Dural arteriovenous fistula-induced thalamic dementia: report of 4 cases

Nonhemorrhagic neurological deficits are underrecognized symptoms of intracranial dural arteriovenous fistulas (dAVFs) having cortical venous drainage. These symptoms are the consequence of cortical venous hypertension and portend a clinical course with increased risk of neurological morbidity and mortality. One rarely documented and easily misinterpreted type of nonhemorrhagic neurological deficit is progressive dementia, which can result from venous hypertension in the cortex or in bilateral thalami. The latter, which is due to dAVF drainage into the deep venous system, is the less common of these 2 dementia syndromes. Herein, the authors report 4 cases of dAVF with venous drainage into the vein of Galen causing bithalamic edema and rapidly progressive dementia. Two patients were treated successfully with endovascular embolization, and the other 2 patients were treated successfully with endovascular embolization followed by surgery. The radiographic abnormalities and presenting symptoms rapidly resolved after dAVF obliteration in all 4 cases. Detailed descriptions of these 4 cases are presented along with a critical review of 15 previously reported cases. In our analysis of these 19 published cases, the following were emphasized: 1) the clinical and radiographic differences between dAVF-induced thalamic versus cortical dementia syndromes; 2) the differential diagnosis and necessary radiographic workup for patients presenting with a rapidly progressive thalamic dementia syndrome; 3) the frequency at which delays in diagnosis occurred and potentially dangerous and avoidable diagnostic procedures were used; and 4) the rapidity and completeness of symptom resolution following dAVF treatment.

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MR Detection of Dilated Deep Medullary Veins in Intracranial Dural Arteriovenous Fistulas with retrograde Leptomeningeal Venous Drainage

Interventional Neuroradiology ◽

10.1177/159101990200800306 ◽

2002 ◽

Vol 8 (3) ◽

pp. 265-272 ◽

Cited By ~ 2

Author(s):

C.M. Chan ◽

K.M. Cheng ◽

J.Y.L. Cheung ◽

F.W.T. Lee ◽

K.W. Tang ◽

...

Keyword(s):

Neurological Complication ◽

Venous Drainage ◽

Neurological Complications ◽

Venous Hypertension ◽

Neurological Deficits ◽

Intracerebral Haematoma ◽

Arteriovenous Fistulas ◽

Severe Degree ◽

The Brain ◽

Deep Medullary Veins

Patients with dural arteriovenous fistula (DAVF) are at higher risk of developing neurological deficits when there is retrograde leptomeningeal venous drainage. Our aim is to demonstrate the presence of dilated deep medullary veins in the brain on magnetic resonance imaging (MR) in this group of patients, and to assess their clinical significance. Nine patients with angiographically proven DAVF associated with leptomeningeal venous drainage who had MR before treatment were studied. MR was performed in at least two orthogonal planes before and after gadolinium administration. The dural fistula was located at the cavernous sinus in five patients, at the transverse-sigmoid sinus in three and at the tentorium in one. Dilated deep medullary veins were noted in six patients. Of these, four showed parenchymal abnormalities which included intracerebral haematoma, venous infarction, brain oedema and T2 hyper-intensity in brainstem. Venous varix was present in one patient. No neurological complication or parenchymal change was observed in the three patients without dilated deep medullary veins. Therefore, in patients with intracranial DAVF associated with leptomeningeal venous recruitment, the MR finding of dilated deep medullary veins suggests a more severe degree of venous hypertension and congestion in the brain. This subgroup of patients has a much higher chance of neurological complications and warrants urgent intervention.

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The role of surgery for high-grade intracranial dural arteriovenous fistulas: importance of obliteration of venous outflow

Journal of Neurosurgery ◽

10.3171/2008.9.jns08733 ◽

2009 ◽

Vol 110 (5) ◽

pp. 913-920 ◽

Cited By ~ 41

Author(s):

James K. Liu ◽

Aclan Dogan ◽

Dilantha B. Ellegala ◽

Jonathan Carlson ◽

Gary M. Nesbit ◽

...

Keyword(s):

Surgical Intervention ◽

Transarterial Embolization ◽

Endovascular Embolization ◽

Venous Hypertension ◽

Neurological Deficits ◽

Definitive Treatment ◽

Radical Excision ◽

Venous Outflow ◽

Arteriovenous Fistulas ◽

Dural Arteriovenous Fistulas

Object Surgical intervention may be required if endovascular embolization is insufficient to completely obliterate intracranial dural arteriovenous fistulas (DAVFs). The authors report their 14-year experience with 23 patients harboring diverse intracranial DAVFs that required surgical intervention. Methods Between 1993 and 2007, 23 patients underwent surgery for intracranial DAVFs. The following types of DAVFs were treated: superior petrosal sinus (in 10 patients); parietooccipital (in 3); confluence of sinuses and ethmoidal (in 2 each); and tentorial, falcine, occipital, transverse-sigmoid, superior sagittal, and cavernous sinuses (in 1 patient each). In all cases, the authors' goal was to obliterate the DAVF venous outflow by direct surgical interruption of the leptomeningeal venous drainage. Transarterial embolization was used primarily as an adjunct to decrease flow to the DAVF prior to definitive treatment. Results Complete angiographic obliteration of the DAVF was achieved in all cases. There were no complications of venous hypertension, venous infarction, or perioperative death. There were no recurrences and no further clinical events (new hemorrhages or focal neurological deficits) after a mean follow-up of 45 months. Conclusions The authors' experience emphasizes the importance of occluding venous outflow to obliterate intracranial DAVFs. Those that drain purely through leptomeningeal veins can be safely obliterated by surgically clipping the arterialized draining vein as it exits the dura. Radical excision of the fistula is not necessary.

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Intracranial Hemorrhage From Dural Arteriovenous Fistulas: Symptoms, Early Rebleed, and Acute Management: A Single-Center 8-Year Experience

Neurosurgery Open ◽

10.1093/neuopn/okaa019 ◽

2020 ◽

Vol 1 (4) ◽

Author(s):

Wilhelm Sorteberg ◽

Angelika Sorteberg ◽

Eva Astrid Jacobsen ◽

Pål Rønning ◽

Per Kristian Eide

Keyword(s):

Intracranial Hemorrhage ◽

Aneurysmal Subarachnoid Hemorrhage ◽

Venous Drainage ◽

Venous Hypertension ◽

Thunderclap Headache ◽

Neurological Deficits ◽

Venous Aneurysm ◽

Acute Management ◽

Arteriovenous Fistulas ◽

Dural Arteriovenous Fistulas

Abstract BACKGROUND Cerebral dural arteriovenous fistulas (dAVFs) presenting with hemorrhage are so rare that reports on their characteristics and guidelines for their acute management are scarce. OBJECTIVE To identify characteristics of the clinical and radiological presentation of hemorrhaging dAVFs, and establish their frequency of early rebleed so that implications for their acute management can be drawn. METHODS Retrospective analysis of all patients admitted with intracranial hemorrhage from a dAVF during the years 2011 to 2018. RESULTS Twenty patients (14 males) with a median age of 61 yr (27-75 yr) were included. Thunderclap headache was the presenting symptom in 13 (65%) patients. Rebleed prior to treatment occurred in 35% of the patients at median 7.5 h (range 3-96 h) after the ictus. All dAVFs had retrograde venous drainage and a venous aneurysm with a bleb was the source of hemorrhage in 16 (80%) patients, all of them presenting with headache. In contrast, patients bleeding due to diffuse venous hypertension presented with neurological deficits. Endovascular treatment was successful in 2 cases; hence, definite dAVF treatment was surgical in 18 (90%) patients. At median 7 mo (2-29 mo) after the ictus, 13 (65%) patients were in Glasgow Outcome Scale-Extended 7 or 8. CONCLUSION The typical presentation of hemorrhage from a cranial dAVF is thunderclap headache. The origin of hemorrhage is often a ruptured venous aneurysm with a bleb. The high frequency of early rebleeds warrants management strategies equivalent to those established for aneurysmal subarachnoid hemorrhage. Overall outcome is favorable.

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Dural arteriovenous fistula presenting with dementia and bulbar symptoms

BMJ Case Reports ◽

10.1136/bcr-2020-234907 ◽

2020 ◽

Vol 13 (7) ◽

pp. e234907

Author(s):

Christiana Avye Hall ◽

David Swienton ◽

Esteban Luis Taleti

Keyword(s):

Arteriovenous Fistula ◽

Dural Arteriovenous Fistula ◽

Venous Hypertension ◽

Neurological Deficits ◽

Progressive Dementia ◽

Arteriovenous Fistulas ◽

Neurological Morbidity ◽

Dural Arteriovenous Fistulas ◽

Bulbar Symptoms ◽

Tectal Plate

Dural arteriovenous fistulas are relatively rare. Some cases are difficult to diagnose, leading to unnecessary investigations, treatments and delays, particularly if the presentation is atypical. We report a case of a man who presented with progressive dementia and bulbar symptoms, both under-recognised non-haemorrhagic neurological deficits, caused by cortical venous hypertension. Brain imaging showed unusual bilateral thalamic, tectal plate and midbrain oedema. The patient was investigated and treated for alternative aetiologies, before being correctly diagnosed and managed using angiographic embolisation. His clinical and radiological signs improved significantly following treatment, reducing his risk of neurological morbidity and mortality.

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Advances in Surgical Approaches to Dural Fistulas

Neurosurgery ◽

10.1227/neu.0000000000000228 ◽

2014 ◽

Vol 74 (suppl_1) ◽

pp. S32-S41 ◽

Cited By ~ 19

Author(s):

Patrick P. Youssef ◽

Albert Jess Schuette ◽

C. Michael Cawley ◽

Daniel L. Barrow

Keyword(s):

Treatment Options ◽

Venous Drainage ◽

Venous Hypertension ◽

Surgical Approaches ◽

Dural Sinus ◽

Intraoperative Angiography ◽

Indocyanine Green Videoangiography ◽

Arteriovenous Fistulas ◽

The Past ◽

Dural Arteriovenous Fistulas

Abstract Dural arteriovenous fistulas are abnormal connections of dural arteries to dural veins or venous sinuses originating from within the dural leaflets. They are usually located near or within the wall of a dural venous sinus that is frequently obstructed or stenosed. The dural fistula sac is contained within the dural leaflets, and drainage can be via a dural sinus or retrograde through cortical veins (leptomeningeal drainage). Dural arteriovenous fistulas can occur at any dural sinus but are found most frequently at the cavernous or transverse sinus. Leptomeningeal venous drainage can lead to venous hypertension and intracranial hemorrhage. The various treatment options include transarterial and transvenous embolization, stereotactic radiosurgery, and open surgery. Although many of the advances in dural arteriovenous fistula treatment have occurred in the endovascular arena, open microsurgical advances in the past decade have primarily been in the tools available to the surgeon. Improvements in microsurgical and skull base approaches have allowed surgeons to approach and obliterate fistulas with little or no retraction of the brain. Image-guided systems have also allowed better localization and more efficient approaches. A better understanding of the need to simply obliterate the venous drainage at the site of the fistula has eliminated the riskier resections of the past. Finally, the use of intraoperative angiography or indocyanine green videoangiography confirms the complete disconnection of fistula while the patient is still on the operating room table, preventing reoperation for residual fistulas.

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Embolization of a superior sagittal sinus dural arteriovenous fistula under intrasinus balloon protection: A case report

Interventional Neuroradiology ◽

10.15274/inr-2014-10098 ◽

2015 ◽

Vol 21 (1) ◽

pp. 94-100 ◽

Cited By ~ 8

Author(s):

Yongxin Zhang ◽

Qiang Li ◽

Qing-hai Huang

Keyword(s):

Dural Arteriovenous Fistula ◽

Superior Sagittal Sinus ◽

Therapeutic Option ◽

Venous Drainage ◽

Endovascular Embolization ◽

Arteriovenous Fistulas ◽

Sagittal Sinus ◽

Dural Arteriovenous Fistulas ◽

Cerebral Venous Drainage ◽

Balloon Protection

Endovascular embolization has evolved to become the primary therapeutic option for dural arteriovenous fistulas (DAVFs). While guaranteeing complete occlusion of the fistula orifice, the goal of DAVF embolization is also to ensure the patency of normal cerebral venous drainage. This paper describes a case of successful embolization of a complex DAVF in the superior sagittal sinus with a multistaged approach using a combination of transvenous and transarterial tactics. The strategies and techniques are discussed.

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Epidermoid Cysts and Cholesterol Granulomas Centered on the Posterior Fossa: Twenty Years of Diagnosis and Management

Neurosurgery ◽

10.1227/00006123-198712000-00004 ◽

1987 ◽

Vol 21 (6) ◽

pp. 798-805 ◽

Cited By ~ 79

Author(s):

Howard Ian Sabin ◽

Lorenzo Tommaso Bordi ◽

Lindsay Symon

Keyword(s):

Posterior Fossa ◽

Diagnostic Procedures ◽

Jugular Foramen ◽

Neurological Deficits ◽

Petrous Apex ◽

Epidermoid Cysts ◽

Diagnosis And Management ◽

Presenting Symptoms ◽

Review Period ◽

Contrast Ventriculography

Abstract The clinical features, diagnosis, and management of 23 posterior fossa epidermoid cysts and 9 petrous apex lesions presenting to one unit over a period of 20 years are summarized. Of the epidermoid cysts, 13 were entirely infratentorial, but the other 10 had an additional supratentorial component. Presenting symptoms and signs were usually long-standing and at onset had often been vague and nonspecific. With time, however, a variety of neurological deficits that depended on the site of the lesion developed. These were generally combinations of cerebellopontine (CP) angle and jugular foramen syndromes, deafness, facial palsy, and motor weakness. Diagnostic procedures have changed greatly over the review period. Computed tomography and magnetic resonance imaging have replaced air encephalography and contrast ventriculography. The better preoperative localization of these lesions allows rational planning of the surgical approach required for optimal tumor exposure, which is essential for any attempt at total excision, considering the large size of the majority of these tumors when diagnosed. We favor operation through a posterior fossa craniectomy for those tumors restricted to the CP angle or 4th ventricle, but routinely use a combined supra- and infratentorial approach if the lesion has a more rostral component. The infiltrating nature of epidermoid cysts within the cranium compromises the extent of excision if neurological deficit is not to be increased, but we attempt as complete an excision of tumor and capsule as possible in the hope that many years will pass before symptoms recur. Cholesterol granulomas seem to respond well to simple cavity drainage and have shown no tendency to recur.

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A variant of arteriovenous fistulas within the wall of dural sinuses. Results of combined surgical and endovascular therapy

Journal of Neurosurgery ◽

10.3171/jns.1991.74.2.0199 ◽

1991 ◽

Vol 74 (2) ◽

pp. 199-204 ◽

Cited By ~ 92

Author(s):

Stanley L. Barnwell ◽

Van V. Halbach ◽

Christopher F. Dowd ◽

Randall T. Higashida ◽

Grant B. Hieshima ◽

...

Keyword(s):

Venous Drainage ◽

Venous Hypertension ◽

Neurological Dysfunction ◽

Dural Sinus ◽

Unique Type ◽

Content Type ◽

Av Fistula ◽

Arteriovenous Fistulas ◽

Av Fistulas ◽

Cortical Venous Drainage

✓ Dural arteriovenous (AV) fistulas are thought to be acquired lesions that form in an area of thrombosis within a sinus. If the sinus remains completely thrombosed, venous drainage from these lesions occurs through cortical veins, or, if the sinus is open, venous drainage is usually into the involved sinus. Among 105 patients with dural A V fistulas evaluated over the the past 5 years, seven had a unique type of dural AV fistula in the superior sagittal, transverse, or straight sinus in which only cortical venous drainage occurred despite a patent involved sinus; the fistula was located within the wall of a patent dural sinus, but outflow was not into the involved sinus. This variant of dural AV fistulas puts the patient at serious risk for hemorrhage or neurological dysfunction caused by venous hypertension. Three patients presented with hemorrhage, one with progressive neurological dysfunction, one with seizures, and two with bruit and headaches. A combination of surgical and endovascular techniques was used to close the fistula while preserving flow through the sinus.

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Open and endovascular treatment of spinal dural arteriovenous fistulas: a 10-year experience

Journal of Neurosurgery Spine ◽

10.3171/2016.9.spine16394 ◽

2017 ◽

Vol 26 (4) ◽

pp. 519-523 ◽

Cited By ~ 20

Author(s):

Matthew J. Koch ◽

Christopher J. Stapleton ◽

Pankaj K. Agarwalla ◽

Collin Torok ◽

John H. Shin ◽

...

Keyword(s):

Vascular Malformations ◽

Management Strategies ◽

Case Series ◽

Endovascular Embolization ◽

Definitive Treatment ◽

Type I ◽

Arteriovenous Fistulas ◽

Presenting Symptoms ◽

Dural Arteriovenous Fistulas ◽

Spinal Dural Arteriovenous Fistulas

OBJECTIVE Vascular malformations of the spine represent rare clinical entities with profound neurological implications. Previously reported studies on management strategies for spinal dural arteriovenous fistulas (sDAVFs) appeared before the advent of modern liquid embolic agents. Authors of the present study review their institutional experience with endovascularly and surgically treated sDAVFs. METHODS The authors performed a retrospective, observational, single-center case series on sDAVFs treated with endovascular embolization, microsurgical occlusion, or both between 2004 and 2013. The mode, efficacy, and clinical effect of treatment were evaluated. RESULTS Forty-seven patients with spinal arteriovenous malformations were evaluated using spinal angiography, which demonstrated 34 Type I sDAVFs (thoracic 20, lumbar 12, and cervical 2). Twenty-nine of the patients (85%) were male, and the median patient age was 63.3 years. Twenty patients underwent primary endovascular embolization (16 Onyx, 4 N-butyl cyanoacrylate [NBCA]), and 14 underwent primary surgical clipping. At a mean follow-up of 36 weeks, according to angiography or MR angiography, 5 patients treated with endovascular embolization demonstrated persistent arteriovenous shunting, whereas none of the surgically treated patients showed lesion persistence (p = 0.0237). Thirty patients (88%) experienced some resolution of their presenting symptoms (embolization 17 [85%], surgery 13 [93%], p = 1.00). CONCLUSIONS Microsurgical occlusion remains the most definitive treatment modality for sDAVFs, though modern endovascular techniques remain a viable option for the initial treatment of anatomically amenable lesions. Treatment of these lesions usually results in some clinical improvement.

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Endovascular treatment of intracranial dural arteriovenous fistulas with spinal perimedullary venous drainage

Journal of Neurosurgery ◽

10.3171/jns.1992.77.5.0718 ◽

1992 ◽

Vol 77 (5) ◽

pp. 718-723 ◽

Cited By ~ 64

Author(s):

Y. Pierre Gobin ◽

Andre Rogopoulos ◽

Armand Aymard ◽

Mazen Khayata ◽

Daniel Reizine ◽

...

Keyword(s):

Endovascular Treatment ◽

Endovascular Therapy ◽

Sinus Thrombosis ◽

Venous Drainage ◽

Transverse Sinus ◽

Venous Hypertension ◽

Arterial Phase ◽

Content Type ◽

Arteriovenous Fistulas ◽

Av Fistulas

✓ Intracranial dural arteriovenous (AV) fistulas with spinal perimedullary venous drainage are rare lesions that have distinctive clinical, radiological, and therapeutic aspects. Five patients presented with an ascending myelopathy, which extended to involve the brain stem in three cases. Myelography and magnetic resonance imaging showed slightly dilated spinal perimedullary vessels. Spinal angiograms were normal in the arterial phase. Diagnosis was only possible after cerebral angiography, which demonstrated posterior fossa AV fistulas fed by meningeal arteries and draining into spinal perimedullary veins. Endovascular treatment alone resulted in angiographic obliteration of the lesion in three patients. Two patients required surgery in addition to endovascular therapy. One patient died postoperatively, and in one a transient complication of embolization was observed. Improvement after treatment was good in two cases and fair in two. Transverse sinus thrombosis was observed in three cases and was probably the cause of the aberrant venous drainage of the fistula into the spinal perimedullary veins. The pathophysiology is related to spinal cord venous hypertension. These lesions were classified as Type 5 in the Djindjian and Merland classification of dural intracranial AV fistulas. Endovascular therapy is a safe effective method in the treatment of these fistulas and should be tried first.

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