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CHAPTER

42

Carotid-Cavernous Sinus Fistulas


Neil R. Miller

DIRECT CAROTID-CAVERNOUS SINUS FISTULAS Anatomy Pathogenesis Clinical Manifestations Diagnosis Natural History Treatment Prognosis after Treatment

DURAL CAROTID-CAVERNOUS SINUS FISTULAS (DURAL ARTERIOVENOUS MALFORMATIONS) Pathogenesis Clinical Manifestations Diagnosis Natural History Treatment Prognosis after Treatment

A carotid-cavernous sinus fistula (CCF) is an abnormal communication between the cavernous sinus and the carotid arterial system. CCFs can be classified by etiology (traumatic vs. spontaneous), velocity of blood flow (high vs. low flow), and anatomy (direct vs. dural; internal carotid vs. external carotid vs. both) (15). Some fistulas are characterized by a direct connection between the cavernous segment of the internal carotid artery and the cavernous sinus (Fig. 42.1). These fistulas usually are of the high flow type. Most often caused by a single, traumatic tear in the arterial wall, they are called direct carotid-cavernous sinus fistulas (4). Other CCFs are dural (1). Many of these lesions are actu-

ally congenital arteriovenous malformations that develop spontaneously, often in the setting of atherosclerosis, systemic hypertension, connective tissue disease, and during or after childbirth. Dural carotid-cavernous sinus fistulas consist of a communication between the cavernous sinus and one or more meningeal branches of the internal carotid artery (Fig. 42.2), the external carotid artery (Fig. 42.3), or both (Fig. 42.4) (1). These fistulas usually have low rates of arterial blood flow. In this chapter, we discuss the pathology, causes, clinical manifestations, diagnosis, treatment, and prognosis of both direct and dural CCFs.

DIRECT CAROTID-CAVERNOUS SINUS FISTULAS


Direct CCFs represent 7090% of all CCFs in most large series (2,3). They occur in both men and women of all ages. ANATOMY A direct CCF results from a single tear in the wall of the cavernous segment of the internal carotid artery. This produces a direct connection between the artery and one or more of the venous channels within the cavernous sinus. The arteriovenous connection usually is short, tangential, and endothelialized (68). It thus is identical in anatomy and hemodynamics to traumatic arteriovenous fistulas elsewhere in the body. Most direct CCFs originate along the course of the cavernous segment of the internal carotid artery, usually from the most proximal region (9), and may project anteriorly, posteriorly, superiorly, or inferiorly. On rare occasions, a fistula can develop between the supraclinoid portion of the internal carotid artery or one of its branches and the cavernous sinus, producing symptoms and signs identical with those caused by more typical direct fistulas (10). Rarely, a direct CCF occurs spontaneously or after trauma in the setting of a persistent primitive trigeminal artery originating from the internal carotid artery within the cavernous sinus (11). PATHOGENESIS Direct CCFs most often result from head trauma (5,12). The most frequent settings are motor vehicle accidents, fights, and falls (1316). The injury may be penetrating or nonpenetrating, and it may be associated with basal or facial skull fracture (17). The injury may be so severe that the 2263

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B
Figure 42.1. Appearance of a direct carotid-cavernous sinus fistula (type A of Barrow [1]). A, Angiographic appearance after a selective injection of the left internal carotid artery shows a collection of contrast material in the cavernous sinus (arrow). Note that the fistula drains anteriorly into the superior ophthalmic vein (arrowheads). There was no contribution from the ipsilateral external carotid artery, nor was there any contribution from the contralateral internal or external carotid arteries. The patient was a 26-year-old woman who suffered a head injury in a motor vehicle accident and developed a red left eye associated with proptosis of the eye and binocular diplopia. B, Artists drawing of a direct carotid-cavernous sinus fistula. Note that there is a single tear in the wall of the internal carotid artery. There is no contribution from either the extradural branches of the internal carotid artery of the extradural branches of the ipsilateral external carotid artery.

A
Figure 42.2. Appearance of dural carotid-cavernous sinus fistula in which the only contribution is from extradural branches of the internal carotid artery. A, A selective left internal carotid arteriogram shows a fistula at the posterior portion of the cavernous carotid artery (arrow). The left external carotid arteriogram was normal. B, Artists drawing shows that this type of fistula is fed only by extradural branches of the internal carotid artery with no contribution from the extradural branches of the ipsilateral external carotid artery (type B of Barrow [1]).

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B
Figure 42.3. Appearance of dural carotid-cavernous sinus in which the only contribution is from extradural branches of the external carotid artery. A, A high-flow fistula is fed by extradural branches of the left external carotid artery, particularly the internal maxillary artery. The fistula drains anteriorly into the ipsilateral superior ophthalmic vein (arrows). There was no contribution from the ipsilateral internal carotid artery or from the contralateral internal or external carotid arteries. The patient was a 65-year-old woman with the gradual onset of redness and swelling of the left eye. B, Artists drawing of the appearance of this type of fistula. Note that the only contribution is from the extradural branches of the external carotid artery (type C of Barrow [1]).

patient requires hospitalization, or it may seem quite trivial, requiring no treatment until symptoms and signs of the fistula develop. Dandy emphasized that the carotid artery may tear or be perforated by a bone fragment during impact from a sudden deformation or fracture through the carotid canal (18). Orbitocranial perforating injury is another cause of traumatic CCF (14). We have seen several patients in whom such a fistula was caused by a stab wound. In all cases, the object (knife, umbrella tip, pencil) passed through the superior orbital fissure and lacerated the internal carotid artery within the cavernous sinus. Direct CCFs may become symptomatic directly after injury, or they may not become evident until several days to weeks later (19). A substantial percentage of direct CCFs are caused by rupture of a preexisting aneurysm of the cavernous segment of the internal carotid artery (3,5,16,20). In some of these patients, the aneurysm has been previously diagnosed because of its mass effect on the structures within the cavernous sinus, whereas, in other patients, development of a CCF is the first sign of the aneurysm (Fig. 42.5). Direct CCFs may be iatrogenic, occurring after various diagnostic and therapeutic procedures. They have developed after a variety of procedures involving the internal carotid artery, including endarterectomy, Fogarty catheterization, and attempted embolization of a cavernous sinus meningioma using a tracker catheter with a guide wire (13,2123). They have also occurred after cranial and percutaneous retrogasserian procedures performed for treatment of trigeminal neuralgia (2527) and after transsphenoidal surgery for pituitary adenoma (27).

Finally, direct carotid-cavernous fistulas have been reported after different kinds of maxillofacial surgery, including rhinoplasty (28), sphenoidotomy (29), external ethmoidectomy and sphenoidectomy (30), myringotomy (31), nasopharyngeal biopsy (32), and maxillary osteotomy (33). Most direct CCFs that become symptomatic after some type of surgery do so immediately, but in some cases, several days to weeks may pass before the fistula produces external manifestations. Direct CCFs that become symptomatic spontaneously are not particularly rare (2,5,3538). Patients in whom a direct fistula seems to develop spontaneously usually have an underlying systemic vasculopathy, such as systemic hypertension or arteriosclerosis (34), or an underlying connective tissue disorder such as fibromuscular dysplasia or EhlersDanlos syndrome (5,3537). CLINICAL MANIFESTATIONS The most common clinical manifestations of a direct CCF are ocular in nature; however, in some cases, the first manifestations of the fistula are neurologic or rhinologic. Nonocular Manifestations Although direct CCFs usually are not thought to be lifethreatening, in fact, there are numerous reports of patients who have experienced significant, often fatal, epistaxis, subarachnoid hemorrhage, or intracerebral hemorrhage from rupture of the fistula (13,3941). Epistaxis is a very rare complication; however, intracerebral hemorrhage occurs in

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Figure 42.4. Appearance of a dural carotid-cavernous fistula that is fed by extradural branches from both the internal and external carotid arteries. A, Selective left internal carotid arteriogram, lateral view, shows a large collection of contrast material in the cavernous sinus (arrow). The fistula drains anteriorly into the left superior ophthalmic vein, which is markedly enlarged. B, Selective left external carotid arteriogram, lateral view, shows multiple contributions from extradural branches of the left external carotid artery. C, Artists drawing of this type of fistula. The fistula is fed by extradural branches of both the internal and the external carotid arteries (type D of Barrow [1]).

about 3% of cases (42). Epistaxis and intracerebral hemorrhage usually occur shortly after the trauma, whereas subarachnoid hemorrhage typically occurs months or years later after the injury (43). Direct fistulas most likely to produce intracerebral hemorrhage are those that drain posteriorly into cerebral veins (39). Rare direct CCFs may cause a steal phenomenon in cerebral vessels, the result of which may be a debilitating or even fatal stroke (44). Ocular Manifestations The direction of blood flow through a direct CCF may be posterior, into the superior and inferior petrosal sinuses, or

anterior, into the orbital veins. Although posteriorly draining fistulas occasionally cause isolated ocular motor nerve pareses, the most severe ocular manifestations occur in patients with anterior redirection of arterial blood through normal orbital venous channels. These manifestations are caused by a combination of diminished arterial flow to the cranial nerves within the cavernous sinus, stasis of both venous and arterial circulation within the eye and orbit, and an increase in episcleral and orbital venous pressure. The ocular manifestations of a direct CCF usually are ipsilateral to the side of the fistula, but they may be bilateral (Fig. 42.6), or even contralateral (Fig. 42.7) (13,45). The lateralization of ocular manifestations depends on the venous

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Figure 42.6. Bilateral ocular manifestations in a patient with a unilateral direct carotid-cavernous sinus fistula. The patient was a 66-year-old woman with hypertension who developed swelling and redness of the left eye followed by ptosis of the left eyelid, diplopia, and redness of the left eye after hitting her head against the windshield in a motor vehicle accident. Angiography confirmed a direct carotid-cavernous sinus fistula originating from the right internal carotid artery. Note dilation of conjunctival vessels of both eyes, with left ptosis and bilateral proptosis.

Figure 42.5. Appearance of a patient who developed a spontaneous direct carotid-cavernous sinus fistula after rupture of a previously asymptomatic cavernous aneurysm. A, The patient has marked proptosis of the left eye, swelling and ecchymosis of the left upper and lower eyelids, and hemorrhagic conjunctival chemosis. B, There is diffuse hemorrhagic chemosis of the conjunctiva. Note that the patient is attempting to look upward to the left, but the left eye is almost completely immobile.

drainage of the cavernous sinuses, including the connections between the two sinuses through the intercavernous sinuses and the basilar sinus, the presence or absence of cortical venous drainage, and the presence or absence of thrombosis within a sinus or a superior ophthalmic vein on one or both sides. Most patients who have bilateral ocular manifestations have a unilateral fistula with cortical venous drainage; however, bilateral CCFs may develop, especially after trauma (46). Proptosis Proptosis is one of the most common signs observed in patients with a direct CCF, occurring in almost all patients

if the fistula is left untreated (Fig. 42.8) (13,14,45). In most cases, proptosis develops rapidly on the side of the fistula, becoming pronounced within a few days although some cases have been described in which proptosis has developed months or even years after head trauma. In such cases, it is assumed that the internal carotid artery was injured at the time of trauma, but a fistula did not develop until shortly before proptosis appeared. In some patients, proptosis develops not only on the side of the fistula but also on the opposite side (13). In about onethird of cases with bilateral proptosis, the proptosis develops simultaneously on both sides (42). In the remainder, the second eye becomes affected days to weeks after the first eye. Once proptosis begins, it increases slowly for several weeks until it finally stabilizes. The eye usually is pushed directly forward, but it may be deviated when ocular motor paresis is present (discussed later). The severity of proptosis varies considerably. Some patients have only a few millimeters of proptosis, and most have 10 mm or less (13); however, Birch-Herschfeld described a patient with 16 mm of proptosis (47). Most patients who have significant proptosis caused by a direct CCF also are aware of a cranial bruit (discussed later) or have evidence of ocular pulsation, although this is not always the case (48,49). Eyelid and Facial Changes In the early stages of a direct CCF, the eyelids may become moderately or even severely swollen (Fig. 42.9) (14). With time, the persistent swelling can become associated with progressive and even grotesque changes in the eyelid

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Figure 42.8. Proptosis from direct carotid-cavernous sinus fistula. Note moderate right proptosis and dilated conjunctival vessels in a 21-year-old woman who was injured in a motor vehicle accident.

B
Figure 42.7. Contralateral ocular manifestations from a direct carotidcavernous sinus fistula. The patient was a 65-year-old woman who developed swelling, redness, and protrusion of the left eye. A, External appearance of the patient at presentation reveals swelling of the left upper eyelid and redness of the left eye. The right eye appears normal. B, Selective right common carotid artery arteriogram, anteroposterior view, reveals a direct carotid-cavernous sinus fistula with immediate crossover to the left side via the intercavernous sinus and subsequent drainage through the left superior ophthalmic vein and superficial cerebral veins.

Figure 42.9. Swelling of eyelids in patients with direct carotid cavernous sinus fistulas. A, The left upper and lower eyelids are moderately swollen in a 16-year-old boy with a left-sided fistula. B, Severe swelling of left upper and lower eyelids in a 44-year-old woman with a left-sided fistula.

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and its vasculature (Fig. 42.10). Ultimately, racemose undulating dilation of periorbital vessels may produce chronic dermal cyanosis and thickening that resembles the changes seen in patients with congenital facial arteriovenous malformations (Fig. 42.11). When the superior ophthalmic vein is

Figure 42.11. Marked facial changes from a chronic, left-sided, direct carotid-cavernous sinus fistula. The patient had suffered a gunshot injury to the left side of the face 8 years previously. Note marked dilation of subcutaneous, periorbital and upper facial vessels. (From Harbour RC, Luxenberg MN. A severe carotid-cavernous fistula treated with a balloon catheter. Arch Ophthalmol 1986;10410841085.)

enlarged, the medial portion of the upper eyelid may be considerably stretched and swollen. Chemosis of Conjunctiva Conjunctival chemosis occurs in most patients with a direct CCF (14,45). It may develop before proptosis becomes apparent, and it may become quite marked (Figs. 42.5 and 42.12). Because the tarsus of the upper eyelid is thicker and firmer than the tarsus of the lower eyelid, the superior bulbar and palpebral conjunctiva usually remains nonchemotic, with the chemosis being limited to the interpalpebral bulbar conjunctiva and the inferior palpebral conjunctiva, regardless of the rate of blood flow through the fistula. In severe cases, the inferior palpebral conjunctiva may actually prolapse through the interpalpebral fissure (Figs. 42.13). If not kept well lubricated, the prolapsed conjunctiva may become necrotic and infected.

B
Figure 42.10. Chronic eyelid and facial changes in a patient with a longstanding left-sided, direct carotid-cavernous sinus fistula. A, Note prominent dilated subcutaneous eyelid and facial vessels, discolored dark appearance of affected eyelid, and redness and proptosis of left eye. B, Side view of patient shows dilation of large and small facial vessels.

Figure 42.12. Conjunctival chemosis in a patient with a right-sided direct carotid-cavernous sinus fistula. Note severe inferior chemosis.

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Although it initially may be mistaken for conjunctivitis or episcleritis, the peculiar dilation and tortuousity of the affected vessels usually is quite distinctive. The extent of arterialization of the conjunctival and episcleral veins is variable. It may be generalized or limited to only two or three vessels. Active arterial bleeding from such arterialized vessels rarely may occur (50). Ocular Pulsations Ocular pulsations are caused by transmission of the pulse waves from the internal carotid or ophthalmic artery to the ophthalmic veins. In patients with a direct CCF, the pulses on the side of the fistula invariably have increased amplitudes, and the resultant asymmetry of the pulses on the two sides may assist in diagnosis (discussed later) (14,51). Abnormal ocular pulsations may be visible or only palpable. Visible ocular pulsations usually are more readily detected from the side than from the front, but it is possible to overlook a pulsation that can be easily palpated. Palpable ocular pulsations are detected as a sensation of thrusts of blood passing in the vessels being felt, or as a heaving sensation of the eye against fingers that are placed on the closed eyelid overlying the affected eye. In fact, the increased pulse does not affect just the eye, but also the vessels of the eyelids, the orbit, and frequently the temporal fossa. Interestingly, most patients are not conscious of increased ocular pulsations, even when they are 6 mm or greater. Increased ocular pulsations probably develop as soon as a newly formed CCF begins to drain anteriorly. They may be detected within hours after trauma by observation, palpation, or other means. Applanation tonometry is an effective method of detecting increased ocular pulsations, even when such pulsations are not visible, and a pneumotonometer can be used to measure directly the abnormal ocular pulses, which may be 25 times those of the normal eye (Fig. 42.15) (51). Pulsating Exophthalmos When a direct, high-flow CCF drains anteriorly to the orbit, it may produce both visible ocular pulsation and proptosis: pulsating exophthalmos (45). In this setting, the exophthalmos almost always is associated with conjunctival chemosis, arterialization of conjunctival vessels, and a bruit that is audible to both the patient and the examiner (discussed later). When a patient with previous head trauma develops pulsating exophthalmos associated with these other signs, the diagnosis of direct, CCF usually is obvious. Pulsating exophthalmos can be caused by conditions other than a direct CCF, however. Both spontaneous, high-flow, dural CCFs (discussed later) and orbital arteriovenous fistulas occasionally produce pulsating exophthalmos indistinguishable from that caused by direct CCFs. In such cases, the correct diagnosis usually is not made until appropriate neuroimaging studies are performed. Other causes of pulsating exophthalmos include congenital absence of the sphenoid bone, as seen in patients with neurofibromatosis type 1, and acquired dehiscence of the frontal or sphenoid bones (e.g., after trauma). In these conditions, the cranial pulse is

Figure 42.13. Prolapse of inferior conjunctiva associated with proptosis and redness of right eye in a 24-year-old man with a right-sided direct carotid-cavernous sinus fistula following a motorcycle accident.

Arterialization of the Conjunctiva and Episcleral Veins As arterial blood is forced anteriorly into the orbital veins, the conjunctival and episcleral veins become dilated, tortuous, and filled with arterial blood (Fig. 42.14). This arterialization of conjunctival vessels is a hallmark of a CCF.

B
Figure 42.14. Dilated, tortuous, and arterialized conjunctival vessels in two patients with direct carotid-cavernous sinus fistulas. These vessels are typically bright red.

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Figure 42.15. Asymmetric ocular pulse amplitudes in a patient with a left-sided direct carotid-cavernous sinus fistula. Note that the intraocular pressure is higher in the eye with the larger pulse amplitude.

transmitted to the eye, which is also pushed forward by the frontal or temporal lobe (52,53). There is no associated bruit. An arteriovenous fistula between the internal carotid artery and the jugular vein produced pulsating exophthalmos in a patient described by Terry and Mysel (54). The fistula was in the upper part of the neck. At operation, the vein was dissected free from the artery. When both the vein and the common carotid artery were ligated, the ocular pulsation and an accompanying bruit disappeared. A traumatic middle meningeal arteriovenous fistula may also arterialize orbital veins and produce pulsating exophthalmos (55). Tumors in the orbit rarely produce pulsating exophthalmos. The tumors usually are vascular and slow growing (56). In such cases, ocular pulsation and bruit appear late, and the condition usually is not confused with a direct CCF. Corneal Damage Exposure keratopathy is the most frequent corneal sign encountered in patients with a direct CCF. It usually is related to the severity of proptosis, unless a traumatic facial nerve paresis also is present. The keratopathy may be aggravated by trigeminal neuropathy caused by the injury or by the effects of the fistula on the trigeminal nerve within the cavernous sinus (6). In rare cases, filamentary keratitis may develop in the setting of trigeminal sensory neuropathy, even though proptosis is not severe (Fig. 42.16).

Although exposure keratopathy is the most common corneal sign in patients with direct CCF, it is not the only sign. The cornea also may become turbid and hazy in patients with secondary glaucoma or anterior segment ischemia (discussed later). Bruit In many patients with a direct CCF, the initial symptom is a buzzing, swishing, or roaring sound that is synchronous with the heartbeat. This bruit (from the French word for noise) is principally systolic in timing, and it usually increases when the heart is beating actively; e.g., during exercise. The bruit usually decreases in amplitude when the patient is at rest or when the affected internal carotid artery is compressed, although failure of carotid compression to eliminate a bruit heard over the eye does not exclude the possibility of a CCF (57). A bruit present in a patient with a direct CCF may be heard only by the patient (subjective bruit) or by both the patient and the examiner (objective bruit). It is extremely unusual for a patient to be unaware of a bruit that can be heard by the examiner. Indeed, the bruit that results from a direct CCF is often a great source of annoyance (45), preventing the patient from working, relaxing, and even sleeping. A bruit that is produced by a direct CCF is best heard when a stethoscope is placed over the affected eye. Several authors have reported an objective bruit in every one of their patients with traumatic CCFs (13,14); other authors, however, have emphasized that not all patients with a direct CCF have a bruit, even if they have significant ocular symptoms and signs (49). It also must be emphasized that a bruit is not pathognomonic of a CCF. Bruits are common in otherwise healthy infants and young children (58), and they also may be heard in children with anemia or rickets or over the skull in some patients with increased intracranial pressure. A bruit also may represent the transmitted sound of a cardiac murmur, or it may be caused by a vascular anomaly in the orbit. Diplopia

Figure 42.16. Neovascularization of the cornea and filamentary keratitis (arrows) in a patient with a carotid-cavernous sinus fistula.

Diplopia occurs in about 6070% of patients with a direct CCF. The diplopia may be caused by dysfunction of one or more of the ocular motor nerves, the extraocular muscles,

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or both, and the degree of limitation of eye movement varies from mild limitation in only one direction to complete ophthalmoplegia. When a direct CCF is caused by trauma, the ocular motor nerves may be damaged at the time of initial injury, especially when the injury is severe enough to cause a basal skull fracture. Ophthalmoparesis usually is present immediately after injury in such patients, but it may not be appreciated if the patient is comatose or has substantial facial and orbital damage. When a direct CCF results from rupture of a cavernous aneurysm, ocular motor nerve paresis may occur before rupture from compression of one or more nerves, with the abducens nerve most often affected. In such cases, a careful history invariably indicates that diplopia began weeks to hours before the aneurysm ruptured. Ophthalmoparesis also may be caused by damage to one or more of the ocular motor nerves by the fistula itself. This damage may be caused by compression of the nerve(s) by the fistula, ischemia from alterations in the blood flow in the vasa nervorum of the ocular motor nerves, or both. In this setting, diplopia and ophthalmoparesis may not develop until several days to weeks after the development of other symptoms and signs of the fistula. Of the three ocular motor nerves, the abducens nerve is most often affected by a direct CCF (13,14,42,45). The abducens nerve may be the only ocular motor nerve affected (Fig. 42.17) (59), or it may be damaged along with one or both of the other ocular motor nerves. The particular vulnerability of the abducens nerve to damage from a CCF is related to its location within the cavernous sinus. Whereas the oculomotor and trochlear nerves are located in the deep layer of the lateral wall of the cavernous sinus, the abducens nerve is located within the body of the sinus, between the lateral wall of the cavernous segment of the internal carotid artery and the lateral wall of the sinus. The abducens nerve thus is more likely to be damaged by the hemodynamic and mechanical

changes that occur when the fistula develops. Among 33 patients with 34 traumatic, direct CCFs, Kupersmith et al. found abducens nerve paresis in 28 (85%) (13). Even though abducens nerve paresis is the most common ocular motor nerve paresis that occurs in patients with a direct carotid-cavernous fistula, either oculomotor or trochlear nerve paresis may develop in such patients, not only from the initial trauma but also as a direct result of the fistula itself. Kupersmith et al. reported oculomotor nerve paresis in 22 of 33 patients (67%) and trochlear nerve paresis in 17 of 33 patients (49%) with traumatic CCF, but it is not clear in what percentage of these cases only one nerve was affected (13). We have examined a young man with a direct CCF who had diplopia from an ipsilateral, isolated trochlear nerve paresis. The paresis resolved after the fistula was successfully treated. Mechanical restriction of the extraocular muscles, instead of, or in addition to, ocular motor nerve paresis, may cause diplopia in patients with a direct carotid-cavernous fistula. The restriction is the result of venous stasis and orbital edema, often with enlargement of the extraocular muscles themselves (6). In this setting, ophthalmoparesis and diplopia occur at the same time as, or shortly after, signs of orbital congestion. Patients with a direct CCF, ophthalmoparesis, and diplopia who do not have significant proptosis, chemosis, and orbital edema almost always have neuropathic limitation of eye movement. When signs of orbital congestion are prominent, it may be impossible to determine if ophthalmoparesis is neuropathic, myopathic, or both unless there is evidence of damage to the parasympathetic or sympathetic pupillomotor fibers. Even in this setting, limitation of eye movement may be both neuropathic and myopathic (Fig. 42.18). Visual Loss Visual loss associated with a direct CCF may be immediate or delayed. Immediate visual loss usually is caused by

Figure 42.17. Isolated abducens nerve paresis in a patient with a traumatic carotid-cavernous sinus fistula. The patient has mild abduction weakness of the left eye, but the rest of the eye movements were normal. The patients pupils were dilated with mydriatics before these photographs were taken.

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Figure 42.18. Combined mechanical and neuropathic limitation of eye movement in a patient with a traumatic, right-sided direct carotid-cavernous sinus fistula. The right eye is immobile. There is a complete right ptosis and anisocoria with the right pupil larger than the left, indicating damage to the right oculomotor nerve; however, the right eye also is substantially proptotic, and forced duction testing indicated some degree of mechanical limitation of eye movement.

ocular or optic nerve damage that occurs at the time of head injury (13,14). When visual loss is associated with a lacerated or ruptured globe, extensive arterial bleeding may complicate attempted repair or enucleation (60). Delayed visual loss usually is caused by retinal dysfunction, but it may be related to vitreous hemorrhage, anterior ischemic optic neuropathy, or even corneal ulceration (61,62). Dandy and Follis suggested that the optic nerve could be damaged in patients with long-standing fistulas from compression of the nerve by a distended cavernous sinus or from retrobulbar ischemia (6), and Hedges et al. described a patient in whom one of these two mechanisms was thought to be responsible for an optic neuropathy that occurred 7 weeks after development of a traumatic CCF (63). The optic neuropathy resolved after the fistula was successfully occluded. Visual loss is not uncommon in patients with a direct CCF. In the series reported by Sattler, 73% of patients had impaired vision, with almost 50% of those being blind or nearly so (42). Kupersmith et al. described visual loss in 16 of 33 patients (48%) with direct CCFs (13). The pathophysiology of visual loss from retinal or choroi-

dal dysfunction in patients with direct CCF is complex. Retinal and choroidal blood flow are reduced by: (a) a drop in effective ophthalmic artery perfusion pressure secondary to the hemodynamic alterations caused by the fistula, (b) an increase in venous pressure caused by arterialization of the orbital venous bed, and (c) obstruction or thrombosis of the ophthalmic venous system (64). The result is chronic hypoxia leading to choroidal effusion or detachment on the one hand, and stasis retinopathy, central retinal vein occlusion, or nonrhegmatogenous retinal detachment on the other. Many patients with a direct CCF initially complain of vague dimming of vision and an inability of the affected eye to adapt quickly to changes in brightness. In some patients, the peripheral visual field becomes constricted, although central vision usually is normal unless macular edema or choroidal detachment occurs. Ophthalmoscopic Abnormalities Dilation of retinal veins usually is present in patients with a direct CCF (Fig. 42.19). When the degree of dilation

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Figure 42.19. Unilateral dilation of retinal veins on the side of a direct carotid-cavernous sinus fistula. The patient was a 25-year-old woman with a traumatic, left carotid-cavernous sinus fistula. A, The ophthalmoscopic appearance of the right posterior pole is normal. The retinal vessels are of normal caliber. B, Ophthalmoscopic view of the left posterior pole shows moderate dilation of retinal veins unassociated with retinal hemorrhage.

is mild, it may not be appreciated during direct ophthalmoscopy, although it usually is obvious when indirect ophthalmoscopy is performed and the appearance of one ocular fundus is compared with the appearance of the other. In severe cases, optic disc swelling and retinal hemorrhages may occur in patients with a direct CCF (14). All of these mani-

festations are caused by venous stasis and impaired retinal blood flow, with secondary ischemia or hypoxia. Disc swelling usually is mild, but it may be severe. Retinal hemorrhages are usually flame-shaped (located in the nerve fiber layer) or punctate (located in the outer retinal layers) (Fig. 42.20), but in rare cases, subhyaloid (preretinal) or vitreous

Figure 42.20. Retinal hemorrhages and mild optic disc swelling in patients with direct carotid-cavernous sinus fistula. A, In one patient with a right-sided fistula, the posterior pole shows numerous flame-shaped (superficial) and punctate (deep) intraretinal hemorrhages. B, In another patient, the eye on the side of the lesion shows numerous intraretinal hemorrhages, most of which are of the punctate variety.

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A
Figure 42.21. Central retinal vein occlusion in a patient with a direct carotid-cavernous sinus fistula. A, Appearance of the patient. Note marked conjunctival chemosis and dilation of conjunctival vessels. B, The left ocular fundus shows a typical picture of a central retinal vein occlusion, with optic disc swelling, retinal hemorrhages and exudates, and dilated retinal veins.

hemorrhage is present (45). Thus, the ophthalmoscopic picture ranges from one of mild stasis retinopathy to one of frank central retinal vein occlusion (Fig. 42.21) (64). In extremely rare instances, nonrhegmatogenous retinal detachment (65) or choroidal effusion or detachment (64,66) develops. In such patients, successful treatment of the fistula is associated with spontaneous resolution of the retinal or choroidal process, often with improvement in visual function (64). Trigeminal Nerve Dysfunction Ocular and orbital pain rarely occur in patients with a direct CCF unless there is corneal exposure or ulceration. Nevertheless, occasional patients complain of facial pain in the distribution of the first, and rarely the second, division of the trigeminal nerve. Similarly, rare patients experience a decrease in corneal sensation, facial sensation, or both on the side of the fistula (45). Presumably, both pain and hypesthesia are related to ischemia or compression of the ophthalmic and maxillary divisions of the trigeminal nerve within the cavernous sinus. Glaucoma Glaucoma develops in 3050% of patients with a direct CCF and may be caused by several different mechanisms (14,45,45a). The most common cause is increased episcleral venous pressure (Fig. 42.22). Intraocular pressure in such cases usually is mildly elevated, but some patients have intraocular pressures as high as 5060 mm Hg (13). According to Weekers and Delmarcelle, intraocular pressure rises millimeter for millimeter with a corresponding increase in episcleral venous pressure in this type of glaucoma (67). Excessively high intraocular pressure may be associated with the development of central retinal artery occlusion. A second cause of glaucoma in patients with a direct CCF is orbital congestion, which usually occurs in association

with severe proptosis and chemosis. Intraocular pressure usually is quite high in such cases, and the elevated pressure may be difficult, if not impossible, to control. Neovascular glaucoma occurs in some patients with a direct CCF (Fig. 42.23). It is always associated with evidence of chronic retinal hypoxia and retinal neovascularization, and it is particularly common after central retinal vein occlusion (62). Finally, rare patients with a direct CCF develop angleclosure glaucoma without rubeosis iridis or neovascularization of the anterior chamber angle. In such patients, arterialization of orbital venous drainage channels causes elevated pressure in the vortex veins, central retinal vein, and episcleral vessels. Engorgement of the choroidal bed increases the volume of the posterior compartment, producing a forward shift in the iris-lens diaphragm. Hyperemia of the ciliary body and iris may contribute further to shallowing of the anterior chamber (68). DIAGNOSIS A direct CCF should be suspected in any patient who suddenly develops chemosis, proptosis, and a red eye. When these signs occur after a head injury, the diagnosis should be obvious, but even when there is no history of trauma, one should consider the possibility of a ruptured cavernous aneurysm, particularly if there is a history of preexisting diplopia. A patient suspected of harboring a direct CCF should be questioned about a subjective bruit, and careful auscultation of the eye and orbital region should be performed using the bell of the stethoscope. In addition, the intraocular pressure and ocular pulse should be measured. We have found the pneumotonometer to be an ideal instrument for performing such measurements (Fig. 42.15) (51). When a careful history and clinical examination support the diagnosis of a direct CCF, appropriate confirmation

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Figure 42.22. Chronic glaucoma from raised episcleral venous pressure in a patient with a right-sided, traumatic carotidcavernous sinus fistula. The patient had refused treatment of the fistula, and he was noncompliant with regard to antiglaucoma medication. Intraocular pressure in the right eye consistently measured 3035 mm Hg. A, External appearance of patients right eye shows minimal proptosis and several dilated, tortuous conjunctival veins. B, The left eye appears normal. C, Ophthalmoscopic view of the right optic disc shows moderate glaucomatous cupping. The cupdisc ratio is about 0.6. D, Ophthalmoscopic view of the left optic disc shows a normal optic disc with no significant cupping.

Figure 42.23. Neovascular glaucoma in a patient with a direct carotidcavernous sinus fistula. The patient has mild clouding of the cornea, an irregularly dilated pupil, and dilated iris vessels, along with the typical picture of dilated conjunctival and episcleral vessels.

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should be obtained. Computed tomographic (CT) scanning, CT angiography, magnetic resonance (MR) imaging, MR angiography, and orbital ultrasonography can be used to confirm the diagnosis, showing enlargement of extraocular muscles, dilation of one or both superior ophthalmic veins, enlargement of the affected cavernous sinus, and abnormal intracranial vessels (Fig. 42.24) (2,6972). Duplex carotid ultrasonography can be used to detect the fistula and to determine its flow rate (73), and transorbital or transcranial color Doppler imaging can also be used to show one or both enlarged superior ophthalmic veins within which the blood flow is toward, rather than away from the eye (74). The ultimate diagnostic test, however, is catheter angiography (Figs. 42.142.4 and 42.7). The angiographic technique should include selective catheterization of the ipsilateral common carotid artery, the ipsilateral internal and external carotid arteries, the contralateral internal carotid artery with compression of the ipsilateral internal carotid artery, and the dominant vertebral artery also with compression of the ipsilateral internal carotid artery. This techique will permit

the neurointerventionalist to determine the exact location and morphology of the fistula, the rate and direction of blood flow through the fistula, the anatomy of the venous drainage of the fistula, whether or not the fistula has produced a vascular steal phenomenon, the patency of the circle of Willis, and the anatomy of the carotid bifurcation on the side of the fistula (12). Only in this way can the optimum treatment of the fistula and the potential risks of such treatment be determined. NATURAL HISTORY Almost all patients will experience progressive ocular difficulties if a direct CCF is left untreated. Over months to years, there is increasing proptosis, chemosis, and visual loss, with the most severe complications being central retinal vein occlusion and secondary glaucoma. As noted previously, rhinologic and neurologic complications can occur in patients with direct CCFs. Such complications may occur at the time of the development of the fistula,

Figure 42.24. The results of diagnostic tests in patients with clinical evidence of a direct carotid-cavernous sinus fistula. A, Orbital ultrasonography (A mode) shows enlargement of the superior ophthalmic vein in cross section. B, CT, axial view, shows an enlarged left superior ophthalmic vein in a patient with a direct carotid-cavernous sinus fistula on that side. C, MRI, axial view, in a patient with a right-sided carotidcavernous sinus fistula shows an enlarged superior ophthalmic vein on the side of the fistula. The vein appears as a black curvilinear structure beginning at the apex of the orbit and continuing forward in the superolateral orbit as a curvilinear tubular structure (horizontal arrowhead). A small portion of the nasal component of the vein is also seen (vertical arrowhead).

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shortly thereafter, or months to years later. The most significant of these complications are epistaxis, subarachnoid hemorrhage, intracerebral hemorrhage, and cerebral infarction (13,3941,44). TREATMENT The optimum treatment of a direct CCF is closure of the abnormal arteriovenous communication with preservation of internal carotid artery patency. A variety of procedures can be used to close the fistula, but many (e.g., ligation of the internal carotid artery, occlusion of the fistula using the nondetachable balloon tip of a Fogarty catheter, trapping of the fistula by occlusion of the artery both proximal and distal to the fistula) also occlude the internal carotid artery (75). When the artery is occluded, the patient may suffer extensive neurologic deficits from hypoxic damage to the ipsilateral cerebral hemisphere. In addition, occlusion of the internal carotid artery may so reduce arterial blood flow to the eye that the patient develops hypotony, proliferative retinopathy, neovascular glaucoma, blindness, and even devastating ischemic necrosis of the eyelids and orbital contents (Fig. 42.25) (61,62,76,77). Several techniques permit successful closure of a direct carotid-cavernous fistula without occlusion of the internal carotid artery (4,5,78). Direct obliteration of the fistula can be performed by surgical repair of the damaged portion of the cavernous internal carotid artery (7,16,7880). This procedure can be difficult to perform, requires a craniotomy, and has significant potential morbidity. Nevertheless, it may be successful in closing the fistula without sacrificing the internal carotid artery, particularly when combined with intraoperative angiography and temporary balloon occlusion of the internal carotid artery (79). This technique usually is used when endovascular coil or balloon embolization of the fistula (discussed later) cannot be performed for technical reasons (16,79). Endovascular closure of direct CCFs is most often accomplished by embolization using a variety of agents, primarily platinum coils and detachable balloons (5,25,25ac). These materials usually are introduced into the cavernous sinus through the internal carotid artery, but in selected cases, they may be introduced either transvenously through the inferior petrosal sinus, pterygoid plexus, or the superior ophthalmic vein, or directly into the cavernous sinus via a craniotomy, transethmoidal transsphenoidal approach, or even a direct puncture through the superior orbital fissure (8186). The most common coils used for closure of a direct CCF are platinum detachable coils. Some of these coils are straight except for a curved end. Others have complex shapes, but they can be straightened so that they can be delivered via a microcatheter that is advanced up the internal carotid artery, through the hole in its wall, and into the cavernous sinus or can be placed in the superior ophthalmic vein and advanced directly into the cavernous sinus. Once the coils are released, they revert back to their original shape (Fig. 42.26), thus promoting thrombosis in the area of the fistula. Detachable coils also can be used to close direct fistulas involving the cavernous sinus that occasionally arise

Figure 42.25. Ischemic ocular necrosis before and after attempted treatment of a direct carotid-cavernous sinus fistula. The patient was a 39-yearold man who had suffered multiple fractures including a basal skull fracture when he was struck by an automobile. Shortly thereafter, he developed marked proptosis of the right eye associated with swelling of the eyelids and chemosis of the conjunctiva. Arteriography showed a right-sided, direct carotid-cavernous sinus fistula. A, Preoperative appearance of the patient shows patchy necrosis of the eyelids, conjunctiva, and cornea (arrow). About 3 weeks after injury, the patient underwent attempted treatment of the fistula. The right internal carotid artery was clipped above the fistula, muscle emboli were introduced into the cavernous segment of the vessel, and both the right internal and right external carotid arteries were ligated in the neck. B, After surgery, the lid edema and erythema are reduced, but the cornea is completely opacified and the conjunctiva is sloughing. C and D, Progressive necrosis, perforation, and sloughing of the cornea 7 and 9 days after operation. (From Spencer WH, Thompson HS, Hoyt WF. Ischaemic ocular necrosis from carotid-cavernous fistula: Pathology of stagnant anoxic inflammation in orbital and ocular tissues. Br J Ophthalmol 1973; 57145152.)

from other intracranial arteries, such as the posterior communicating artery (87). Detachable, flow-guided balloons are used to close many direct CCFs, particularly those that originate from aneurysms (Fig. 42.27) (88). The balloon is attached to the end of a catheter that usually is advanced up the ipsilateral internal carotid artery and into the cavernous sinus through the tear in the cavernous portion of the vessel (3,5,16,8890). Alternatively, the balloon can be placed in the cavernous sinus by a venous route using the inferior petrosal sinus, pterygoid plexus, or superior ophthalmic vein (88,9193). In rare

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B
Figure 42.26. Detachable platinum coil system. A, Appearance of a typical coil. Note that the coil is straight within the catheter but regains its normal curved structure when it is outside the catheter. B, Diagram of a Guglielmi detachable coil and of a tracker catheter with two tip markers: A, circular memory diameter; B, platinum portion of the coil (0.01 or 0.015 inch in diameter; 440 cm in length when straightened); C, solder junction between platinum coil and stainless steel delivery wire; D, detachable portion of coil; E, proximal radiopaque marker on the delivery wire; F, teflon lamination; G, delivery wire (0.01 inch); H, catheter distal marker; I, catheter proximal marker; J, catheter shaft. (From Guglielmi G, Vin uela F. Intracranial aneurysms. Neurosurg Clin North Am 1994;5:427435.)

cases, the cavernous sinus can be approached surgically, and the balloon placed directly in the sinus (94). Once the balloon is in proper position in the cavernous sinus, it is inflated and detached, occluding the fistula but leaving the internal carotid artery patent (Fig. 42.28). Some fistulas are so large that more than one balloon must be used to occlude them

Figure 42.27. Photograph of inflated detachable balloon still attached to catheter.

(95). Although there are some patients in whom balloon occlusion of the fistula also results in occlusion of the internal carotid artery, this complication is uncommon (3,16,96) and is related in large part to the experience of the interventional neuroradiologist. Indeed, both the rates of success and complication of this procedure are related primarily to the precise technique used and the skill of the interventional neuroradiologist. Experienced investigators have reported success rates of 90100% with complication rates of 25% (25,16,93,96). Complications most often result after balloon occlusion of a direct CCF when there is planned or unplanned occlusion of the internal carotid artery. These complications, primarily those related to reduction or interruption of blood supply to the ipsilateral eye and cerebral hemisphere, include stroke and even death. Even when the fistula is successfully closed and the internal carotid artery remains patent, as occurs in over 80% of cases, complications may result. The most common are transient worsening of ocular signs, particularly proptosis, ophthalmoparesis, and pain (2,13). These side effects usually resolve within several weeks. New neurologic deficits, particularly ocular motor nerve pareses, may also develop after otherwise successful occlusion of a direct CCF (9799). Abducens and oculomotor nerve pareses are especially common. Ocular motor nerve pareses usually develop within a few hours after closure of the fistula. These pareses are caused in most cases by edema within the cavernous sinus or from interruption of the normal blood supply in the vasa nervorum (100), but other mechanisms are occasionally responsible. Regardless of the pathophysiology, most ocular motor pareses that occur after otherwise successful closure of a direct CCF resolve within days to weeks. When they do not do so, consideration may be given to deflating the balloon using an endovascular approach (99). In rare cases, sepsis occurs in otherwise successfully treated patients with direct fistulas. This complication can produce severe neurologic and systemic deficits leading to permanent neurologic disability or even death (3). Partial closure of a fistula may cause severe and potentially life-threatening complications. If the anterior drainage of the fistula is successfully blocked but the fistula remains open, it may drain posteriorly into cortical veins, resulting in arterialization of these vessels. This can lead to intracerebral hemorrhage if the persistent fistula is not recognized and treated. Halbach et al. reported a variety of complications after embolization procedures for direct CCFs associated with Ehlers-Danlos syndrome, including neck hematoma and fatal pontine hemorrhage (101). These authors attributed the complications to increased vascular fragility in this disease. Debrun et al. also emphasized that the treatment of direct CCFs associated with Ehlers-Danlos syndrome is more difficult and more risky than is the treatment of most direct CCFs (36). When treatment of a direct CCF sacrifices internal carotid artery patency (e.g., ligation of the internal carotid artery) but fails to occlude the fistula, a transvenous approach to the fistula through either the inferior petrosal sinus or the superior ophthalmic vein should be attempted (78,93). This

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Figure 42.28. Preoperative and postoperative angiographic appearance of a direct carotid-cavernous sinus fistula after closure of the fistula with a detachable balloon. A, Selective left internal carotid arteriogram shows a direct fistula (arrow) with drainage anteriorly into the left superior ophthalmic vein (arrowheads). B, After balloon occlusion of the fistula, the appearance of the left internal carotid artery has become normal, and there is no longer any abnormal drainage anteriorly. Note that the fistula is obliterated, and the internal carotid artery is preserved.

treatment usually is successful and should be attempted before resorting to more invasive procedures such as a direct approach to the cavernous sinus. On rare occasions, a balloon placed in the cavernous sinus to occlude a direct fistula subsequently will migrate distally. Patients in whom this occurs may develop a variety of neurologic deficits, including hemiparesis and diplopia. In such cases, it may be appropriate to consider endovascular deflation of the balloon under imaging guidance (102). In view of the highly successful and reasonably safe treatment available for a direct CCF, it is unusual for any of the visual complications of such a lesion to require treatment.

Nevertheless, glaucoma may require treatment with topical or oral pressure-lowering agents, at least until definitive therapy of the fistula can be performed. Similarly, proliferative retinopathy, central retinal vein occlusion, and neovascular glaucoma that result from delayed treatment of the fistula may require medical therapy, photocoagulation, or both (2). PROGNOSIS AFTER TREATMENT After attempted occlusion of a direct CCF using one or more detachable balloons or platinum coils, closure of the fistula should be verified by either intraoperative or postop-

Figure 42.29. Improvement in intraocular pressure and ocular pulse amplitude after balloon occlusion of a left-sided, traumatic, direct carotid-cavernous sinus fistula. Left, Before treatment of the fistula, the intraocular pressure is about 30 mm Hg in the left eye compared with 15 mm Hg in the right eye. The amplitude of the ocular pulse in the left eye is about 23 times that of the pulse in the right eye. Right, after treatment, the intraocular pressure in the left eye has decreased to about 25 mm Hg, and the ocular pulse amplitudes of the two eyes are symmetric.

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erative angiography (Fig. 42.28). The ocular pulse amplitudes of the two eyes can also be compared using a pneumotonometer (51). Equalization of ocular pulse amplitudes or reduction of the ocular pulse amplitude on the side of the fistula below that of the opposite side suggests that the fistula has been closed successfully (Fig. 42.29); however, normalization of the ocular pulse amplitudes could also occur if the fistula were still open but no longer draining anteriorly. In such a case, the fistula might be filling superficial cerebral veins, and the patient might be at risk for experiencing a potentialy life-threatening intracranial hemorrhage. Thus,

we believe that all patients who undergo attempted occlusion of a direct CCF should have post-treatment cerebral angiography. When angiography indicates complete closure of the fistula with preservation of patency of the internal carotid artery, the fistula usually does not reopen (103). Once a fistula is successfully closed, most of the ocular symptoms and signs resolve or at least improve and do not recur (13,14,103). The rate and extent of improvement are related in part to the severity of the signs and in part to the length of time that the fistula was present. In almost all patients, successful closure of a direct CCF is associated with

C
Figure 42.30. Rapid improvement in ocular manifestations after successful balloon occlusion of direct carotid-cavernous sinus fistulas. A, Preoperative appearance of a 16-year-old boy who developed a left-sided carotid-cavernous sinus fistula after a motor vehicle accident. The left eye is proptotic and red, there is moderate left ptosis, and there is limitation of eye movement in all directions. B, Twenty-four hours after successful balloon occlusion of the fistula, the patients eye is already less red and proptotic, the left upper eyelid is less ptotic, and eye movement is improved. C, Preoperative appearance of a 44-year-old woman with a left-sided carotid-cavernous sinus fistula that was initially mistaken for a cavernous sinus thrombosis. The patient has severe left upper and lower eyelid swelling, redness and proptosis of the left eye, and almost complete ophthalmoparesis. D, Five days after successful balloon occlusion of the fistula, there is marked improvement in the appearance of the eyelids, reduction in proptosis and conjunctival chemosis, and improvement in movement of the left eye.

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immediate disappearance of a preexisting bruit, increased ocular pulsations, and thrill (13). Other signs likely to resolve after treatment are eyelid engorgement, conjunctival chemosis, dilated conjunctival vessels, stasis retinopathy, disc swelling, and elevated intraocular pressure (13,14). These signs may begin to improve within days after treatment (Fig. 42.30), but complete resolution may take months (Fig. 42.31). Proptosis usually improves but may never completely resolve. Similarly, patients with ophthalmoparesis

may have complete return of ocular motility and resolution of diplopia in days to weeks if their eye movement difficulties have been primarily caused by the mechanical effects of orbital congestion or if they have mild neuropathic limitation of eye movement. In patients with long-standing or severe single or multiple ocular motor nerve pareses (particularly when the pareses were caused by the injury and not the fistula per se), improvement in ocular motility may not improve for months after closure of the fistula, and some of

Figure 42.31. Long-term resolution of ocular manifestations of a direct carotid-cavernous sinus fistula after successful closure of the fistula with preservation of the ipsilateral internal carotid artery. A, Preoperative appearance of a 27-year-old woman with a traumatic right-sided carotid-cavernous fistula. The patient has moderate right proptosis and redness of the right eye. She also had generalized limitation of eye movement. B, Three months after successful balloon occlusion of the fistula, the right eye appears normal. There is no longer any redness or proptosis, and the eye moves normally in all directions. C, Preoperative appearance of a 21-year-old Asian woman who developed a left-sided, direct carotid-cavernous sinus fistula after an automobile accident. The patient has moderate proptosis and redness of the left eye. She also had intermittent horizontal diplopia. D, Two months after successful balloon occlusion of the fistula via the superior ophthalmic vein, the left eye appears normal. Both the redness and proptosis have completely resolved. The patient no longer has diplopia.

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these patients have permanent ophthalmoparesis and diplopia requiring prism therapy, strabismus surgery, or occlusion of one eye (59). Of all the symptoms and signs of direct CCF, visual loss is least likely to improve after successful treatment of the fistula, even when the internal carotid artery remains patent (13). Patients whose visual loss is caused by choroidal pa-

thology (e.g., choroidal effusion or detachment) are more likely to experience visual improvement than are patients whose visual loss is caused by optic neuropathy or primary retinal dysfunction (64). Nevertheless, even patients with severe visual loss caused by optic neuropathy or retinopathy may experience marked improvement in vision after treatment (63,64,95,104,105).

DURAL CAROTID-CAVERNOUS SINUS FISTULAS (DURAL ARTERIOVENOUS MALFORMATIONS)


The blood supply to the region of the cavernous sinus is provided by interconnecting branches of the internal and external carotid arteries, and it is from these vessels that dural CCFsoften called dural arteriovenous fistulas arise. Such fistulas usually are separated anatomically into three types: (a) shunts between meningeal branches of the internal carotid artery and the cavernous sinus, (b) shunts between meningeal branches of the external carotid artery and the cavernous sinus, and (c) shunts between meningeal branches of both the internal and external carotid arteries and the cavernous sinus (Figs. 42.242.4) (1,3). Of these types, the third is by far the most common. PATHOGENESIS Dural CCFs usually become symptomatic spontaneously. The pathogenesis of these fistulas is controversial (3). Newton and Hoyt speculated that spontaneous dural CCFs form after rupture of one or more of the thin-walled dural arteries that normally traverse the cavernous sinus (106). After rupture, extensive preformed dural arterial anastomoses not directly involved in the fistula may dilate and contribute collateral blood supply, resulting in an angiographic appearance indistinguishable from a congenital vascular malformation. Indeed, sequential arteriography demonstrates that the feeder vessels of dural CCFs change with time as the vessels spontaneously open and close (107). Although the theory of Newton and Hoyt is favored by some investigators (1), it fails to explain why spontaneous dural CCFs are more common in elderly women than in men. A second theory for the origin of dural CCFs was espoused by Houser et al., who suggested that most of these lesions develop in response to spontaneous venous thrombosis in the cavernous sinus in an attempt to provide a pathway for collateral venous outflow (108). This theory is favored by most investigators because it also explains the pathogenesis of arteriovenous fistulas that develop in the sigmoid and other dural sinuses (3). Certain factors may predispose to the development of symptomatic dural CCFs. These include pregnancy, systemic hypertension, atherosclerotic vascular disease, connective tissue disease (e.g., Ehlers-Danlos syndrome), and minor trauma (Fig. 42.32) (5,109,110). Iatrogenic dural carotid-cavernous fistulas occasionally occur. Borden and Liebman reported the case of a woman who developed such a fistula following attempted transsphenoidal removal of a recurrent pituitary macroadenoma (111). CLINICAL MANIFESTATIONS Dural CCFs usually occur in middle-aged or elderly women, but they may produce symptoms at any age, even in childhood or infancy, in both sexes (112). The symptoms and signs produced by these lesions are influenced by a number of factors, including the size of the fistula, location within the cavernous sinus, rate of flow, and especially whether drainage is posterior, anterior, or both (113,114). The route of drainage of the fistula is probably related to its basic anatomic configuration, although Grove postulated that many, if not all, fistulas initially drain posteriorly into the inferior petrosal sinus, basilar venous plexus, or both (115). He believed that when this normal pathway for drainage becomes thrombosed, the fistula begins to drain anteriorly, producing visual symptoms and signs. We and others have seen patients whose clinical course suggests that this theory is correct (116). Such patients initially may experience an acute isolated ocular motor nerve paresis, the evaluation of which reveals a posteriorly draining fistula (Fig. 42.33). Shortly thereafter, the patients develop typical signs of an anteriorly draining fistula (Fig. 42.34). Posteriorly Draining Fistulas When dural CCFs drain posteriorly into the superior and inferior petrosal sinuses, they are usually asymptomatic. In some cases, however, such fistulas produce a cranial neuropathy, such as a trigeminal neuropathy (117), facial nerve

Figure 42.32. External appearance of a 39-year-old woman with EhlersDanlos syndrome who developed spontaneous bilateral dural carotidcavernous sinus fistulas. The fistulas were successfully closed using an endovascular approach, but the patient died several months later from unrelated vascular complications of the underlying disease.

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Figure 42.33. Posteriorly draining dural carotid-cavernous sinus fistula (shunt) causing an acute, painful oculomotor nerve paresis. The patient was a 58-year-old woman who developed an acute right-sided fronto-orbital headache. Four weeks later she developed diplopia, and 7 days afterwards she developed right ptosis and a dilated right pupil. She was thought to have an intracranial aneurysm, and an arteriogram was performed. Selective right carotid arteriogram, lateral view, shows a dural arteriovenous fistula of the cavernous sinus (arrows) that drains posteriorly into the inferior petrosal sinus (arrowheads). (From Hawke SH, Mullie MA, Hoyt WF, et al. Painful oculomotor nerve palsy due to dural-cavernous sinus shunt. Arch Neurol 1989;4612521255.)

paresis (118), or an ocular motor nerve paresis (119). In most of these cases, there is no evidence of orbital congestion, although the patient reported by Moster et al. also had ocular symptoms and signs caused by simultaneous anterior drainage of the fistula (118). In most cases of ocular motor nerve paresis caused by a posteriorly draining dural CCF, the onset of the paresis is sudden, and only one of the ocular motor nerves is affected. The oculomotor nerve is most often affected, and the resulting paresis may be complete with involvement of the pupil, incomplete with pupil involvement, or incomplete with pupil sparing. We have never seen a patient with a complete, pupilsparing oculomotor nerve paresis in this setting. In almost all cases, the paresis is associated with ipsilateral orbital or ocular pain, a presentation that initially suggests an intracranial aneurysm (Fig. 42.35) (116,120). The correct diagnosis in such cases is not evident until cerebral angiography is performed. In other cases, the posteriorly draining fistula produces an abducens or trochlear nerve paresis, again usually associated with ocular or orbital pain (Fig. 42.36) (106,119,121,122). The cranial neuropathies that are caused by a posteriorly draining dural CCF usually are the initial sign of the fistula. In many of these cases, failure to diagnose and treat the fistula leads eventually to a change in the direction of the flow of blood in the fistula. The flow becomes anterior, and the patient develops evidence of orbital congestion. In other cases, the blood flow in the fistula initially is anterior, producing orbital manifestations. With time, however, the ante-

Figure 42.34. Cerebral angiography in the patient described in Figure 42.33 several weeks later after the patient developed redness, conjunctival chemosis, and proptosis of the right eye. Repeat selective right internal carotid arteriogram, lateral view, shows that the dural carotid-cavernous sinus fistula (arrows) now drains anteriorly into the superior ophthalmic vein (arrowheads) rather than posteriorly. (From Hawke SH, Mullie MA, Hoyt WF, et al. Painful oculomotor nerve palsy due to dural-cavernous sinus shunt. Arch Neurol 1989;4612521255.)

Figure 42.35. Selective left internal carotid arteriogram, lateral view, in an 86-year-old woman with facial pain and acute binocular diplopia. The examination revealed a left partial third nerve palsy with involvement of the pupil. The patient had no signs or symptoms of orbital disease. The angiogram shows a large dural carotid-cavernous sinus fistula (arrowhead) that drains posteriorly into the inferior petrosal sinus rather than anteriorly into the orbit. (From Lee AG. Neuroophthalmology 1996;16183187.)

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tulas, there often is no objective or subjective bruit, and even when a subjective bruit is present, the patient may not mention it, either because it is mild or because the patient does not associate the sound with his or her ocular symptoms and signs. In the mildest cases, there is redness of one or, rarely, both eyes caused by dilation and arterialization of both conjunctival and episcleral veins (Fig. 42.37). The appearance in these cases may suggest conjunctivitis, episcleritis, or thyroid eye disease; however, careful examination of the dilated vessels usually demonstrates a typical tortuous corkscrew appearance that is virtually pathognomonic of a dural CCF (Fig. 42.38). There also may be minimal eyelid swelling, conjunctival chemosis, proptosis, or a combination of these findings. Diplopia from abducens nerve paresis may be present (Fig. 42.39). Ophthalmoscopy may be normal, or there may be mild dilation of retinal veins. In more advanced dural CCFs, particularly those with a high flow rate, the symptoms and signs are identical with

Figure 42.36. Left common carotid arteriogram, lateral view, in a 44year-old woman with a severe headache and horizontal diplopia. The examination revealed a left sixth nerve palsy. The arteriogram shows a dural carotid-cavernous sinus fistula (arrowhead) draining posteriorly into the inferior petrosal sinus. (From Eggenberger E, Lee AG, Forget TR Jr, Rosenwasser R. A bruital headache and double vision. Surv Ophthalmol 2000; 45147153.)

rior drainage ceases, and posterior flow is associated with the development of the cranial neuropathy. Dural fistulas that drain posteriorly may cause brainstem congestion that may be associated with neurologic deficits (123). In addition, such fistulas rarely may produce intracranial hemorrhage (124). Anteriorly Draining Fistulas Like their direct counterparts, dural CCFs usually produce visual symptoms and signs when they drain anteriorly into the superior and inferior ophthalmic veins. The clinical manifestations of patients with dural carotid-cavernous fistulas that drain anteriorly are therefore similar to, but usually much less severe, than those of patients with direct fistulas, because most dural fistulas contain blood flowing at a low rate. Indeed, dural fistulas usually produce an important and rather characteristic syndrome that, nevertheless, often is misdiagnosed (2,106,113115,125128). Unlike direct fisB
Figure 42.37. Appearance of two different patients with spontaneous dural carotid-cavernous sinus fistulas. A, A 58-year-old woman with mild monocular redness of the right eye with minimal fullness of the eyelids. This appearance is often mistaken for conjunctivitis. B, A 61-year-old man with moderate redness of the left eye associated with mild proptosis of the eye. This appearance often is mistaken for episcleritis or dysthyroid orbitopathy.

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A
Figure 42.38. Appearance of conjunctival and episcleral vessels in two patients with spontaneous, dural carotid-cavernous sinus fistulas. Note dilation, tortuosity, and corkscrew appearance of the veins.

Figure 42.39. Abducens nerve paresis in a 34-year-old woman with a left-sided dural carotid-cavernous sinus fistula. A, The left eye adducts fully on attempted right lateral gaze. B, When the patient attempts to look to the left, the left eye abducts only to just beyond the midline. Note the mild left proptosis and the dilated conjunctival veins of the left eye.

Figure 42.40. Appearance of a right-sided, high-flow, dural carotid-cavernous sinus fistula in a 54-year-old man. The right eye is moderately proptotic, and there is significant chemosis of the conjunctiva. The appearance of this patient is indistinguishable from that of a patient with a high-flow, direct carotid-cavernous sinus fistula.

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those in patients with a direct CCF (45a,113,114,129). In these cases, proptosis, chemosis, and dilation of conjunctival vessels are obvious (Fig. 42.40). Diplopia may result from ophthalmoparesis caused by ocular motor nerve paresis, orbital congestion, or both, and it may be painful, initially suggesting an orbital inflammatory process or even the Tolosa-Hunt syndrome (130,131). Some patients develop facial pain, facial weakness, or both (131a). Raised episcleral venous pressure may produce increased intraocular pressure that occasionally can be quite high (45a,125,126,132). Angle-closure glaucoma may develop from elevated orbital venous pressure, congestion of the iris and choroid, and forward displacement of the iris-lens diaphragm (133). Ophthalmoscopic abnormalities include venous stasis retinopathy with intraretinal hemorrhages, central retinal vein occlusion, proliferative retinopathy, retinal detachment, vitreous hemorrhage, choroidal folds, choroidal effusion, choroidal detachment, optic disc swelling, and neovascular or angle-closure glaucoma (Fig. 42.41) (64,134136). Visual loss, although less frequent than in patients with direct carotid-cavernous fistula, occurs in 2030% of patients with dural CCFs (106,115). It may be caused by ischemic optic neuropathy, chorioretinal dysfunction, or uncontrolled glaucoma (64,125). The ocular manifestations of unilateral dural CCFs almost always are ipsilateral to the fistula, but they may be purely contralateral or even bilateral (Fig. 42.42) (113,114). When unilateral fistulas cause bilateral manifestations, there is a high probability that the fistula is draining into cortical veins (Fig. 42.43) (114). In rare cases, patients have bilateral dural carotid-cavernous fistulas. Most of these cases are associated with bilateral orbital manifestations; however, we examined a patient with bilateral fistulas that caused only left-sided

Figure 42.42. Bilateral ocular manifestations in a patient with a rightsided spontaneous dural carotid-cavernous sinus fistula. The patient has bilateral red eyes, with dilated, tortuous conjunctival and episcleral veins.

ocular manifestations. The left-sided fistula drained anteriorly into the left orbit via the left superior ophthalmic vein. The right-sided fistula drained across the intercavernous sinus and then anteriorly into the left orbit. Although most dural fistulas are unilateral, bilateral spontaneous dural fistulas have been described (137). Patients with bilateral dural CCFs often have severe systemic hypertension, atherosclerosis, or some type of systemic connective tissue disease, particularly Ehlers-Danlos syndrome. In some instances, dural CCFs drain both anteriorly and posteriorly. In most of these cases, the only manifestations are those related to the anterior drainage; however, some

A
Figure 42.41. Central retinal vein occlusion in a 62-year-old woman with a spontaneous, dural carotid-cavernous sinus fistula. A, External appearance shows moderate proptosis of the left eye, associated with conjunctival chemosis and arterialization of conjunctival and episcleral vessels. The patient noted progressive visual loss in the left eye over several days. B, Ophthalmoscopic appearance of left ocular fundus shows changes consistent with a mild central retinal vein occlusion. The left optic disc is normal, but the retinal veins are dilated, and there are numerous intraretinal dot and blot hemorrhages. The visual acuity in this eye was 20/80, but it improved to 20/20 as the hemorrhages cleared spontaneously.

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Figure 42.45. Abducens nerve paresis in a 65-year-old woman with a right-sided, dural carotid-cavernous sinus fistula. The patient had systemic hypertension and presented with acute horizontal diplopia. She initially was thought to have experienced a vasculopathic sixth nerve paresis; however, on a follow-up examination 6 weeks later, it was noted that the conjunctival and episcleral veins of the right eye were dilated and tortuous compared with those of the left eye. Figure 42.43. Selective left internal carotid arteriogram shows a dural carotid-cavernous sinus fistula (single arrowhead) with drainage into the superior ophthalmic vein (double arrowheads) and also into several cortical veins (triple arrowheads).

patients develop manifestations from the posterior drainage, including facial nerve paresis and acute hemiparesis associated with neuroimaging evidence of brainstem congestion (118,138). Because the symptoms and signs of a dural CCF often are mild, usually developing spontaneously and rather slowly, patients with this lesion often are misdiagnosed initially. When the patient simply has a red eye, perhaps with minimal eyelid swelling, he or she may be thought to have a chronic

conjunctivitis or blepharoconjunctivitis that is refractory to topical therapy (Fig. 42.44). In patients who develop diplopia from abducens nerve paresis, the significance of a slightly red eye may be missed (Fig. 42.45). In patients with evidence of orbital congestion, red eye, conjunctival chemosis, etc., diagnoses other than a spontaneous dural CCF, such as dysthyroid orbitopathy, orbital pseudotumor, orbital cellulitis, episcleritis, spheno-orbital meningioma, or even Tolosa-Hunt syndrome, may be considered (106,115,125,130,131,139). The correct diagnosis in such cases may then not be made until symptoms and signs worsen, new symptoms and signs develop, or appropriate diagnostic studies are performed. Conversely, because the disorders mentioned previously often produce clinical manifestations similar to those produced by a CCF, they may be the cause of symptoms and signs initially attributed to a CCF. In addition, trauma to the posterior orbit in the region of the superior orbital fissure may produce such manifestations (140) and we and others have seen patients with congenital or acquired anomalous intracranial venous drainage who developed clinical manifestations suggesting a CCF (141). The correct diagnosis in almost all cases is made by appropriate neuroimaging studies, particularly cerebral angiography (discussed later). DIAGNOSIS The diagnosis of a dural CCF should be considered in any patient who spontaneously develops a red eye, chemosis of the conjunctiva, abducens nerve paresis, or mild orbital congestion with proptosis. Auscultation of the orbit may disclose a bruit, but this is relatively uncommon. Tonometry, however, usually shows asymmetry of the ocular pulse with a greater pulse amplitude on the side of the lesion. The asymmetry in the amplitude of the ocular pulse can be appreciated

Figure 42.44. Spontaneous dural carotid-cavernous sinus fistula mistaken for chronic conjunctivitis in a 54-year-old woman. Note redness and minimal swelling of the left eye without proptosis or chemosis.

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using any tonometer, although we prefer to use a pneumotonometer which provides a direct measurement and an objective record of the pulse (51). When a dural CCF is suspected, CT scanning, CT angiography, MR imaging, MR angiography, orbital ultrasonography, duplex carotid sonography, transorbital and transcranial color Doppler imaging, or a combination of these tests may be of benefit in confirming the diagnosis (72,74,142144). The gold standard diagnostic test, however, as in the case of the direct CCF, is a catheter angiogram (3,145). Because many dural CCFs are fed either by meningeal branches of the external carotid artery or by meningeal branches of both the internal and external carotid arteries, and others are fed by arteries from both sides or are fed by unilateral arteries but produce bilateral symptoms and signs, selective angiography of both internal and external carotid arteries on both sides should always be performed (145). Such angiography has a relatively low morbidity and mortality that is less than 1% in most centers, except in patients with connective tissue disorders such as Ehlers-Danlos syndrome in whom the risks are much greater because of excessive fragility of the extracranial and intracranial vessels. Indeed, Schievink et al. reported a morbidity rate of 36% and a mortality rate of 12% for diagnostic angiography in 17 patients with a dural CCF in the setting of Ehlers-Danlos syndrome (109). NATURAL HISTORY The majority of patients with a dural CCF have no difference in mortality from that of the normal population, because the lesion usually affects only the eyes. Spontaneous intracranial hemorrhage is exceptionally rare (124). Thus, when one considers the natural history of a dural CCF, one is really considering the ocular morbidity. Regardless of whether they drain anteriorly or posteriorly,

2050% of dural CCFs close spontaneously after angiography, or after air flight travel (Figs. 42.39 and 42.46) (146). In some cases, the symptoms and signs begin to resolve within days to weeks after angiography. In others, they do not resolve until months to years after the fistula has become symptomatic. We believe it is appropriate to clinically follow patients who have mild ocular manifestations to see if the fistula will close spontaneously. During the waiting period, we and others advise patients not to alter their lifestyle. Patients being followed should be examined at regular intervals so that their visual function, intraocular pressure, and ophthalmoscopic appearance can be monitored (147). Patients whose fistulas do not close spontaneously occasionally lose vision. In most of these patients, visual loss results from a central retinal vein occlusion (Fig. 42.42) (148). Although patients with dural CCFs often have increased intraocular pressure, they rarely develop glaucomatous optic nerve damage. Persistent proptosis, chemosis, and redness of the eye may be associated with significant irritation and epiphora from exposure keratopathy that may require topical therapy. Limitation of eye movement from ocular motor nerve paresis, orbital congestion, or both may produce persistent, bothersome diplopia necessitating prism therapy or occlusion of one eye. Patients with a dural CCF may experience acute worsening of ocular manifestations. The clinical deterioration results from an increase in blood flow through the fistula in some cases, but in others, it is caused by spontaneous thrombosis of the superior ophthalmic vein (51,149). Patients in whom clinical worsening is caused by spontaneous progressive thrombosis of the superior ophthalmic vein usually begin to improve within several weeks, and most eventually experience complete resolution of symptoms and signs (Fig.

Figure 42.46. Spontaneous closure of a dural carotid-cavernous sinus fistula after cerebral angiography. The patient was a 59-year-old man who developed progressive redness and swelling of the right eye. A, The patients right eye is swollen and red. There is significant conjunctival chemosis. Ocular pulse amplitudes were asymmetric, with the higher pulse on the right side. Cerebral angiography confirmed a right-sided dural carotid-cavernous sinus fistula fed by branches of the right internal and external carotid arteries. It was elected to follow the patient without intervention. Within 1 week after the angiogram, the patient began to experience reduction in swelling and redness of the right eye. B, One month after the angiogram, the patient has minimal swelling and redness of the right eye. Intraocular pressure and ocular pulse amplitudes are normal and symmetric.

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Figure 42.47. Worsening of ocular appearance in the setting of spontaneous closure of a dural carotid-cavernous sinus fistula. The patient was a 73-year-old woman who developed a red right eye. A, Appearance of the patient when she was first examined. Note mild redness of the right eye, associated with swelling of the right eyelid and mild proptosis. An evaluation revealed a right-sided dural carotid-cavernous sinus fistula fed by branches of the right internal and external carotid arteries. It was elected to follow the patient without intervention. Two months later, she experienced an abrupt increase in the swelling and redness of the right eye. B, Appearance of the right eye now shows increased redness, swelling, conjunctival chemosis, and proptosis. Color Doppler imaging showed that the right superior ophthalmic vein was occluded, and a cerebral arteriogram confirmed that the fistula was in the process of spontaneously occluding. C, A month after the onset of worsening and 3 months after the onset of visual manifestations, the right eye shows only minimal redness and swelling. The patient had a normal appearance 3 months later.

42.47). Systemic corticosteroids given when deterioration occurs may lessen the severity of symptoms and signs and perhaps reduce the length of time until recovery occurs (149). Patients in whom a dural CCF persists or in whom such a fistula is not recognized may experience major hemorrhagic and other complications when they undergo intraocular or orbital surgery performed for other reasons, such as cataract or strabismus (125). Others may undergo uncomplicated surgery, only to lose vision subsequently from ischemic complications of the fistula (150). TREATMENT Various investigators have advocated direct surgery (151), conventional radiation therapy (152), stereotactic radiosurgery (153), or intermittent self-compression of the affected internal carotid artery for treatment of dural CCF. Although these treatments may be appropriate for selected patients, endovascular embolization is the optimum treatment for those lesions that produce progressive or unacceptable symptoms and signs including visual loss, diplopia, intolerable bruit, severe proptosis, and, most importantly, cortical venous drainage (3,5,78,88,96,127,129,154,155). A number of synthetic and natural materials can be used for embolization, including absorbable gelatin (Gelfoam), Silastic, platinum coils, low-viscosity silicone rubber, autogenous clot, muscle or dura, tetradecyl sulfate (a sclerosing agent), polyvinyl alcohol particles (lvalon), ethanol, oxidized cel-

lulose (Oxycel), and isobutyl-2-cyanoacrylate glue (Bucrylate) (3,88,127,156160). In patients with a fistula fed only by meningeal branches of the external carotid artery or by meningeal branches from both the external and internal carotid arteries, the embolization material is introduced via a microcatheter placed in the external carotid artery and passed into the specific branch or branches that feed the fistula (3,127,159,161). The internal carotid artery usually is not embolized unless the interventionalist can successfully catheterize the meningohypophyseal trunk or other meningeal feeders from the artery (111). When the fistula is fed only by branches from the external carotid artery, successful closure of these branches is often possible and is associated with rapid resolution of all ocular symptoms and signs (discussed later) (1,127). When a dural fistula is fed by branches from both the external and internal carotid arteries, some authors recommend embolization of the feeders from the external carotid artery using various particulate agents such as polyvinyl alcohol particles or glue in the hopes that this will reduce the blood flow in the fistula sufficiently that nonembolized feeders from the internal carotid artery will thrombose spontaneously (146,160). If this does not happen, the fistula can be treated by introduction of platinum detachable coils or detachable balloons into the cavernous sinus using a transvenous route via the femoral or internal jugular vein into the inferior or superior petrosal sinus, directly or indirectly (e.g., via the facial or angular veins) into the superior or inferior ophthalmic vein or into the ipsilateral or contralateral ptery-

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E
Figure 42.48. Results of transvenous occlusion of dural carotid-cavernous sinus fistulas. A, Preoperative appearance of a 35year-old woman with a long-standing, left-sided fistula. B, Four months after occlusion of the fistula through the left superior ophthalmic vein using a detachable balloon. C, Preoperative appearance of a 34-year-old man with a left-sided fistula. D, Six months after balloon occlusion via the left superior ophthalmic vein. E, Preoperative appearance of a 65-year-old woman with a left-sided fistula. F, Two months after occlusion of the fistula through the left superior ophthalmic vein using detachable platinum coils.

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goid plexus (Fig. 42.48) (1,35,78,83,84,88,92,93,142,155, 157,158,162174,174a). In some cases, more than one session and more than one approach is needed (168,171). The transvenous approach using coils or balloons can also be used as the initial procedure for these dural fistulas as well as for fistulas fed only by branches from the internal carotid artery. In some cases, passing a catheter through the inferior petrosal sinus is difficult or impossible, and the indirect superior ophthalmic vein approach via the external carotid artery seems to us more complicated than necessary. The direct superior ophthalmic vein approach, however, performed by either percutaneous puncture or surgical exposure of the vessel, can be accomplished in the majority of cases and, in fact, may be the best initial treatment for all dural CCFs (93,127,163,175,176). If neither standard embolization techniques nor transvenous balloon occlusion is successful in closure of a dural CCF, sonography can be used to guide direct percutaneous access through the facial vein to embolize the lesion (177) or direct surgery on the cavernous sinus may be considered (3,96,127,178). This technique has a significant complication rate, however, and it should not be performed unless the fistula is producing such devastating visual difficulties that treatment is thought to be required regardless of the consequences. We do not advocate occlusion or embolization of the internal carotid artery for most patients with dural CCFs because of the significant risk of neurologic and visual sequelae (127). Successful closure of dural CCFs by standard particulate or glue embolization is possible in 7095% of cases (96,127,146,179). When transvenous balloon or coil occlusion of the fistula is used, the rate of successful closure is 90100% (93,129). The complication rates of endovascular treatment of dural CCFs are extremely low but include hemorrhage at the catheter site or in the orbit, local infection, sepsis, and both transient and permanent neurologic deficits, particularly ocular motor nerve pareses (127,180,181). Complications are particularly common in patients with connective tissue disorders such as Ehlers-Danlos syndrome (109,182). Devoto et al. reported the development of increasing proptosis, chemosis, and markedly elevated intraocular pressure associated with a mid-dilated and poorly reactive pupil during attempted transvenous closure of a dural CCF (183). The patient was treated with intravenous mannitol and acetazolamide, as well as topical timolol maleate and apraclonidine. At the same time, the interventionalist introduced larger coils into the anterior portion of the cavernous sinus. Within several minutes, the condition had resolved, and the patient had a successful result with vision of 20/20 the next day. Finally, should an attempt at closing a dural fistula transvenously via the superior ophthalmic vein be unsuccessful and the superior ophthalmic vein sacrificed or ligated during the procedure, there is a significant risk of increased venous pressure in the orbit with subsequent neovascular glaucoma and severe visual loss (184). For this reason, physicians attempting this form of treatment should always have another alternative available, such as a transvenous approach through the inferior petrosal sinus, endovascular occlusion of the

artery or arteries supplying the fistula, or direct surgery on the fistula (185). The visual complications of a dural CCF usually do not require local treatment. Occasionally, increased intraocular pressure requires treatment with topical or oral pressurelowering agents, and surgery has been advocated for patients in whom medical therapy does not reduce the intraocular pressure to an acceptable level (2,125). It seems to us, however, that if intraocular pressure remains unacceptably elevated despite maximum medical therapy, and intraocular surgery is contemplated, then definitive treatment of the fistula should be performed instead. If treatment of the fistula cannot be performed or is unsuccessful, or if the intraocular pressure remains elevated, intraocular surgery may be needed (186). Similarly, although the proliferative retinopathy that may occasionally accompany a severe, high-flow dural fistula can be treated successfully with photocoagulation (186,187), it is best to treat the fistula producing the retinopathy whenever possible. Again, if the fistula cannot be treated, or treatment is unsuccessful, photocoagulation may be needed to preserve vision. PROGNOSIS AFTER TREATMENT It is not unusual for dural CCFs to recanalize or form new abnormal vessels after transarterial embolization with particles or other material (188). Recurrence of ocular symptoms and signs heralds the recurrence of the fistula, and patients in whom manifestations recur require repeat angiography and consideration of further treatment. We are less concerned about incomplete closure or recurrence if we have closed the fistula transvenously using one or more detachable balloons or multiple detachable platinum coils. As with direct CCFs, symptoms and signs usually begin to improve within hours to days after successful, permanent closure of a dural CCF (93,127,163). Any preexisting bruit immediately disappears, and intraocular pressure immediately returns to normal. Proptosis, conjunctival chemosis, redness of the eye, and ophthalmoparesis (whether caused by orbital congestion or ocular motor nerve paresis) usually resolve completely within weeks to months, and most patients have a normal or near-normal external appearance within 6 months. Choroidal and retinal pathology also resolve over the same period of time. As is the case with patients who have direct CCFs, patients with visual loss caused by choroidal effusion or detachment usually experience substantial if not complete recovery of visual function (188), whereas patients with visual loss caused by retinal damage (e.g., central retinal vein occlusion, retinal detachment) usually have persistently poor visual function (64). Patients whose dural CCFs are treated with techniques other than endovascular closure, such as stereotactic radiosurgery, often take longer to improve than patients whose fistulas are closed by endovascular techniques (190,191). Nevertheless, these techniques may provide excellent results over time. Conversely, patients who undergo successful balloon or coil embolization of a dural CCF may experience both transient and permanent complications. The most common transient complications are ocular motor nerve pareses

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and facial or orbital pain, but more severe, permanent complications, such as ophthalmic artery occlusion, may occur (192) resulting in permanent blindness in the affected eye, and some patients experience persistent ocular motor nerve pareses, most often sixth nerve pareses, that may require prism therapy, surgical correction, or both.
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