Cervical spine dural arteriovenous fistula presenting with congestive myelopathy of the conus

Geibprasert, Sasikhan; Pongpech, Sirintara; Jiarakongmun, Pakorn; Krings, Timo

doi:10.3171/2009.4.spine08118

Cited by 25 publications

(19 citation statements)

References 24 publications

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“…Neither the location of pathologic vessels nor the intramedullary imaging findings seem to be related to the height of the fistula. 30 On T1-weighted scans, the swollen cord is slightly hypointense and enlarged. Following contrast administration, diffuse enhancement may be seen within the cord as a sign of chronic venous congestion with a breakdown of the bloodϪspinal cord barrier (Fig 4).…”

Section: Clinical Featuresmentioning

confidence: 99%

“…29 Low cervical DAVFs (below C2 and above T1) are extremely rare. 30,31 In approximately 2% of patients, double spinal DAVFs or an association of a spinal dural with a spinal pial AV shunt may be present, raising the possibility of a potential etiologic connection. 32,33 Etiology and Pathophysiology It is presumed that SDAVFs are acquired diseases, though their exact etiology is not known.…”

Section: Epidemiologymentioning

confidence: 99%

“…7 Concerning the DSA appearance, the classic SDAVFs are typically of the slow-flow type, supplied by radiculomeningeal arteries and draining into radicular veins, which directly connect with either ascending or descending perimedullary veins; however, occasionally these angiographic characteristics may be difficult to differentiate from other simulating vascular shunting lesions (ie, radicular AV malformations [rAVMs]; epidural AV shunts, which belong to the ventral and dorsal epidural group of DAVFs; or perimedullary AV fistulas). 30 rAVMs or AV malformations of the nerves usually have abnormal vessels forming a nidus surrounding the nerve root, whereas SDAVFs have an apparent shunt zone with converging radicular feeders into the same draining vein. These patients also often present with radicular pain and not direct symptoms of congestive venous myelopathy.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…The delayed venous return following ASA injection indicates venous congestion and underlines the necessity to search for a shunting lesion, whereas in most cases, a normal venous return following injection of the anterior spinal artery (ASA) will exclude the possibility of an SDAVF (Fig 6). 30 After injection into the segmental artery harboring the AV fistula, the early venous filling and the retrograde contrast uptake of the radiculomedullary veins are visualized. Often an extensive network of dilated perimedullary veins is visible.…”

Section: Clinical Featuresmentioning

confidence: 99%

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Spinal Dural Arteriovenous Fistulas

Krings¹,

Geibprasert²

2009

AJNR Am J Neuroradiol

374

376

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SUMMARY:Spinal dural arteriovenous (AV) fistulas are the most commonly encountered vascular malformation of the spinal cord and a treatable cause for progressive para-or tetraplegia. They most commonly affect elderly men and are classically found in the thoracolumbar region. The AV shunt is located inside the dura mater close to the spinal nerve root where the arterial blood from a radiculomeningeal artery enters a radicular vein. The increase in spinal venous pressure leads to decreased drainage of normal spinal veins, venous congestion, and the clinical findings of progressive myelopathy. On MR imaging, the combination of cord edema, perimedullary dilated vessels, and cord enhancement is characteristic. Therapy has to be aimed at occluding the shunting zone, either by superselective embolization with a liquid embolic agent or by a neurosurgical approach. Following occlusion of the fistula, the progression of the disease can be stopped and improvement of symptoms is typically observed. Despite being the most commonly encountered spinal vascular malformation, spinal dural arteriovenous fistulas (SDAVFs) are rare and still underdiagnosed entities, which, if not treated properly, can lead to considerable morbidity with progressive spinal cord symptoms. Because presenting clinical symptoms are unspecific, the neuroradiologist is often the first clinician to raise the possibility of this diagnosis, which initially rests mainly on MR imaging. For a thorough understanding of the disease and for planning the therapeutic strategy, however, selective spinal digital subtraction angiography (DSA) still is necessary. The aim of the following article is to review the epidemiology, etiology, clinical and imaging features, and therapeutic approaches of this type of spinal vascular malformation. Because an understanding of spinal vascular malformations both from an etiologic and pathophysiologic standpoint is based on the spinal vascular anatomy, we will start by briefly describing the salient features of the spine and spinal cord arterial supply and venous drainage followed by a classification of spinal vascular malformations in general and a classification of dural arteriovenous (AV) shunts in particular. Embryology and Anatomy of the Spinal VasculatureDevelopment of the neural plate starts during the third gestational week and is derived from the embryologic ectoderm. This process is induced by the underlying notochord and adjacent mesoderm, which regulate the development of the surrounding structures, including the nerves, blood vessels, and somites.1 In this stage, the angioblasts initially form small cell clusters (blood islands) within the embryonic and extraembryonic mesoderm.2 Formation of the neural tube begins early in the fourth week (days 22-23) with closure of the rostral and caudal neuropore during days 25-27, which coincides with the establishment of the intrinsic blood vascular circulation within the spinal cord.3 Two longitudinal collector systems form in the subarachnoid space at the dorsal and ventral surfac...

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Section: Clinical Featuresmentioning

confidence: 99%

Section: Epidemiologymentioning

confidence: 99%

Section: Differential Diagnosismentioning

confidence: 99%

Section: Clinical Featuresmentioning

confidence: 99%

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Spinal Dural Arteriovenous Fistulas

Krings¹,

Geibprasert²

2009

AJNR Am J Neuroradiol

374

376

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“…Patients with radicular AVMs often present with radicular pain and only rarely show congestive venous myelopathy. 23 In terms of the relationship between spinal and intracranial DAVSs, the spinal DAVS lesions probably are the embryologic homolog of the intracranial DAVSs draining into the petrosal vein or bridging veins of the medulla. 11 They represent the lateral epidural group of DAVSs proposed by Geibprasert et al 14 These lateral epidural shunts are characterized by male predominance, later age of onset, and the presence of cortical venous reflux, which has also been shown in our series.…”

Section: Discussionmentioning

confidence: 99%

Angiographic Characteristics and Treatment of Cervical Spinal Dural Arteriovenous Shunts

Kim¹,

Willinsky²,

Geibprasert³

et al. 2010

AJNR Am J Neuroradiol

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SUMMARY: Spinal DAVSs of the cervical level are rare lesions. The purpose of this study is to describe the clinical and angiographic characteristics of cervical spinal DAVSs. From a prospectively collected database including 449 cases of brain and spinal DAVSs, lesions located at the cervical level were selected. The clinical presentation, angiographic characteristics, and treatment outcome were assessed. Twelve cases of spinal DAVSs were identified at the level of the cervical spinal canal (male to female ratio ϭ 8:4; mean age ϭ 56.5 years). Five patients (41.7%) presented with hemorrhage including SAH (n ϭ 4) and cerebellar hemorrhage (n ϭ 1). Coincidental spinal DAVSs with cranial DAVSs or brain AVMs were noted in 5 cases (41.7%). The spinal DAVS was the symptomatic lesion in 10 cases and was incidentally discovered during evaluation for SAH from a coincidental lesion in 2 cases. Combined endovascular and surgical resection resulted in symptomatic improvement in 10 patients. In conclusion, DAVSs of the cervical spine are rare lesions which often present with hemorrhage and are frequently associated with complex coincidental vascular lesions. Combined endovascular and surgical treatment will result in good outcome.ABBREVIATIONS: APA ϭ ascending pharyngeal artery; ASA ϭ anterior spinal artery; AVF ϭ arteriovenous fistula; AVM ϭ arteriovenous malformation; br ϭ branch; Caud ϭ caudal; Cbll ϭ cerebellar; CostoCerv ϭ costocervical artery; DAVS ϭ dural arteriovenous shunt; DSA ϭ digital subtraction angiography; FU ϭ follow-up; hem ϭ hemorrhage; ICA ϭ internal cerebral artery; Lt ϭ left; min ϭ minimal; NA ϭ not available; n-BCA ϭ n-butyl 2-cyanoacrylate; OA ϭ occipital artery; PVA ϭ polyvinyl alcohol; Rost ϭ rostral; Rt ϭ right; SCA ϭ superior cerebral artery; SAH ϭ subarachnoid hemorrhage; SupPetSin ϭ superior petrosal sinus; ThCerv ϭ thyrocervical artery; Tsin ϭ transverse sinus; VA ϭ vertebral artery S pinal DAVS is a disease in which an abnormal arteriovenous communication develops between the dural branch of a radicular artery and a radicular or leptomeningeal vein along the dura.1 It is by far the most common spinal arteriovenous shunt, typically characterized by presentation beyond the fourth or fifth decade with male predominance and venous congestive myelopathy. The most prevalent location is along the thoracolumbar spine, with rare occurrence in the cervical level.2 This disproportionate distribution has been attributed to the differences of venous drainage patterns. The thoracolumbar cord drains via small-caliber radiculospinal veins, which may make venous drainage somewhat tenuous and sensitive to hemodynamic alterations. In contrast, the venous drainage at the cervical level is more divergent and, therefore, may be less susceptible to the development of various vascular lesions.2 Literature regarding cervical spinal DAVSs is limited to a few case series. 3-6The purpose of this study was to assess the clinical and angiographic characteristics of cervical spinal DAVSs from a large prospectively collected s...

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Spinal Dural Arteriovenous Fistula: A Review

Maimon

Luckman

Strauss

2016

Advances and Technical Standards in Neurosurgery

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Spinal dural arteriovenous fistula (SDAVF) is a rare disease, the etiology of which is not entirely clear. It is the most common vascular malformation of the spinal cord, comprising 60-80 % of the cases. The clinical presentation and imaging findings may be nonspecific and misleading, often mistaking it for other entities like demyelinating or degenerative diseases of the spine.This chapter describes the imaging findings, clinical signs, and symptoms of this disease and also the available treatment options according to the current literature.Angiography is still considered the gold standard for diagnosis; however, MRI/MRA is increasingly used as a screening tool. Modern endovascular techniques are becoming increasingly more effective in treating SDAVF offering a less invasive treatment option; however, they still lag behind surgical success rates which approach 100 %. The outcome of both treatment options is similar if complete obliteration of the fistula is obtained and depends mainly on the severity of neurological dysfunction before treatment.Heightened awareness by radiologists and clinicians to this rare entity is essential to make a timely diagnosis of this treatable disease. A multidisciplinary treatment approach is required in order to make appropriate treatment decisions.

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Cervical spine dural arteriovenous fistula presenting with congestive myelopathy of the conus

Cited by 25 publications

References 24 publications

Spinal Dural Arteriovenous Fistulas

Spinal Dural Arteriovenous Fistulas

Angiographic Characteristics and Treatment of Cervical Spinal Dural Arteriovenous Shunts

Spinal Dural Arteriovenous Fistula: A Review

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