistula (dAVF) brain are inadequately understood. This study aimed to understand the functional connectivity alterations of brain regions widely affirmed for explicit and implicit memory functions in dAVF patients (DP) and look into the frequency effects of the altered functional networks., Methods: Resting-state functional magnetic resonance imaging (rsfMRI) analysis was done in the memory-associated regions of 30 DP and 30 healthy controls (HC). Frequency decomposition was used to determine potential frequency-dependent functional connectivity changes. They underwent neuropsychological tests and were correlated with changes in memory networks compared with HC., Results: The results showed weaker functional connectivity among the medial temporal lobe and sub-regions in DP suggestive of dysfunction of explicit and implicit memory functions, which corroborated with the positive correlation between memory scores and hippocampal-parahippocampal connectivity of DP, along with a significant group difference of lower memory and cognitive performance in DP assessed by neuropsychological tests. A frequency-dependent study of the altered rsFC revealed lower functional connectivity strength and impaired neural coupling manifested at some sub-band frequencies indicative of disturbed cortical rhythm in DP., Conclusion: This pilot study gives insights into significant intrinsic functional connectivity changes in the memory regions of the dAVF brain. The results may have clinical implications in the choice of interventional management of dAVF and can impact clinical decision making for realizable prevention of progressive memory impairment and irreversible brain damage in such patients., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Given the growing prevalence of initial endovascular treatment for type 1 spinal dural arteriovenous fistulae (dAVF), there are an increasing number of patients presenting with progressive symptoms related to recurrent previously embolized spinal dAVF. This study's goal was to identify demographic, clinical, and radiographic variables among patients who have failed embolization of type I spinal dAVF., Methods: A retrospective review of 24 consecutive surgeries for type I spinal dAVF performed by the senior author (A.D.L.) identified 5 patients who underwent open surgery for failed embolization. These 5 cases were reviewed for location of fistula, time from embolization to recurrence, preoperative functional status, fistulous point encountered at surgery, and clinical outcome of the patient at 3-month follow-up. A representative example case is reviewed in detail., Results: The median age at time of recurrence was 63 years (range 51-73 years). The median timing of embolization to recurrence of neurologic symptoms was 5 months (range 1-54) and to surgery 7 months (range 2-60 months). The level of the spinal dAVF was most frequently at T12-L1 (n = 3). Spinal magnetic resonance arteriography led to localization of the spinal dAVF in 2 patients and spinal catheter angiogram in 3 cases. All patients had definitive radiographic cure of the dAVF at last clinical follow-up., Conclusions: The increased use of endovascular treatment of spinal dAVF has led to the treatment of refractory cases with a greater degree of surgical complexity. Open surgical ligation continues to provide the most definitive treatment outcomes for this complex spinal vascular entity., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: Dural arteriovenous fistula (DAVF) is a vascular malformation. Although rare, DAVFs can represent a potentially reversible cause of dementia. Bilateral white matter changes can be detected on the magnetic resonance imaging of patients with DAVFs. Familiarity with symptoms of DAVFs can reduce the incidence of misdiagnosis of dementia syndrome. This study aimed to explore the clinical manifestation, imaging characteristics, and prognosis of dementia with bilateral white matter changes caused by DAVFs., Case Report: A 56-year-old man presented with memory deterioration, tinnitus, and weakness in both lower limbs for over 2 months. Magnetic resonance imaging of the brain revealed white matter signal changes in the bilateral ventricles and centrum semiovale, which manifested as low signal on T1-weighted imaging, high signal on T2-weighted imaging, fluid-attenuated inversion recovery, and diffusion-weighted imaging. Digital subtraction angiography revealed DAVFs along the lateral sinus. The patients' condition improved significantly after endovascular embolization of the DAVFs., Conclusions: DAVFs presenting with dementia are rare and cases with bilateral white matter alterations can be easily misdiagnosed as other diseases. If the patient has dementia and bilateral alterations in the white matter, a DAVF should be considered. This type of dementia is reversible and may be associated with venous hypertension caused by arteriovenous fistulas. Early digital subtraction angiography is important to reduce misdiagnoses., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Dural arteriovenous fistulas (DAVFs) are a group of diseases involving problematic shunts between dural arteries and venous structures such as sinuses, meningeal veins, or even cortical veins. To focus on craniocervical junction dural arteriovenous fistulas (DAVFs), we introduce a minimally invasive technique with midline incision combined with intraoperative digital subtraction angiography (DSA). This hybrid technique can minimize the incision wound to an average of 6 cm which leads to less destruction and lower risk of adverse events., Method: Using this minimally invasive approach, surgical obliteration was achieved in 6 patients with craniocervical junction DAVFs. A minimal midline incision was made over the C1-2 level, measuring approximately 5 to 7 cm in length. C1 hemilaminectomy was performed for DAVF obliteration followed by intraoperative DSA for confirmation of complete obliteration., Results: Among these 6 patients, the radiculomedullary artery was the most common feeding artery. The mean length of the operation (including DSA performance) was 6.5 ± 1.4 h. None of these cases showed cerebrospinal fluid leakage or exacerbation of neurological symptoms after the operation., Conclusion: Using intraoperative DSA, the minimally invasive technique offers more precise but less destructive access than conventional far lateral suboccipital craniotomy. Most importantly, intraoperative DSA provided verification of complete closure for shunts that could not be examined for indocyanine green (ICG) dye because the microscope did not have a clear line of sight. In our experience, this technique shows encouraging results of fistula obliteration., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Dural arteriovenous fistula (dAVF) accounts for approximately 10% of all intracranial vascular malformations. While they can be benign lesions, the presence of retrograde venous drainage and cortical venous reflux makes the natural course of these lesions aggressive high risk of haemorrhage, neurological injury and mortality. Endovascular treatment is often the first line of treatment for dAVF. Both transarterial and transvenous approaches are used to cure dAVF. The selection of treatment approach depends on the angioarchitecture of the dAVF, the location, the direction of venous flow. Surgery and, to a lesser extent, stereotactic radiosurgery are used when endovascular approaches are impossible or unsuccessful.