Late diagnosis of spinal dural arteriovenous fistulas resulting in severe lower-extremity weakness: a case series

OBJECTIVEVascular malformations of the cervical spine are exceedingly rare. To date there have been no large case series describing the clinical presentation and angioarchitectural characteristics of cervical spine vascular malformations. The authors report their institutional case series on cervical spine vascular malformations diagnosed and treated at their institution.METHODSThe authors retrospectively reviewed all patients with spinal vascular malformations from their institution from January 2001 to December 2018. Patients with vascular malformations of the cervical spine were included. Lesions were characterized by their angioarchitectural characteristics by an interventional neuroradiologist and endovascular neurosurgeon. Data were collected on clinical presentation, imaging findings, treatment outcomes, and long-term follow-up. Descriptive statistics are reported.RESULTSOf a total of 213 patients with spinal vascular malformations, 27 (12.7%) had vascular malformations in the cervical spine. The mean patient age was 46.1 ± 21.9 years and 16 (59.3%) were male. The most common presentations were lower-extremity weakness (13 patients, 48.1%), tetraparesis (8 patients, 29.6%), and lower-extremity sensory dysfunction (7 patients, 25.9%). Nine patients (33.3%) presented with hemorrhage. Fifteen patients (55.6%) had modified Rankin Scale scores of 0–2 at the time of diagnosis. Regarding angioarchitectural characteristics, 8 patients (29.6%) had intramedullary arteriovenous malformations (AVMs), 5 (18.5%) had epidural arteriovenous fistulas (AVFs), 4 (14.8%) had paraspinal fistulas, 4 (14.8%) had mixed epidural/intradural fistulas, 3 (11.1%) had perimedullary AVMs, 2 (7.4%) had dural fistulas, and 1 patient (3.7%) had a perimedullary AVF.CONCLUSIONSThis retrospective study of 27 patients with cervical spine vascular malformations is the largest series to date on these lesions. The authors found substantial angioarchitectural heterogeneity with the most common types being intramedullary AVMs followed by epidural AVFs, paraspinal fistulas, and mixed intradural/extradural fistulas. Angioarchitecture dictated the clinical presentation as intradural shunts were more likely to present with hemorrhage and acute onset myelopathy, while dural and extradural shunts presented as either incidental lesions or gradually progressive congestive myelopathy.

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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 ◽

Dural Arteriovenous Fistulas ◽

Sensory Disturbances ◽

Spinal Dural Arteriovenous Fistulas

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.

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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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Microsurgical obliteration of a thoracic spinal perimedullary arteriovenous fistula

Neurosurgical FOCUS ◽

10.3171/2014.v3.focus14376 ◽

2014 ◽

Vol 37 (v2supplement) ◽

pp. Video13 ◽

Cited By ~ 1

Author(s):

Justin M. Caplan ◽

Mari Groves ◽

Ignacio Jusue-Torres ◽

Jennifer E. Kim ◽

Jason Liauw ◽

...

Keyword(s):

Lower Extremity ◽

Arteriovenous Fistula ◽

Urinary Retention ◽

Arteriovenous Malformations ◽

Vascular Lesions ◽

Intraoperative Angiography ◽

Lower Extremity Weakness ◽

Arteriovenous Fistulas ◽

Thoracic Spinal ◽

Dural Arteriovenous Fistulas

Spinal vascular lesions are rare and may be classified as a) dural arteriovenous fistulas (AVFs), b) arteriovenous malformations, or c) perimedullary AVFs. In this narrated video illustration, we present the case of a 71-year-old woman who presented with progressive bilateral lower extremity weakness and urinary retention who was diagnosed with a thoracic spinal perimedullary arteriovenous fistula. The diagnostic studies included a thoracic MRI and spinal angiogram. A multilevel thoracic laminoplasty was performed for microsurgical obliteration of the AVF. The techniques of intraoperative angiography, thoracic laminoplasty and microsurgical obliteration and resection of the AVF are reviewed.The video can be found here: http://youtu.be/5vVp3oq5sLg.

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Embolization of Sacral Dural Arteriovenous Fistulas: A Case Series and Literature Review

Interventional Neurology ◽

10.1159/000454929 ◽

2017 ◽

Vol 6 (1-2) ◽

pp. 73-81 ◽

Cited By ~ 3

Author(s):

Anthony M. Alvarado ◽

Diogo C. Haussen ◽

Koji Ebersole ◽

Raul G. Nogueira ◽

Michael G. Abraham

Keyword(s):

Intraoperative Monitoring ◽

Case Series ◽

Embolic Agent ◽

Spinal Angiography ◽

Lower Extremity Weakness ◽

Ethylene Vinyl Alcohol ◽

Arteriovenous Fistulas ◽

Dural Arteriovenous Fistulas ◽

Embolization Treatment ◽

Rare Location

The authors report 2 cases of sacral dural arteriovenous fistula (AVF) presenting with gradual neurologic decline characterized by progressive lower extremity weakness and bowel and bladder incontinence. Spinal angiography demonstrated a sacral dural AVF with perimedullary vein engorgement and drainage extending to the level of the cervical spine in both cases. The fistulas were completely obliterated with n-BCA (n-butyl cyanoacrylate) embolic agent in one case, and with ethylene vinyl alcohol (Onyx) in the second, resulting in improvement of the symptoms in both patients. The unique features of this case report include the rare location of the fistula's origin, the necessity for complete spinal angiography, and the use of intraoperative monitoring in one case to guide embolization treatment.

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Recurrence of “cured” dural arteriovenous fistulas after Onyx embolization

Neurosurgical FOCUS ◽

10.3171/2012.2.focus1224 ◽

2012 ◽

Vol 32 (5) ◽

pp. E12 ◽

Cited By ~ 35

Author(s):

Peter Adamczyk ◽

Arun Paul Amar ◽

William J. Mack ◽

Donald W. Larsen

Keyword(s):

Case Series ◽

Endovascular Embolization ◽

Arteriovenous Fistulas ◽

Contributory Factors ◽

Catheter Embolization ◽

Dural Arteriovenous Fistulas ◽

Spinal Dural Arteriovenous Fistulas ◽

Cure Rates

Endovascular embolization with Onyx has been increasingly used to treat intracranial and spinal dural arteriovenous fistulas (DAVFs). Several case series have been published in recent years reporting high DAVF cure rates with this technique. Although it is seldom reported, DAVF recurrence may occur despite initial “cure.” The authors present 3 separate cases of a recurrent DAVF after successful transarterial Onyx embolization. Despite adequate Onyx penetration into the fistula and draining vein, these cases demonstrate that DAVF recanalization may reappear with filling from previous or newly recruited arterial feeders. Other published reports of DAVF recurrence are examined, and potential contributory factors are discussed. These cases highlight the need for awareness of this possible phenomenon and suggest that follow-up angiography should be considered in patients treated with catheter embolization.

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