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Va s c u l a r a n d I n t e r ve n t i o n a l R a d i o l o g y R ev i ew

Baleato Gonzlez et al. Imaging Arteriovenous Fistulas Vascular and Interventional Radiology Review

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Imaging Arteriovenous Fistulas


Sandra Baleato Gonzlez1 Joan C. Vilanova Busquets 2 Roberto Garca Figueiras 3 Carmen Villalba Martn 3 Carmen Seoane Pose 3 Anxo Martnez de Alegra3 Jose A. Castieira Mourenza3
Baleato Gonzlez S, Vilanova Busquets JC, Garca Figueiras R, et al.

OBJECTIVE. Arteriovenous stulas (AVFs) are abnormal communications with shunting of blood from an artery to a vein. AVFs mainly involve the peripheral vascular system but can affect virtually any organ or system in the body. They may be congenital, created surgically for hemodialysis, or caused by pathologic processes. This review describes the diverse origins of AVFs and illustrates the spectrum of imaging ndings at radiography, ultrasound, CT, MRI, and digital angiography for AVFs in different locations: the CNS, thorax, abdomen, musculoskeletal system, and peripheral vascular system. We also discuss syndromes associated with AVFs and describe recent interventional techniques for treating AVFs. CONCLUSION. Familiarity with the spectrum of imaging ndings in AVFs is essential for the accurate interpretation of images and facilitates diagnosis and therapeutic management. Radiologists can play a critical role in the diagnosis and treatment of AVFs. Digital angiography is helpful in elaborating a vascular map for endovascular treatment.

Keywords: arteriovenous stulas, arteriovenous malformations, stula therapeutic embolization, hemodynamics, vascular system DOI:10.2214/AJR.09.2631 Received February 23, 2009; accepted after revision April 15, 2009.
1 Department of Radiology, Hospital POVISA, C/. Salamanca No. 5, Vigo, Pontevedra 36211, Spain. Address correspondence to S. Baleato Gonzlez (baleatorum@hotmail.com). 2 3

Clnica Girona and Hospital Santa Caterina, Girona, Spain.

Department of Radiology, Complexo Hospitalario Universitario de Santiago de Compostela, Santiago de Compostela, Spain. AJR 2009; 193:14251433 0361803X/09/19351425 American Roentgen Ray Society

rteriovenous stulas (AVFs) are abnormal communications with shunting of blood from an artery to a vein that mainly involve the peripheral vascular system but can affect virtually any organ or system in the body. These vascular lesions are uncommon. Depending on the location of the AVF, patients may be asymptomatic or may experience symptoms ranging from mild to fatal. They can have different causes: congenital; surgically created for hemodialysis treatments; or pathologic processes, such as neoplasms, trauma, or erosion of an arterial aneurysm. Arteriovenous malformations (AVMs) are defects of the circulatory system that are generally believed to arise during embryonic or fetal development or soon after birth. The criteria for the diagnosis of AVF by color Doppler ultrasound and duplex ultrasound include low- and high-resistance ow in the supplying artery, high-velocity arterialized waveform in the draining vein, and turbulent high-velocity ow spectrum at the junction of the artery and vein. CT and MR angiography typically show early contrast lling in the vein during the arterial phase. Digital angiography is helpful in identifying the arteries feeding the stula and in elaborating a vascular map for endovascular

treatment. Most patients can be successfully treated with catheter embolization. This review describes the diverse origins of AVFs, illustrating the spectrum of imaging ndings at radiography, ultrasound, CT, MRI, and digital angiography for AVFs in different locations: the CNS, chest, abdomen, genitals, and peripheral vascular system. We also discuss recent interventional techniques for treating AVFs. CNS Intracranial AVFs Carotidcavernous stulas (CCFs) are abnormal communications between the carotid arterial system and the venous cavernous sinus. CCFs constitute approximately 1015% of all intracranial AVMs [1]. CCFs are usually classied as either direct or indirect as depicted on angiograms. Further classication is based on their etiologic and hemodynamic qualities. Direct CCFs may result from trauma, rupture of cavernous carotid aneurysms, or a tear in the wall of a congenitally weak cavernous internal carotid artery secondary to collagen vascular disease. Indirect CCFs are usually spontaneous and idiopathic. The clinical presentation of CCF depends on its size, duration, and location and on venous drainage patterns and collateral vascular anatomy.

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Baleato Gonzlez et al. Patients may be asymptomatic or may present with different ocular symptoms (Fig. 1A), headache, or cranial nerve decits; however, critical neurologic signs such as intra cranial hemorrhage are uncommon. CT and MRI of the brain have a role in the identication of these anomalous communications. MR angiography shows abnormal early lling of a dural sinus during the early arterial phase. Although MR angiography is reliable in screening for dural AVFs (Fig. 1B), cerebral angiography (Fig. 1C) is the reference standard for diagnosing dural AVFs because it allows the anatomy of the arterial pedicles to be documented in detail and elucidates the exact relationship between normal cortical veins and the stula. Several treatment options are available for patients with CCF: conservative treatment, radiation therapy, and surgical or transcatheter embolization. Neurointerventional techniques can now be used to treat these lesions successfully [2], thus obviating surgery. The tear between the artery and the vein is best closed through the blood vessels by placing either balloons or coils in the abnormal communication (Fig. 1D). Spinal AVFs Dural AVFs are the most common type of spinal cord AVMs, accounting for 8085% [3]. Dural AVFs consist of an abnormal communication between a radicular artery and a radicular vein within the dural sleeve of a nerve root. The vein communicates with the coronal venous plexus along the surface of the spinal cord (Fig. 2). These dural AVFs lead to the insidious development of venous hypertension and progressive myelopathy. Venous congestion causes progressive neurologic deterioration that can manifest as sensory disturbances, paraparesis, and sphincter dysfunction [4, 5]. Dural AVFs can be eliminated with endovascular or surgical therapy. Chest Pulmonary AVFs are abnormal communications between a pulmonary artery and pulmonary vein. Pulmonary AVFs are rare lung

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Fig. 1 65-year-old man with exophthalmos and congestion of eyelids, conjunctiva, and orbit who was referred for imaging. His medical history included motorcycle crash with intracranial trauma a few months earlier. A, Photograph shows eye. B, Axial maximum-intensity-projection MR angiogram from time-of-ight sequence reveals prominent ophthalmic vein (arrow ). C, Lateral projection of right internal carotid angiogram shows ophthalmic vein (arrow ) is visible during early arterial phase. D, Angiogram shows endovascular embolization achieved complete occlusion of carotid cavernous stula (arrow ).

Fig. 2 35-year-old man with 6-month history of progressive lower extremity weakness. A, Gadolinium-enhanced turbo spin-echo T1weighted MR image shows numerous engorged veins (arrows ) within spinal canal. B, Thoracic spinal angiogram conrms diagnosis of dural arteriovenous stula.

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Imaging Arteriovenous Fistulas anomalies. They are usually congenital [6]. In most cases (70%), pulmonary AVFs affect patients with Rendu-Osler-Weber syndrome (hereditary hemorrhagic telangiectasia) (Fig. 3), which is a systemic autosomal-dominant disorder manifested by mucocutaneous telangiectasias and AVMs, and are a potential source of morbidity and mortality. Lesions can affect the nasopharynx, CNS, lung, liver, and spleen as well as the urinary and gastrointestinal tracts. The incidence of these malformations in the lungs of patients with Rendu-Osler-Weber syndrome ranges from 15% to 33%. The clinical signs of pulmonary AVF depend on the number of stulas and the sizes of the stulas. Many patients have no symptoms; others have difculty breathing and blood in their sputum. When pulmonary involvement is extensive, dyspnea is the most common clinical symptom owing to a rightto-left shunt that produces hypoxemia, a condition that is usually well tolerated. Pulmonary AVF with right-to-left pulmonary shunting is the major cause of transient ischemic attack, brain abscess, and ischemic stroke in patients with Rendu-Osler-Weber syndrome due to paradoxical embolization of bland or septic material into the cerebrovasculature [7]. The rst-line treatment of pulmonary AVF consists of interventional radiology procedures: embolization with coils, silicone balloons, or the Amplatzer vascular plug, a cylindric self-expanding device. The Amplatzer vascular plug has several advantages: It has a reduced risk of migration compared with the other options, releases easily, and can completely occlude an AVF with a single plug [8]. Abdomen Hepatic AVFs Intrahepatic vascular connections between the hepatic artery, the portal vein, and the hepatic veins are rare. Three types of intrahepatic shunts between the major vessels of the liver are possible: portosystemic venous (portal vein to hepatic vein or vena cava), arterioportal (hepatic artery to portal vein), and arteriosystemic (hepatic artery to hepatic vein). These shunts are rarely seen with cross-sectional imaging techniques. Dynamic CT and MRI allow evaluation of the liver during the arterial and portal venous phases of contrast enhancement and characterization of these anomalies. Arterioportal stulas may be intra- or extrahepatic and acquired or congenital. Arterioportal shunts occur in liver disorders such
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C
Fig. 3 43-year-old woman with history of Rendu-Osler-Weber syndrome who presented with dyspnea. A and B, Coronal maximum-intensity-projection ( A ) and oblique volume-rendered (B) images show vascular malformation. C and D, Selective angiography images conrm pulmonary arteriovenous stula (AVF). Amplatzer device was introduced to occlude AVF.

as cirrhosis, trauma, congenital vascular malformations, and hepatic neoplasms. The most frequently reported abnormal communications are the small arterioportal shunts that occur in cirrhotic livers (Fig. 4A). Intrahepatic arterioportal shunts can be minute or large [9]. Arterioportal shunts are typically associated with hepatocellular carcinoma (Fig. 4B) and are reported in 63% of these patients. Congenital arterioportal stulas can be associated with hereditary hemorrhagic telangiectasia (Fig. 4C), Ehlers-Danlos syndrome, and biliary atresia. The identication of a specic abnormal communication in the presence of multiple hepatic vascular malformations on CT or MRI is the key to suspecting the diagnosis. AVF from penetrating trauma (Figs. 4D and 4E) is rare. Infrequently, intrahepatic arterioportal shunts may cause life-threatening portal hypertension. Surgical intervention is necessary only when interventional radiologic procedures such as em-

bolization have failed. Conservative therapy, surgery (portal vein ligation or hepatic lobectomy), and transcatheter embolization have been used to treat intrahepatic portosystemic venous shunts. Renal AVFs Renal AVFs comprise 7080% of arteriovenous communications in the kidney [10]. Renal AVFs can result from trauma, surgery, percutaneous intervention, tumors, inammation, or erosion of an aneurysm directly into a vein (idiopathic AVF). Renal AVFs are usually asymptomatic, but signs and symptoms associated with renal AVFs include ank pain, hematuria, hypertension, and a palpable abdominal mass [11]. Cardiomegaly or congestive heart failure occurs in 50% of symptomatic patients [10]. Renal Doppler sonography is a noninvasive imaging technique that can show the anomalous communication. Conventional angio g
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Fig. 4 Arterioportal stulas. A, Axial MDCT image shows arterioportal stula (arrow ) in 61-year-old man. B, 42-year-old man with hepatocellular carcinoma (white arrow ) producing arterioportal shunt. Early lling of portal vein (black arrow ) is depicted. Arterial phase coronal maximum-intensity-projection image shows early enhancement of portal vein during arterial phase. C, 33-year-old man with Rendu-Osler-Weber syndrome. Contrast-enhanced arterial phase abdominal CT image shows multiple intrahepatic shunts. D and E, 42-year-old woman with abdominal trauma. Selective arteriography images show early contrast lling in main portal vein and aneurysmal site of communication between dilated hepatic artery and main portal vein. Arterioportal stula was embolized with coils. Fig. 574-year-old woman with iatrogenic arteriovenous stula after having undergone percutaneous biopsy. A, Renal aortogram reveals stula in lower pole branches. B, Angiogram obtained after treatment of stula shows embolization is complete.

raphy (Fig. 5A) may still be necessary for diagnosis and management of renal AVFs. Transcatheter intraarterial occlusion (Fig. 5B), surgery, or a combination of these techniques for the treatment of renal AVFs is indicated in patients with hypertension, congestive heart failure, or hematuria [12]. Mesenteric AVFs Mesenteric AVFs are rare. They most commonly occur after gastrointestinal surgery or penetrating abdominal trauma (Fig. 6). Only 32 cases of iatrogenic mesenteric AVF have been documented [13]. Patients with mesenteric AVF may present abdominal pain, nausea, vomiting, diarrhea, or bleeding A from varices. Clinical ndings are few, with abdominal bruits and ascites being the most common [14]. Mesenteric AVFs are treated with endovascular embolization of the feeding vessel with coils or surgery. B Aortocaval Fistulas Aortocaval stulas are most commonly due to an abdominal aortic aneurysm, occurring in up to 1% of all abdominal aortic aneurysms and in as many as 6% of ruptured

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Imaging Arteriovenous Fistulas traumatic arterial priapism is characterized by increased ow in the corpora cavernosa. The origin of this increased inow is usually an AVF (Fig. 8) or pseudoaneurysm, although iatrogenic injection therapy with papaverine may produce a similar result in adults. This type of priapism is painless because there is no tissue ischemia [19]. Traumatic priapism develops immediately after trauma in two thirds of patients; in the remaining third, priapism appears 115 hours after trauma. This suggests that hemodynamically relevant stulas may develop from vascular defects that initially are very small [20]. The management of traumatic priapism remains a challenging clinical problem. Both conservative methods and surgery are associated with a high risk of erectile dysfunction. Angiography with selective embolization is a safe and effective method to correct posttraumatic priapism [21, 22]. Peripheral Vascular System Peripheral vascular malformations are some of the most difcult lesions to diagnose and treat. Vascular malformations are lesions that present at birth and grow commensurately with the child. Trauma, surgery, and hormone changes inuenced by birth control pills, puberty, and pregnancy may cause the lesion to expand and grow hemodynamically. The main locations of peripheral vascular malformations are the head and neck, 40% of cases; extremities, 40%; and trunk, 20%. Vascular malformations are subcategorized as lymphatic, capillary, venous, arteriovenous, and mixed on the basis of the predominant type of vessel involved. Alternatively, vascular malformations can be classied as low- or high-ow lesions on the basis of hemodynamic ow characteristics. High-ow lesions include congenital AVMs, AVFs, and acquired vascular lesions. Lowow lesions include venous malformations, lymphatic malformations, capillary venous malformations, and mixed lesions. Angiography is useful to conrm the diagnosis and evaluate the extent of the lesion. Evaluation of the characteristics of ow of vascular malformations is essential to determine a treatment adapted for the patient. Conservative treatment is recommended in patients with tolerable symptoms. Peripheral vascular malformations are treated by surgical resection, arterial embolization, or direct percutaneous puncture with embolic materials (sclerotherapy). Percutaneous sclerosis of

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Fig. 6 62-year-old woman with history of abdominal trauma 5 years earlier who presented with abdominal pain. Auscultation revealed audible machinery-type bruit in right ank. A and B, Unenhanced ( A ) and enhanced (B) CT scans show nodular mass (arrows ) in mesentery that enhanced during arterial phase (B). C and D, Angiograms show large arteriovenous stula between superior mesenteric artery and branches of superior mesenteric vein. Selective angiography of superior mesenteric artery shows early venous opacication during arterial phase. Branches of mesenteric artery were surgically ligated.

abdominal aortic aneurysms [15]. Classic signs of an aortocaval stula include a palpable abdominal mass with a machinery bruit and high-output cardiac failure. Other presenting signs and symptoms include lower limb edema, recurrent deep vein thrombosis, venous claudication, pulsating varicose veins, and priapism. A denitive diagnosis of aortocaval stula can be difcult because the classic signs are present in only 2050% of cases [16]. The initial diagnosis is based on clinical suspicion. Early diagnosis and early surgery can markedly improve the prognosis of patients with aortocaval stula. Surgery for aortocaval stula is technically complex, and precautions must be taken to prevent intraopera-

tive bleeding and the passage of air, thrombi, or atherosclerotic debris emboli into the IV catheter through the stula. Aortocaval stula is a severe complication of abdominal aortic aneurysms (Fig. 7A) that may be fatal and requires early diagnosis and prompt treatment. An aortocaval stula may be the consequence of penetrating injuries, such as stab or gunshot wounds, or even, exceptionally, of blunt trauma. Aortocaval stulas have also been reported as a complication of surgical procedures [17] (Fig. 7B). Genitals Arterial priapism arising after trauma to the perineum or penis is distinct from and less common than venous priapism [18]. Post-

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Fig. 7 Aortocaval stulas. A, 61-year-old man who presented with history of intermittent back pain. Physical examination revealed pulsatile abdominal mass. CT scan shows aortocaval stula (arrow ) complicating abdominal aortic aneurysm. We observed early, equal enhancement of inferior vena cava and aorta and loss of normal aortocaval fat plane. B, 29-year-old man with iatrogenic aortocaval stula after lumbar disk surgery who presented with cool and pale lower limbs with decreased pulses. Angiography image shows lling of inferior vena cava during arterial phase, which conrms diagnosis of aortocaval stula.

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B
Fig. 8 38-year-old man who presented with painless priapism after perineal trauma 6 days earlier. Physical examination revealed penile tumescence. A, Angiogram shows arteriovenous stula (arrow ) after selective catheterization of internal iliac artery. B, Angiogram image shows detumescence was achieved after successful embolization with gelatinbased absorbable hemostat (Spongostan, Ferrosan).

Fig. 9 Angiogram of forearm in 37-yearold woman shows arteriovenous stula arises from radial artery and cephalic vein (large arrow ). Pseudoaneurysm arising from radial artery (small arrows ) is result of repeated puncture.

for hemodialysis vascular access when this method can be used. The reasons for this preference include factors such as superior long-term patency rates and a low incidence of infection and other complications; however, radiocephalic AVFs are subject to complications such as venous stenosis, stenosis at the site of suture, or thrombosis [24]. Different methods of imaging can evaluate the patient with dysfunctional hemodialysis access. Sonography is the best technique to visualize the morphology of thrombi or to assess ow changes in cases in which stenosis or thrombosis is clinically suspected. Fistulography provides useful anatomic information for further surgical placement of AVFs and remains the reference standard for evaluating the patency of hemodialysis stulas and for guiding thrombolysis or percutaneous transluminal angioplasty (Fig. 9); MR angiography using time-of-ight or phase-contrast sequences and MDCT angiography may be considered noninvasive alternatives to digital subtraction angiography for the evaluation of failed radiocephalic AVFs [25]. Hand AVFs Multiple AVFs in the hand usually originate from congenital remnants of the embryonic anlagen of the arteries and veins. There are four major types of vascular malformations of the hand and forearm: infantile hemangioma, which is a benign neoplasm; AVF, which is usually acquired; congenital AVM; and predominantly venous lesions, including

low-ow vascular malformations is currently the treatment of choice [23]. Arterial embolization is the most effective treatment in high-ow lesions, with subsequent surgical resection occasionally being necessary.

Radial AVFs Radiocephalic AVFs (Brescia-Cimino AVFs) are surgically constructed to provide vascular access for long-term hemodialysis and continue to be regarded as the primary choice

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Imaging Arteriovenous Fistulas

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A
Fig. 10 High-ow malformation in 35-year-old man who presented with large lesion protruding from hand. A and B, Arterial phase ( A ) and delayed phase (B) angiograms reveal chaotic vascular loops with multiple arteriovenous stulas.

dysplasias and cavernous venous malformations. Common symptoms associated with AVMs in the hand and forearm include pain and swelling, discoloration, ischemic symptoms distal to the lesion, and extremity overgrowth and size discrepancy when compared with the normal side. The diagnosis of congenital AVFs is best made using arteriography (Fig. 10) because

arteriography shows the site of the abnormal communications. MR angiography is useful in diagnosing, characterizing, and determining the extent of lesions. Treatment of an AVM is indicated only for symptomatic lesions and should be directed at eradicating the nidus of the lesion near the area where symptoms are reported. Vascular malformations are extremely difcult to treat; the op-

Fig. 11 Angiogram shows stula between femoral artery and vein with early lling of inferior vena cava in 64-year-old man.

timum treatment strategy may involve surgical resection, transcatheter embolization, or a combination of the two. Femoral AVFs Femoral AVF formation from arterial puncture is less common than other complications such as pseudoaneurysm, hematoma, or thrombus formation; nevertheless, nearly 1% of patients undergoing cardiac catheterization develop a femoral AVF. Femoral AVFs may be associated with pseudoaneurysms or may consist of an isolated endothelial channel [26]. Clinically, high-pressure arterial ow entering veins through an AVF will often produce varicosities and thin-walled venous dilatation. Large AVFs are also associated with ischemia of the distal extremity or high-output congestive heart failure. The diagnosis of femoral AVF can be made using Doppler and duplex sonography in the extremities. Normal arterial waveforms have a typical trochaic high-resistance pattern, whereas AVF arterial waveforms have a very low resistance pattern. Angiography (Fig. 11)

A
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Fig. 12 Arteriovenous malformation (AVM) and arteriovenous stula (AVF). A, MR angiogram shows AVM in distal left calf of 21-year-old man. AVM drains into persistent sciatic vein. B, MR angiogram shows chaotic vascular loops and multiple AVFs with hypertrophy of right leg of 17-year-old woman. Arteriovenous shunting is revealed by early appearance of draining veins.

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A shows early venous opacication during the arterial phase. One third of iatrogenic femoral AVFs close spontaneously within 1 year. Surgical intervention may be necessary; however, implantation of endovascular covered stents is an effective and safe method for the percutaneous closure of pseudoaneurysms and AVFs [27]. Syndromes Associated With AVFs AVFs are associated with a wide spectrum of different manifestations in several characteristic syndromes, most of which are neurocutaneous malformations. Klippel-Trenaunay-Weber Syndrome This syndrome represents a combination of cutaneous hemangioma, bone and soft-tissue hypertrophy, and varicose veins. The lower limb is involved in 95% of cases and the upper limb, in 5% [28]. AVF may also be present and is the feature that distinguishes Klippel-Trenaunay-Weber syndrome from Parkes-Weber syndrome. MRI is helpful in imaging the softtissue hypertrophy. In addition, MR angiography can be helpful in identifying and dening vascular malformations (Figs. 12A and 12B). The management of Klippel-TrenaunayWeber syndrome is primarily conservative; however, surgical treatment is indicated in some patients. Cobb Syndrome Cobb syndrome is a rare noninherited disorder that involves the association of spinal

D
Fig. 13 30-year-old man who was admitted for treatment of cauda equina syndrome. A, Photograph shows cutaneous nevus on right leg (S1 territory). B and C, Sagittal dorsal T2-weighted turbo spin-echo MR images show perimedullary dorsal and lumbar arteriovenous formation (AVF) (arrows , B) and medullary involvement (myelopathy) (arrows , C) secondary to venous hypertension. D, Selective catheterization of Adamkiewicz artery shows spinal AVF (arrow ). E, Surgical view shows venous varicosities.

angiomas or AVMs with congenital, cutaneous vascular lesions in the same dermatome [29]. The most common dermatologic manifestations comprise nevus ammeus and angiokeratomas. Cobb syndrome resembles other neurocutaneous syndromes such as Rendu-Osler-Weber syndrome, Sturge-Weber syndrome, Klippel-Trenaunay syndrome, and Wyburn-Mason syndrome. Patients typically present with sudden onset of radicular pain in the back or lower limb associated with numbness that can be localized below a specic dermatome. Less commonly, patients may present with weakness or bowel or bladder dysfunction.

MRI reveals multiple spinal AVMs, and conventional spinal arteriograms can show this high-ow vascular anomaly (Fig. 13). Current treatment options include combinations of embolization, neurosurgical intervention, corticosteroid therapy, and radiotherapy. Because spinal AVMs are often extensive, curative therapy may not be possible. Conclusion Familiarity with the spectrum of imaging ndings using different techniques is essential for the accurate interpretation of AVFs. Radiologists can play a critical role in the diagnosis and treatment of AVFs. Digital angiography is

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Imaging Arteriovenous Fistulas helpful in elaborating a vascular map for endovascular treatment. References
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