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Schematic drawing of the dural arteriovenous fistula and surrounding venous structures in case 1. APA indicates the ascending pharyngeal artery; DV, drainage vein; CS, cavernous sinus; SPS, superior petrosal sinus; IPS, inferior petrosal sinus; BP, basilar plexus; MS, marginal sinus; JV, jugular vein.
Source publication
We report 2 patients with dural arteriovenous fistula of the anterior condylar vein in which the patients presented with rare clinical symptoms related to unusual venous drainage patterns. The first patient had progressive myelopathy and showed venous drainage into the anterior spinal vein. The second had ocular signs and showed retrograde drainage...
Context in source publication
Context 1
... anterior condylar vein usually joins the inferior petrosal sinus that drains to the jugular bulb, but in this case, the inferior petrosal sinus went straight down to the jugular vein without joining the jugular bulb in the venous phase of the vertebral angiography. These anatomical features are shown schematically in Figure 4. ...
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Subacute necrotizing myelopathy (SNM) or Foix-Alajouanine syndrome is a rare disease characterized by progressive neurological dysfunction caused by a spinal dural arteriovenous fistula (AVF). Radiological diagnosis is usually suspected when there is intramedullary nonspecific enhancement and perimedullary flow voids. Ring-enhancement is rarely rep...
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We report a 62-year-old woman who presented with a myelopathy at the lower thoracic level. Left vertebral angiography revealed a dural arteriovenous fistula (DAVF) at the craniocervical junction, draining into an anterior spinal vein. Below the T7 level, the spinal cord gave high signal on T2-weighted images and enhanced with Gd-DTPA. The patient w...
Introduction:
We report the cases of a 68-year-old male with a filum terminale arteriovenous fistula (AVF) who was initially misdiagnosed with neuromyelitis optica spectrum disorder (NMOSD) based on imaging findings and false-positive aquaporin-4 IgG (AQP4-IgG).
Case presentation:
A 68-year-old male presented with slowly progressive weakness and...
Background:
Intra-operative imaging during surgical ligation of a spinal dural arteriovenous fistula (SDAVF) is usually based on fluorescence angiography, intra-operative ultrasound with Doppler modality and intra-operative digital subtraction angiography. The aim of our work was to explore the potential role of contrast enhanced ultrasound (CEUS)...
Spinal dural arteriovenous fistula is a rare vascular lesion of the spinal cord associated with progressive myelopathy. Symptoms include progressive gait dysfunction, weakness, sensory loss, and bowel and bladder dysfunction. Because these symptoms overlap with other common causes of myelopathy and the disease is rare, spinal dural arteriovenous fi...
Citations
... A case of an AC-AVF has been reported, in which the superior ophthalmic vein was surgically exposed at the supraorbital margin followed by direct puncture of the superior ophthalmic vein to reach the shunted pouch and densely embolize the shunt. 17 However, in addition to cosmetic problems, this method of direct puncture of the superior ophthalmic vein exposed by resection may cause bleeding and difficulty in identification of the superior ophthalmic vein, supraorbital nerve injury, and eyelid elevator muscle injury. 18 To our knowledge, no case has been reported in which direct puncture of the external jugular vein was used to access the shunted pouch of an AC-AVF and embolization was safely achieved. ...
BACKGROUND
The main feeding artery of an anterior condylar arteriovenous fistula (AC-AVF) is the ascending pharyngeal artery and rarely the internal maxillary artery.
OBSERVATIONS
A 58-year-old male with a history of sinusitis since adolescence presented with a 5-year history of bilateral pulsatile tinnitus and a 2-month history of right ocular symptoms. Angiography showed that the peripheral branches of the bilateral internal maxillary arteries were the main feeding arteries of the AC-AVF and that they gathered in the clivus with a relatively large shunted pouch in the left jugular tubercle. Shunt flow drained to the right external jugular vein via the right superior ophthalmic vein. A sheath was placed in the right external jugular vein, and a small distal access catheter was guided to the right superior ophthalmic vein to allow the microcatheter to reach the shunted pouch. Selective angiography of the contralateral sphenopalatine artery allowed us to confirm the gathering site of the feeding arteries and the shunted pouch and archive the complete occlusion.
LESSONS
Selective angiography of the contralateral sphenopalatine artery may be useful to confirm the gathering site of the peripheral branches of the bilateral internal maxillary arteries in an AC-AVF.
... It has been reported that dAVFs at the ACC are rare, accounting for 1.8-3.6% of intracranial dAVFs; [2,4] however, detailed imaging evaluation has revealed a significant number of dAVFs at the ACC with shunts to veins other than the ACC. [1,3,8,10] One vein, the JTVC, is in the vicinity of the hypoglossal canal and communicates with the ACV and jugular vein. [6] In the present case, detailed imaging evaluation revealed a shunt that was not in the ACC but in the JTVC [ Figure 1d]. ...
Background:
Dural arteriovenous fistulas (dAVFs) occurring near the hypoglossal canal are rare. Detailed evaluation of vascular structures can identify shunt pouches at the jugular tubercle venous complex (JTVC) in the bone near the hypoglossal canal. Although the JTVC has several venous connections, including the hypoglossal canal, there have been no reports of transvenous embolization (TVE) of a dAVF at the JTVC using an approach route other than the hypoglossal canal. This report describes the first case of complete occlusion with targeted TVE using an alternative approach route in a 70-year-old woman presenting with tinnitus diagnosed with dAVF at the JTVC.
Case description:
The patient had no history of head trauma or other preexisting conditions. Magnetic resonance imaging (MRI) showed no abnormal findings in the brain parenchyma. Magnetic resonance angiography (MRA) revealed a dAVF near the ACC. The shunt pouch was located in the JTVC, near the left hypoglossal canal, with feeders from the bilateral ascending pharyngeal arteries and occipital arteries, left meningohypophyseal trunk, and odontoid arch of the left vertebral artery. TVE was performed near the shunt pouch. Localized packing of the shunt point was achieved. The patient's tinnitus improved. Postoperative MRI showed disappearance of the shunt without any complications. No recurrence was observed on MRA 6 months after treatment.
Conclusion:
Our results suggest targeted TVE is an effective treatment for dAVFs at the JTVC.
... Intraosseous AVF with a shunt existing in the intraosseous venous pouch was initially reported by Malik et al. [3]. Some reports of intraosseous AVFs were located around the clivus, such as hypoglossal canal, anterior/posterior condylar vein, petrous apex, upper clivus, or foramen magnum [4][5][6][7][8][9][10][11][12]. Piske et al. described AVFs can develop wherever veins had a transosseous course, as in the emissary veins [13]. ...
PurposeArteriovenous fistulas (AVFs) adjacent to the clivus, such as cavernous sinus dural AVFs (CSDAVFs) and condylar AVFs, sometimes have an intraosseous shunted pouch and recruit blood supply from transosseous feeders. Precise analysis of transosseous feeders regarding the clival lesion has not yet been performed. Therefore, this study aimed to clarify the characteristics and identity of transosseous feeders in clival lesions.Methods
Patients with CSDAVFs and condylar AVFs, who underwent high-resolution cone-beam computed tomography or three-dimensional rotational angiography in our institute, were included. The frequency, type of branch, penetrating point, and termination of intraosseous feeders were retrospectively evaluated.ResultsA total of 31 patients with 36 lesions in CSDAVFs and 8 patients with 8 lesions in condylar AVFs underwent angiography. For CSDAVFs, 38 transosseous feeders were detected in 23 out of 31 patients, including 22 in the pharyngeal branch of the ascending pharyngeal artery (APhA), 6 in the hypoglossal branch of the APhA, 6 in the accessory meningeal artery, 3 in the meningohypophyseal trunk (MHT), and 1 in the anterior branch of the middle meningeal artery. For condylar AVFs, 24 transosseous feeders were detected in all 8 patients, including 11 in the pharyngeal branches of the APhA, 7 in the hypoglossal branch of the APhA, 6 in the segmental artery from the vertebral artery, and 2 in the MHT.Conclusion
Transosseous feeders of AVFs around the clivus, which are frequently seen in AVFs of this area, mainly arise from ventral clival branches and from pharyngeal branches of the APhA.
... Myelopathy is present in 37-38% of CCFAVFs, while SAH and brainstem dysfunction occur in 35-45% and 3-8% of cases, respectively (12,15). Therefore, its rarity sometimes leads to an incorrect diagnosis of another brainstem or spinal cord disease, especially in cases of lesions localised to the brain stem (1, 11,14). ...
Although arteriovenous fistulas (AVFs) at the craniocervical junction (CCJAVFs) are rare, they often develop into a subarachnoid haemorrhage when they have an ascending venous drainage, or cause venous congestion of the spinal cord with descending venous drainage. Isolated brainstem lesions due to CCJAVF are extremely rare, and, to our knowledge, the vascular architectural features that could cause such lesions are unknown. We present a case of CCJAVF manifesting as isolated brainstem congestion and review the literature on the vessel architecture of these rare lesions. A 64-year-old man was admitted to our hospital with gradually worsening nausea, dysphagia, double vision, grogginess, and gait disturbances. On admission, the patient showed dysarthria, horizontal ocular nystagmus to the left, paresis of cranial nerves IX and X, and ataxia on the right side. Magnetic resonance imaging (MRI) revealed an isolated lesion in the medulla. Cerebral angiography (CAG) showed a CCJAVF with the coexistence of intradural AVF and dural AVF, fed by the right first cervical radiculomedullary, right vertebral, and intradural posterior inferior cerebellar arteries, which were drained by the anterior spinal vein in an ascending direction. The patient underwent direct surgery to occlude dural and intradural fistulas. Postoperatively, the patient returned to work with full recovery from the neurological deficits via rehabilitation. MRI revealed vanishing brainstem congestion, and CAG revealed complete disappearance of the AVF. CCJAVFs with venous drainage around the brainstem, regardless of their direction (ascending or descending), can cause isolated brainstem congestion, although this condition is rare.
... Although performed less frequently by our group, TA obliteration of fistulas of the foramen magnum region are well described in the literature. 23 This approach is particularly important when sinus stenosis or occlusion of the ipsilateral jugular system precludes access to the shunt. 17 TA embolization therefore remains an important alternative tool, although some authors favor TA as a first-line maneuver. ...
Background
Dural arteriovenous fistulas (AVF) of the foramen magnum region (FMR) are technically challenging lesions to treat. Transvenous (TV), transarterial (TA), and surgical approaches have been described, but the optimum treatment strategy is not defined.
Objective
To report treatment strategies and outcomes for FMR-AVF at a single, high-volume referral center.
Methods
A retrospective review from January 2010 to August 2020 identified patients with FMR-AVF at a single referral center. Angiographic features, treatment (observation, endovascular, surgical), and follow-up of angiographic and clinical results were recorded. The technical aspects of TV embolization are then presented in detail.
Results
29 FMR-AVF were identified in 28 patients. Of these, 24/29 (82.8%) were treated and 5/29 (17.2%) were observed. Treatment was endovascular in 21/24 (87.5%), combined (endovascular+surgical) in 2/24 (8.3%), and surgical in 1/24 (4.2%). Endovascular treatments were 76.2% TV, 14.3% TA, and 9.5% combined TV/TA. Sufficient follow-up data were available for 20/28 (71.4%) with mean follow-up of 16.8 months. No AVF recurrence was seen for TA/TV, combined endovascular/surgical, or surgical groups, and there was one recurrence (7.1%) in the TV group. Symptomatic improvement was seen in all groups: TV (71.4% complete, 28.6% partial), TA (66.7% complete, 33.3% no follow-up), TV+TA (100% partial), endovascular/surgical (100% complete), and surgical (100% partial). Minor non-neurologic complications included 1/14 (7.1%) in the TV group and 1/3 (33.3%) in the TA/TV group.
Conclusion
Endovascular treatment is safe and effective for most FMR-AVF. TV embolization has a high cure rate with few complications.
... 1 Both transvenous and transarterial approaches have been described. [9][10][11][12] Successful treatment of FMR AVFs demands rigorous preparation and knowledge of both the arterial supply and venous drainage patterns to achieve a durable cure and avoid nontarget embolization. ...
Background and purpose:
AVFs of the foramen magnum region, including fistulas of the marginal sinus and condylar veins, have complex arterial supply, venous drainage, symptoms, and risk features that are not well-defined. The purpose of this study was to present the angioarchitectural and clinical phenotypes of a foramen magnum region AVF from a large, single-center experience.
Materials and methods:
We retrospectively reviewed cases from a 10-year neurointerventional data base. Arterial and venous angioarchitectural features and clinical presentation were extracted from the medical record. Venous drainage patterns were stratified into 4 groups as follows: type 1 = unrestricted sinus drainage, type 2 = sinus reflux (including the inferior petrosal sinus), type 3 = reflux involving sinuses and cortical veins, and type 4 = restricted cortical vein outflow or perimedullary congestion.
Results:
Twenty-eight patients (mean age, 57.9 years; 57.1% men) had 29 foramen magnum region AVFs. There were 11 (37.9%) type 1, nine (31.0%) type 2, six (20.7%) type 3, and 3 (10.3%) type 4 fistulas. Pulsatile tinnitus was the most frequent symptom (82.1%), followed by orbital symptoms (31.0%), subarachnoid hemorrhage (13.8%), cranial nerve XII palsy (10.3%), and other cranial nerve palsy (6.9%). The most frequent arterial supply was the ipsilateral ascending pharyngeal artery (93.1% ipsilateral, 55.5% contralateral), vertebral artery (89.7%), occipital artery (65.5%), and internal carotid artery branches (48.3%).
Conclusions:
We present the largest case series of foramen magnum region AVFs to date and show that clinical features relate to angioarchitecture. Orbital symptoms are frequent when sinus reflux is present. Hemorrhage was only observed in type 3 and 4 fistulas.
... Accordingly, the treatment goal does not aim to occlude the perimedullary veins, which could further increase venous pressure with the consequent spinal cord injury and increased bleeding risk; but to occlude the primary draining vein. Beyond the rarity of this type of fistula, we have seen the presence in the literature of the same type of fistulas that were described in another way, 25 such as in a 1998 publication. 26 Yutaka Mitsuhashi et al. 27 described an unusual type of fistula with drainage to medulla bridging veins and to petrosal veins and some of them by description would correspond to our subgroup.The importance of knowing the existence of this type of fistula is that the clinical presentation can be with intracranial symptoms or myelopathy or both and this should be consider so as to make a correct diagnosis. ...
Background and purpose:
A well-known classification of dural arteriovenous fistulas (DAVFs) according to the patterns of venous drainage was described in 1977 by Djindjian, Merland et al. and later revised by Cognard, Merland et al. in 1995. They described 5 types of DAVFs assuming that the type of venous drainage is directly correlated with neurologic symptoms and in particular with hemorrhagic risk. We present a series of cases that combines type IV (DAVF with cortical venous drainage associated with venous ectasia) and type V (DAVF with spinal venous drainage), which we named type IV + V.
Materials and methods:
A retrospective study between 2012 and 2020 in 2 Hospitals was performed on patients that met inclusion criteria for a diagnosis of this type of DAVF. Demographics, location, clinical presentation and outcomes of endovascular embolization were studied.
Results:
Five (2,3%) patients out of 220 had a type IV + V DAVF. All cases had an aggressive presentation, either subarachnoid hemorrhage, myelopathy or both. All patients were treated with endovascular transarterial embolization achieving complete angiographic occlusion in one session and total remission of symptoms at 3 months.
Conclusions:
This rare type of DAVF, combines two aggressive venous drainage patterns. For that reason, patients with type IV+V DAVF probably have a more aggressive natural history and worst outcome due to risk of intracranial and/or spinal hemorrhage and myelopathy, thus requiring urgent diagnostic and treatment. Larger studies are needed to better understand this type of DAVF.
... 4,8 Few HC-DAVFs drain into cortical and perimedullary veins increasing the risk of myelopathy and intracerebral hemorrhage. 2,4,8,9 A thorough understanding of the skull base venous anatomy is critical for the diagnosis of HC-DAVFs. [4][5][6]10 In many cases, MRA source images help to localize the fistula to the hypoglossal canal due to arterialization of the ACC and ACV 3 . ...
Hypoglossal canal dural arteriovenous fistulas (HC-DAVF) involve the anterior condylar vein (ACV) and anterior condylar confluence (ACC). They often present with tinnitus, bruit, and hypoglossal nerve palsy. The most common treatment in HC-DAVFs is transvenous embolization using coils and the most direct transvenous route is the trans-internal jugular vein access. When this approach is not feasible, a treatment attempt is possible through alternative routes. We report 2 patients with DAVFs involving the anterior condylar confluence. The first patient presented with pulsatile tinnitus and hypoglossal nerve palsy, and was treated by a standard transjugular approach. The second patient presented with pulse-synchronous bruit. Following an unsuccessful attempt of the transjugular approach, the fistulous point was reached via the deep cervical vein and complete occlusion was achieved by coil deployment. Both patients had complete regression of symptoms. Endovascular therapy is the elective treatment for HC-DAVFs and the transjugular approach is the most commonly used. The deep cervical vein (DCV) can be an alternative transvenous route when the transjugular approach fails.
... The estimated detection rate was 0.29 per 100,000 persons per year according to a Japanese survey published in 2016 (1). In rare circumstances, patients with intracranial DAVFs could be complicated with brainstem engorgement, which might lead to delayed or false diagnosis and subsequent improper management (2)(3)(4). An illustrate case of intracranial DAVF with brainstem engorgement was presented in Figure 1. ...
Background: In rare circumstances, patients with intracranial (dural arteriovenous fistulas) DAVFs could be complicated with brainstem engorgement, which might lead to delayed or false diagnosis and subsequent improper management.
Methods: On July 2th, 2019, a systematic search was conducted in the PubMed database for patients with intracranial DAVFs complicated with brainstem engorgement.
Results: Sixty-eight articles reporting of 86 patients were included for final analysis. The patients were aged from 20 to 76 years (57.10 ± 12.90, n = 82). The female to male ratio was 0.68 (35:51). Thirty-three (40.2%, 33/82) patients were initially misdiagnosed as other diseases. The specific location distributions were cranio-cervical junction, cavernous sinus, superior petrosal sinus, transverse and/or sigmoid sinus, tentorium, and other sites in 27 (32.5%), 11 (13.2%), 9 (10.8%), 10 (12.0%), 21 (25.3%), and 5 (6.0%) patients, respectively. The Cognard classification of DAVFs were II, III, IV, and V in 9 (10.7%, 9/84), 1 (1.2%, 1/84), 1 (1.2%, 1/84), and 73 (86.9%, 73/84) patients. Eighteen (22%, 18/82) patients were demonstrated to have stenosis or occlusion of the draining system distal to the fistula points. The mean follow-up period was 7.86 (n = 74, range 0–60 months) months. Fifty-four (70.1%, 54/77) patients experienced a good recovery according to the mRS score.
Conclusions: Intracranial DAVFs complicated with brainstem engorgement are rare entities. Initial misdiagnosis and delayed definite diagnosis are common in the past three decades. The treatment outcome is still unsatisfactory at present. Early awareness of this rare entity and efficiently utilizing the up to date investigations are of utmost importance.
... 2) In 1999, the association of the anterior condylar vein with the AC-AVF was fi rst described by Ernst et al. 3) Since then, AVFs in this region have been usually referred to as anterior condylar (vein or canal) DAVFs or hypoglossal canal DAVFs. [4][5][6][7][8][9][10][11] Involvement of the other adjacent structures in this region with DAVFs has been also described; Miyachi et al. reported DAVFs involving the anterior condylar confl uence (ACC) and referred to them as ACC-DAVFs. 12) Other authors also reported DAVFs located within the bone adjacent to the hypoglossal canal. ...
The anterior condylar arteriovenous fistula (AC-AVF) is a relatively rare AVF that affects the vasculatures adjacent to the hypoglossal canal. We aimed to discuss the etiology and definition of the AC-AVF from the viewpoint of the osseous venous anatomy. Our recent study, which used modern imaging technology (CT digital subtraction venography and cone beam CT reconstructed from 3D rotational angiography), elucidated the intraosseous venous anatomy in this region and the precise fistulous locations of AC-AVFs. Those findings suggest that the AC-AVF is a group of “osseous” AVF that involves the anterior condylar vein and jugular tubercle venous complex (JTVC), and the osseous veins connected to them. The AC-AVF develops in osseous veins adjacent to the hypoglossal canal, and it is one of the most common subtypes of osseous AVFs. The angioarchitectures and etiology of AC-AVFs discussed herein are essential to understand this clinical entity.
