Spinal Vascular Malformations - Typical and Atypical Findings

Bostroem, Azize; Thron, A; Hans, Franz-Josef; Krings, T

doi:10.1055/s-2007-985852

Cited by 18 publications

(11 citation statements)

References 37 publications

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“…49,50 SDAVFs may occur anywhere from the level of the foramen magnum to the sacrum and localization of these lesions may be difficult and challenging, especially in cases in which cord edema occurs distant from the AV shunt location. 51 Thus, the noninvasive evaluation of the shunt location is extremely helpful to guide the invasive conventional angiography. 52 Spinal contrast-enhanced MRA has greatly contributed to localizing these lesions and helping to avoid unnecessary superselective injections of all possible arterial feeders.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…5 Symptoms are related to compression of the adjacent nerve root or cord rather than venous congestion, unless unusual cases of perimedullary reflux occur. 51 Perimedullary AV fistulas, including fistulas of the filum terminale, are pial shunts at the surface of the cord (or the filum) that are invariably supplied by arteries that, under normal circumstances, would supply the cord (ie, the ASA or the posterior spinal artery). In high-flow shunts, venous pouches are commonly encountered in those vascular malformations, especially in young patients with HHT.…”

Section: Differential Diagnosismentioning

confidence: 99%

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Spinal Dural Arteriovenous Fistulas

Krings¹,

Geibprasert²

2009

AJNR Am J Neuroradiol

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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: Differential Diagnosismentioning

confidence: 99%

Spinal Dural Arteriovenous Fistulas

Krings¹,

Geibprasert²

2009

AJNR Am J Neuroradiol

Self Cite

374

376

View full text Add to dashboard Cite

show abstract

“…In other cases, follow-up imaging or selective spinal angiography may be necessary. Spinal cord cavernomas may mimic other tumors, especially if they are large, such as gliomas, ependymomas or anaplastic astrocytomas [58]. …”

Section: Cavernomasmentioning

confidence: 99%

Vascular Malformations of the Spine and Spinal Cord*

Krings

2010

Clin Neuroradiol

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Spinal vascular malformations are rare diseases with a wide variety of neurologic presentations. Their classification depends on the differentiation of shunting versus nonshunting lesions, the latter being the spinal cord cavernomas. In the shunting lesions, the next step in the proposed classification scheme is related to the feeding artery which can subdivide the dural vascular shunts from the pial vascular malformations: while those shunts that are fed by radiculomeningeal arteries (i.e., the counterparts of meningeal arteries in the brain) constitute the dural arteriovenous fistulas, the shunts that are fed by arteries that would normally supply the spinal cord (i.e., the radiculomedullary and radiculopial arteries) are the pial cord arteriovenous malformations (AVMs; whose cranial counterparts are the brain AVMs). Depending on the type of transition between artery and vein, the latter pial AVMs can be further subdivided into glomerular (plexiform or nidus-type) AVMs with a network of intervening vessels in between the artery and vein and the fistulous pial AVMs. The last step in the classification then describes whether the type of fistula has a high or a low shunting volume which will differentiate the "macro-" from the "micro"fistulas. The proposed classification is therefore based on a stepwise analysis of the shunt including its arterial anatomy, its nidus architecture and its flow-volume evaluation. The major advantage of this approach is that it leads to a subclassification with direct implications on the choice of treatment, thereby constituting a simple and practical approach to evaluate these rare diseases.

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“…In other cases, follow-up imaging or selective spinal angiography may be necessary. Spinal cord cavernomas may mimick other tumors, especially if they are large, such as gliomas, ependymomas or anaplastic astrocytomas 36 . Figure 6 Non-shunting vascular malformation of the spinal cord.…”

Section: Cavernomasmentioning

confidence: 99%

Classification of Spinal Vascular Malformations

et al. 2009

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SUMMARY -Spinal vascular malformations are rare diseases with a wide variety of neurological presentations. Their classification depends on the differentiation of shunting versus non-shunting lesions, the latter being the spinal cord cavernomas. In the shunting lesions, the next step in the proposed classification scheme is related to the feeding artery which can subdivide the dural vascular shunts from the pial vascular malformations: while those shunts that are fed by radiculomeningeal arteries (i.e. the counterparts of meningeal arteries in the brain) constitute the dural arteriovenous fistulas, the shunts that are fed by arteries that would normally supply the spinal cord (i.e. the radiculomedullary and radiculopial arteries) are the pial cord arteriovenous malformations (whose cranial counterparts are the brain AVMs). Depending on the type of transition between artery and vein the latter pial AVMs can be further subdivided into glomerular (plexiforme or nidus-type) AVMs with a network of intervening vessels inbetween the artery and vein and the fistulous pial AVMs. The last step in the classification then describes whether the type of fistula has a high or a low shunting volume which will differentiate the "Macro-" from the "Micro-"fistulae. The proposed classification is therefore based on a stepwise analysis of the shunt including its arterial anatomy, its nidus-architecture and its flow-volume evaluation. The major advantage of this approach is that it leads to a subclassification with direct implications on the choice of treatment, thereby constituting a simple and practical approach to evaluate these rare diseases.

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Spinal Vascular Malformations - Typical and Atypical Findings

Cited by 18 publications

References 37 publications

Spinal Dural Arteriovenous Fistulas

Spinal Dural Arteriovenous Fistulas

Vascular Malformations of the Spine and Spinal Cord*

Classification of Spinal Vascular Malformations

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