Arteriovenous fistulas of the brain and the spinal cord

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✓ Arteriovenous (AV) fistulas of cerebral and spinal arteries are characterized angiographically by an immediate AV transition without a capillary bed or “nidus” as occurs in AV malformations (AVM's). The clinical presentation, morphology, radiology, and treatment of 12 patients with cerebral AV fistulas and of 12 patients with spinal AV fistulas are reviewed. In the patients with cerebral lesions, headache and seizure disorders were the most common presentations followed by subarachnoid hemorrhage, cardiac failure, progressive neurological dysfunction, and incidental detection on prenatal ultrasound study. In patients with spinal AV fistulas, weakness and sensory disturbance in the lower extremities were the most frequent clinical presentations followed by back pain, disturbances of micturition, and grand mal seizure. The etiology of the symptom complex produced by AV fistulas in each of these locations differed, with venous hypertension being important in spinal cord lesions.

Of the patients with cerebral lesions, nine had a single AV fistula, one had two fistulas, and two had multiple fistulas. An AVM was observed in five patients with fistulas (two large, three small). Nine patients exhibited extramedullary AV fistulas of the spine, of whom eight had a single fistula and one had three fistulas; three patients had intramedullary spinal AV fistulas. An arterial aneurysm was found in association with two fistulas, one cerebral and one spinal. Venous ectasias or varices, frequently exhibiting mural calcification, were observed to be prominent in all AV fistulas involving cerebral arteries and in two involving spinal arteries. The location and size of the venous complexes reflected the diameter of the fistula. In addition to conventional imaging techniques (cerebral angiography, computerized tomography, and magnetic resonance (MR) imaging), MR angiography was a helpful adjunct in the evaluation of fistulas. Treatment strategies employed for AV fistulas in both locations included open surgical and endovascular procedures, frequently used in combination. A satisfactory outcome was observed in all patients.

Article Information

Address reprint requests to: Francis H. Tomlinson, M.B., B.S., F.R.A.C.S., Department of Neurologic Surgery, Royal Brisbane Hospital, Brisbane, Queensland 4029, Australia.

© AANS, except where prohibited by US copyright law.

Headings

Figures

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    Giant arteriovenous (AV) fistula of the left middle cerebral artery in Case 1. Upper Left: Axial computerized tomography scan following angiography showing calcification in the walls of some of the ectatic veins (curved arrows) and thrombosis (t) within the large varix. The lateral ventricles are dilated. Note the associated edema and mass effect. Lower Left: Magnetic resonance (MR) angiogram, axial projection (three-dimensional time-of-flight acquisition), showing a large left middle cerebral artery with the AV fistula indicated by a “jet” (arrows) located in the anterior portion of the sylvian fissure. The distinct signal medial and proximal to the fistula corresponds to a large aneurysm (arrowhead). The heterogenic signals seen in the posterior portion of the medial varix are consistent with subacute thrombosis (t). Upper Center: Left internal carotid artery angiogram, anteroposterior projection in the early phase, showing dilatation of the M1 portion of the middle cerebral artery (7 mm) and the site of the AV fistula (arrow). There is early filling of the venous structures (arrowheads) and aneurysm (x). Without prior MR angiography, however, the differences between these two structures were not apparent. Upper Right: Left internal carotid artery angiogram, venous phase, demonstrating filling of large ectatic veins that drain into the superior sagittal sinus via two small draining veins (arrowheads). Lower Center and Lower Right: Left internal carotid artery angiograms, obtained 6 weeks after obliteration of the fistula and clipping of the aneurysm. There is normalization of the left hemispheric intracranial vasculature.

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    Right occipitoparietal arteriovenous (AV) fistula in Case 2. Upper Left: Left vertebral angiogram, anteroposterior projection. This early phase shows the calcarine branch (small arrowhead) of the right posterior cerebral artery and the angular branch (large arrowhead) of the right middle cerebral artery, visualized through the right posterior communicating artery. The vessels are juxtaposed in the right parietal occipital sulcus as they enter, through a single AV fistula (arrow), into a small common venous chamber (c) before draining into a large varix (v). Upper Right: Left vertebral angiogram, anteroposterior projection. This late phase shows dilatation of the venous component and the presence of arterial collateral branch (arrows) from the posterior cerebral artery territory to the right angular artery feeding the AV fistula. Lower Left: Superselective angiogram of the right angular artery, anteroposterior projection, after deposition of five fibered helical-shaped coils just proximal to the site of the arteriovenous fistula. While four coils (arrows) remain just proximal to the AV fistula, one coil (arrowhead) has migrated distal to the fistula and lies within the common venous chamber. Inset: Right carotid angiogram, anteroposterior projection, obtained 2 weeks after deposition of coils showing that the coils have migrated into the small common chamber (arrow). The morphology of the fistula and the venous drainage is unchanged. Lower Right: Left vertebral injection angiogram, anteroposterior projection, obtained shortly after surgery. Note the sites of surgical occlusion (arrows) which were superficial, distal to the last angiographically visible collaterals.

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    Single extramedullary arteriovenous (AV) fistula of the anterior spinal artery located at the L-1 vertebral level in Case 3. a: Magnetic resonance image (long TR/short TE), sagittal projection, of the thoracic spinal cord with demonstration of an enlarged edematous cord and extramedullary flow-void areas (arrows). b: Magnetic resonance image (short TR/short TE), sagittal projection, demonstrating the enlarged conus with extramedullary vascular structures anterior and posterior to the spinal cord. c and d: Selective angiograms, anteroposterior view, of the left T-11 radicular artery (artery of Adamkiewicz) before occlusion of the AV fistula: early phase (c) and later phase (d). For part of its course, the anterior spinal artery (small arrowheads) runs parallel to the anterior medullary vein (small arrow). The latter drains faster than the posterior medullary vein (small paired arrows). At the L-1 level, the AV fistula (large straight arrow) is obscured by venous collaterals and mimics an AV malformation. The perimedullary veins (curved arrow) are surrounding the enlarged cord. These structures were important surgical landmarks due to their topographic relationship to the prominent left lateral curve of the anterior spinal artery (large arrowhead). Note the lack of radiculospinal venous drainage causing inadequate venous outflow. e and f: Early (e) and late (f) phases of selective angiograms, lateral view, of the left T-11 radicular artery before fistula occlusion. Features are similar to those seen in c and d. g: Selective angiogram, anteroposterior view in the late phase, of the T-11 radicular artery following surgical occlusion of the anterior spinal artery (arrowhead) just above the AV fistula (large arrow). The circulation is markedly delayed. The small posterior spinal artery on the right (small arrows) fills through perispinal collaterals from the anterior spinal artery. h: Selective angiogram, lateral view in the late phase, of the T-11 radicular artery following surgical treatment. The anterior spinal artery below the AV fistula (large arrow) fills retrogradely via the posterior spinal artery. The latter communicates with the anterior spinal artery above the AV fistula via a perimedullary artery (small arrows).

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