Spinal Dural Arteriovenous Fistula Presenting with Myelopathy

Arteriovenous Fistulas ◽

Degenerative Spinal Disease ◽

Spinal Arteriovenous Malformations ◽

Abstract: Spinal dural arteriovenous fistulas are a rare cause of congestive myelopathy. Symptoms are insidious in onset and may be confused with degenerative spinal disease. MRI characteristically shows edema of the spinal cord with serpiginous flow voids that follow the surface of the spinal cord. Careful evaluation with spinal angiography is required to ensure accurate diagnosis. Spinal dural arteriovenous fistulas differ from spinal arteriovenous malformations in that most fistulas have only a single fistulous point without a nidus. Spinal dural arteriovenous fistulas may be treated successfully with either surgical resection or endovascular embolization depending on their anatomy. Earlier treatment is associated with better outcomes.

Progressive Gait Dysfunction

10.1093/med/9780190495541.003.0034 ◽

2016 ◽

Author(s):

Ji Y. Chong ◽

Michael P. Lerario

Keyword(s):

Vascular Malformations ◽

Arteriovenous Fistulas ◽

Progressive Myelopathy ◽

Spinal Vascular Malformations ◽

Gait Dysfunction ◽

Spinal Arteriovenous Malformations

Neurological Change ◽

Spinal vascular malformations are rare, with dural arteriovenous fistulas (AVFs) accounting for the majority of the pathology. Unlike spinal arteriovenous malformations, which cause abrupt neurological change as a result of hemorrhage, spinal dural AVFs tend to result in a progressive myelopathy through venous congestion and cord edema. If diagnosed and treated early with endovascular embolization or microsurgery, some deficits may be reversible.

Rare association between spinal dural arteriovenous fistulas and dysraphisms: Report of two cases and review of the literature with a focus on pitfalls in diagnosis and treatment

Interventional Neuroradiology ◽

10.1177/1591019917714636 ◽

2017 ◽

Vol 23 (5) ◽

pp. 458-464 ◽

Cited By ~ 5

Author(s):

Giacomo Talenti ◽

Giovanni Vitale ◽

Giacomo Cester ◽

Alessandro Della Puppa ◽

Roberto Faggin ◽

...

Keyword(s):

Vascular Malformations ◽

Diagnosis And Treatment ◽

Review Of The Literature ◽

Arteriovenous Fistulas ◽

Rare Association ◽

Spinal Vascular Malformations ◽

Spinal Arteriovenous Malformations ◽

Spinal vascular malformations are uncommon yet important spinal pathologies commonly classified in congenital and acquired lesions. Spinal lipomas consist of three subtypes: intramedullary lipomas, lipomyelo(meningo)celes and lipomas of the filum. Although the association of spinal arteriovenous malformations (AVM) with other congenital anomalies is well known, the coexistence of dural arteriovenous fistulas (AVF) and tethered spinal cord is exceptionally rare and only eight cases have been reported. We present two cases from our institution and speculate on the possible origin of such a rare but insidious association. We review the current literature with a focus on possible pitfalls in diagnosis and treatment.

Spinal dural arteriovenous fistula associated with a spinal perimedullary fistula

Journal of Neurosurgery Spine ◽

10.3171/spi.2006.4.3.241 ◽

2006 ◽

Vol 4 (3) ◽

pp. 241-245 ◽

Cited By ~ 20

Author(s):

Timo Krings ◽

Volker A. Coenen ◽

Martin Weinzierl ◽

Marcus H. T. Reinges ◽

Michael Mull ◽

...

Keyword(s):

Spinal Cord ◽

Arteriovenous Fistula ◽

Vascular Malformations ◽

Venous Drainage ◽

Elevated Pressure ◽

Arteriovenous Fistulas ◽

Progressive Myelopathy ◽

Dural Arteriovenous Fistulas

✓ Among spinal cord vascular malformations, dural arteriovenous fistulas (DAVFs) must be distinguished from intradural malformations. The concurrence of both is extremely rare. The authors report the case of a 35-year-old man who suffered from progressive myelopathy and who harbored both a DAVF and an intradural perimedullary fistula. During surgery, both fistulas were identified, confirmed, and subsequently obliterated. The fistulas were located at two levels directly adjacent to each other. Although the incidence of concurrent spinal DAVFs is presumed to be approximately 2%, the combination of a dural and an intradural fistula is exceedingly rare; only two other cases have been reported in the literature. One can speculate whether the alteration in venous drainage caused by the (presumably congenital) perimedullary fistula could possibly promote the production of a second dural fistula due to elevated pressure with concomitant venous stagnation and subsequent thrombosis. The authors conclude that despite the rarity of dual pathological entities, the clinician should be aware of the possibility of the concurrence of more than one spinal fistula in the same patient.

СLINICAL ASPECTS, DIAGNOSTICS AND MICROSURGICAL TREATMENT OF SPINAL DURAL ARTERIOVENOUS FISTULAS (LECTURE)

Russian journal of neurosurgery ◽

10.17650/1683-3295-2019-21-2-53-65 ◽

2019 ◽

Vol 21 (2) ◽

pp. 53-65

Author(s):

G. Yu. Evzikov ◽

V. А. Parfenov ◽

А. V. Farafontov ◽

P. V. Kuchuk ◽

S. А. Kondrashin ◽

...

Keyword(s):

Arteriovenous Fistula ◽

Microsurgical Treatment ◽

Arteriovenous Fistulas ◽

Wide Range ◽

The lecture is dedicated to spinal dural arteriovenous fistula – infrequent disorder which not well known among wide range of neurosurgeons. The findings on etiology, clinic and treatment are presented.

Spinal dural arteriovenous fistulas: outcome and prognostic factors

Neurosurgical FOCUS ◽

10.3171/2012.2.focus1218 ◽

2012 ◽

Vol 32 (5) ◽

pp. E11 ◽

Cited By ~ 35

Author(s):

Marco Cenzato ◽

Alberto Debernardi ◽

Roberto Stefini ◽

Giuseppe D'Aliberti ◽

Maurizio Piparo ◽

...

Keyword(s):

Neurological Status ◽

Anatomical Location ◽

Arteriovenous Fistulas ◽

Thoracic Level ◽

Spinal Dural Arteriovenous Fistulas ◽

Better Than

The aim of this study is to review the clinical outcome of patients treated for spinal dural arteriovenous malformations and investigate the presence of pretreatment indicators of outcome after short- and midterm follow-up. The authors retrospectively reviewed the records of 65 consecutive patients treated either surgically or endovascularly in 3 neurosurgery departments between 1989 and 2009. After treatment, 80% of patients reported improvement of at least 1 symptom. Motor symptoms improved more than sensory disorders, pain, or sphincter impairment. Spinal dural arteriovenous fistulas at the thoracic level, and in particular at the lower level, responded better than those in other spinal areas. Spinal dural arteriovenous fistula is a rare pathology with a malignant course that should be treated aggressively. This study appears to confirm that neurological status before treatment, anatomical location of the fistula, and symptoms at presentation are all factors that can predict outcome. Early diagnosis of spinal dural arteriovenous malformations in the lower thoracic area in patients with an Aminoff scale score < 3 was associated with the most favorable outcome.

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):

Superior Sagittal Sinus ◽

Therapeutic Option ◽

Venous Drainage ◽

Arteriovenous Fistulas ◽

Sagittal Sinus ◽

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.

Clinical presentation and prognostic factors of spinal dural arteriovenous fistulas: an overview

Neurosurgical FOCUS ◽

10.3171/2012.1.focus11376 ◽

2012 ◽

Vol 32 (5) ◽

pp. E17 ◽

Cited By ~ 40

Author(s):

Jennifer E. Fugate ◽

Giuseppe Lanzino ◽

Alejandro A. Rabinstein

Keyword(s):

Spinal Cord ◽

Clinical Symptoms ◽

Neurological Deficits ◽

Intramedullary Spinal Cord ◽

Lower Extremity Weakness ◽

Arteriovenous Fistulas ◽

Signal Abnormality ◽

Sensory Disturbances ◽

Spinal dural arteriovenous fistulas (AVFs), the most common type of spinal cord vascular malformation, can be a challenge to diagnose and treat promptly. The disorder is rare, and the presenting clinical symptoms and signs are nonspecific and insidious at onset. Spinal dural AVFs preferentially affect middle-aged men, and patients most commonly present with gait abnormality or lower-extremity weakness and sensory disturbances. Symptoms gradually progress or decline in a stepwise manner and are commonly associated with pain and sphincter disturbances. Surgical or endovascular disconnection of the fistula has a high success rate with a low rate of morbidity. Motor symptoms are most likely to improve after treatment, followed by sensory disturbances, and lastly sphincter disturbances. Patients with severe neurological deficits at presentation tend to have worse posttreatment functional outcomes than those with mild or moderate pretreatment disability. However, improvement or stabilization of symptoms is seen in the vast majority of treated patients, and thus treatment is justified even in patients with substantial neurological deficits. The extent of intramedullary spinal cord T2 signal abnormality does not correlate with outcomes and should not be used as a prognostic factor.

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 ◽

Definitive Treatment ◽

Type I ◽

Arteriovenous Fistulas ◽

Presenting Symptoms ◽

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.

Asymptomatic spinal dural arteriovenous fistulas: pathomechanical considerations

Journal of Neurosurgery Spine ◽

10.3171/2012.2.spine11500 ◽

2012 ◽

Vol 16 (5) ◽

pp. 441-446 ◽

Cited By ~ 19

Author(s):

Kenichi Sato ◽

Karel G. TerBrugge ◽

Timo Krings

Keyword(s):

Spinal Cord ◽

Clinical Symptoms ◽

Dorsal Side ◽

Venous Hypertension ◽

Venous Outflow ◽

Arteriovenous Fistulas ◽

Asymptomatic Patients ◽

Upper Thoracic ◽

Object Spinal dural arteriovenous fistulas (SDAVFs) consist of a shunt with converging feeding vessels arising from radiculomeningeal arteries and draining retrogradely via a radicular vein into the perimedullary veins, thereby causing progressive myelopathy due to venous hypertension in the spinal cord. The purpose of this study was to evaluate the hypothesis that the obstruction of radicular venous outlets could be an additional factor inducing symptomatic venous hypertension due to a decreased outflow in SDAVFs. Methods The authors compared the clinical and imaging findings in patients with asymptomatic SDAVFs identified incidentally at the upper thoracic region with the findings in symptomatic patients who harbored SDAVFs at the same level. Results All symptomatic patients presented with medullary dysfunction. The mean age of patients with asymptomatic SDAVF was 51.5 years, approximately 10 years younger than the patients with symptomatic SDAVF (64.1 years old). Despite the existence of dilated perimedullary vessels in the dorsal side of the spinal cord in all patients, the spinal cord edema seen in symptomatic patients was not detected on the MR images obtained in patients with asymptomatic SDAVF. The spinal angiograms of the asymptomatic patients distinctively demonstrated early radicular venous outflow from affected perimedullary veins to the extradural venous plexus as a potential alternate route for the venous hypertension to be released. Conclusions Obstruction of the radicular venous outflow could be an important factor in inducing spinal congestive edema due to venous hypertension, as well as subsequent clinical symptoms of SDAVFs.

Endovascular management of spinal dural arteriovenous fistulas

Neurosurgical FOCUS ◽

10.3171/2009.2.focus098 ◽

2009 ◽

Vol 26 (5) ◽

pp. E15 ◽

Cited By ~ 46

Author(s):

Walavan Sivakumar ◽

Gabriel Zada ◽

Parham Yashar ◽

Steven L. Giannotta ◽

George Teitelbaum ◽

...

Keyword(s):

Surgical Treatment ◽

Vascular Malformations ◽

Embolic Agent ◽

Endovascular Management ◽

Arteriovenous Fistulas ◽

Spinal Vascular Malformations ◽

Embolic Agents ◽

Object Spinal dural arteriovenous fistulas (DAVFs) are the most common spinal vascular malformations and can be a significant cause of myelopathy, yet remain inefficiently diagnosed lesions. Over the last several decades, the treatment of spinal DAVFs has improved tremendously due to improvements in neuroimaging, microsurgical, and endovascular techniques. The aim of this paper was to review the existing literature regarding the clinical characteristics, classification, and endovascular management of spinal DAVFs. Methods A search of the PubMed database from the National Library of Medicine and reference lists of all relevant articles was conducted to identify all studies pertaining to spinal DAVFs, spinal dural fistulas, and spinal vascular malformations, with particular attention to endovascular management and outcomes. Results The ability to definitively treat spinal DAVFs using endovascular embolization has significantly improved over the last several decades. Overall rates of definitive embolization of spinal DAVFs have ranged between 25 and 100%, depending in part on the embolic agent used and the use of variable stiffness microcatheters. The majority of recent studies in which N-butyl cyanoacrylate or other liquid embolic agents were used have reported success rates of 70–90%. Surgical treatment remains the definitive option in cases of failed embolization, repeated recanalization, or lesions not amenable to embolization. Clinical outcomes have been comparable to surgical treatment when the fistula and draining vein remain persistently occluded. Improvements in gait and motor function are more likely following successful treatment, whereas micturition symptoms are less likely to improve. Conclusions Endovascular embolization is an increasingly effective therapy in the treatment of spinal DAVFs, and can be used as a definitive intervention in the majority of patients that undergo modern endovascular intervention. A multidisciplinary approach to the treatment of these lesions is required, as surgery is required for refractory cases or those not amenable to embolization. Newer embolic agents, such as Onyx, hold significant promise for future therapy, yet long-term follow-up studies are required.