Carotid-Cavernous Sinus Fistulas and Venous Thrombosis
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141 Carotid-Cavernous Sinus Fistulas and Venous Thrombosis Joachim F. Seeger1 Radiographic signs of cavernous sinus thrombosis were found in eight consecutive Trygve 0. Gabrielsen 1 patients with an angiographic diagnosis of carotid-cavernous sinus fistula; six were of 1 2 the dural type and the ninth case was of a shunt from a cerebral hemisphere vascular Steven L. Giannotta · Preston R. Lotz ,_ 3 malformation. Diagnostic features consisted of filling defects within the cavernous sinus and its tributaries, an abnormal shape of the cavernous sinus, an atypical pattern of venous drainage, and venous stasis. Progression of thrombosis was demonstrated in five patients who underwent follow-up angiography. Because of a high incidence of spontaneous resolution, patients with dural- cavernous sinus fistulas who show signs of venous thrombosis at angiography should be followed conservatively. Spontaneous closure of dural arteriovenous fistulas involving branches of the internal and/ or external carotid arteries and the cavernous sinus has been reported by several investigators (1-4). The cause of such closure has been speculative, although venous thrombosis recently has been suggested as a possible mechanism (3]. This report demonstrates the high incidence of progres sive thrombosis of the cavernous sinus associated with dural carotid- cavernous shunts, proposes a possible mechanism of the thrombosis, and emphasizes certain characteristic angiographic features which are clues to thrombosis in evolution, with an associated high incidence of spontaneous " cure. " Materials and Methods We reviewed the radiographic and medical records of eight consecutive patients studied at our hospital in 1977 who had an angiographic diagnosis of carotid- cavernous sinus Received September 24, 1979; accepted after fistula. An additional case of a cerebral hemisphere arteriovenous malformation which revision November 19, 1979. drained in part into the cavernous sinus was included. Eight of the nine patients were Presented at the annual meeting of the Ameri reevaluated clinically at least 1 month after their initial angiography, and five patients had can Society of Neuroradiology, New Orleans, Feb follow-up angiography one or more times. One patient also had orbital venography after ruary 1978. initial angiography. The angiography in most cases included bilateral selective internal and ' Department of Radiology, University of Mich external carotid angiographies and also vertebral angiography in some patients. igan Hospital. Ann Arbor, Ml 48109. Address re print requests to J . F. Seeger. 2 Present address: Section of Neurosurgery, Observations Veterans Administration Wadsworth Hospital, Los Angeles, CA 90073. Angiographic Features 3 Present address: Radiology Service, Veterans Administration Medical Center. Gainesville. FL The cases were divided into three groups according to the initial angiographic 32601. appearance of the fistula: (1) dural- cavernous sinus fistula, six cases; (2) direct AJNR1:141-148, March/ April1980 (3) 0195-6108 / 80/ 0102-0141 $00.00 internal carotid-cavernous sinus fistula, two cases; distant cerebral arterio © American Roentgen Ray Society venous malformation draining into the cavernous sinus, one case. Significant 142 SEEGER ET AL. AJNR: 1, March/ April 1980 angiographic features are summarized in table 1. mosis; and venous congestion [5]. Because our patients Although direct carotid-cavernous sinus fistulas generally exhibited most of these findings in various combinations are regarded as more common, most fistulas in this series (table 2), it was difficult to determine clinically if and when were dural, probably reflecting the referral pattern of our a dural-cavernous sinus fistula had progressed to partial or hospital. even complete sinus thrombosis. Except for bruit, which Five of the six dural-cavernous sinus shunts were of the was present in only some of our patients, the clinical findings low flow, low pressure type [1] and opacified only the of the two entities show considerable overlap. At some time ipsilateral cavernous sinus and its venous connections. Four during their evaluation many of our patients demonstrated of these shunts involved the posterior part of the cavernous an acute, usually transient increase in symptoms, particu sinus and one, the anterior part. Feeding vessels were dural larly headache and chemosis, which probably heralded the branches of one or both external and/ or internal carotid onset of venous thrombosis. The patient with posttraumatic arteries. The sixth dural-cavernous fistula was posttrau direct fistula (case 8) developed signs of cavernous sinus matic, and on the initial examination showed rapid flow from thrombosis on the side opposite the fistula, and the patient dural branches of both internal and both external carotid with distant cerebral vascular malformation (case 9) ex arteri es into both cavernous sinuses. hibited bilateral orbital abnormalities which suggested bilat The two direct fistulas both drained into the midportion of eral cavernous sinus thrombosis. the cavernous sinus. One was due to a lateral tear of the The five patients with spontaneous dural-cavernous fis internal carotid artery and was of the high flow, high pres tulas showed variable degrees of clinical improvement on sure type. The other was secondary to a ruptured cavernous follow-up examinations. Most symptoms, including the bruit, carotid aneurysm and showed only an intermediate flow regressed with time. However, elevated intraocular pressure rate. and diplopia usually persisted much longer, even after the Seven of the nine cases exhibited features suggesting at dural fistula was no longer demonstrable at angiography. least partial sinus or venous thrombosis on the initial angie The patient with posttraumatic dural-cavernous fistula (case grams, and the other two showed evidence of thrombosis 6) showed only slight clinical improvement despite interval on subsequent angiographic examinations. Features of angiographic changes consistent with thrombosis and par coexistent venous thrombosis included one or more of the tial obliteration of the fistula. The patient with posttraumatic following: (1) apparent deformity of abnormal shape of the direct carotid-cavernous shunt (case 8) eventually under opacified part of the cavernous sinus, not related to position went surgical entrapment of the fistula, and the patient with of the patient or location of the shunt; (2) intraluminal filling a distant arteriovenous malformation draining into the cav defects within the cavernous sinus and / or tributaries; (3) an ernous sinus (case 9) had the malformation resected. The atypical venous outflow pattern; (4) unusually slow clearing patient with a direct fistula secondary to a ruptured cavern of the contrast medium from the cavernous sinus, including ous aneurysm (case 7) refused clinical and angiographic puddling or layering; and (5) a change in the pattern of follow-up. venous drainage between angiographic examinations, in cluding the development of collateral pathways. Three pa Representative Case Reports tients developed angiographic features of venous thrombo Case 1 sis on the side opposite the fistula. A 65-year-old woman developed right periorbital pain and head ache afler a " sinus infection" 1 year prior to admission, followed Clinical Features by diplopia 3 months later. Two months before admission she noted proptosis and a " swishing noise" on the right. Physical examination The significant clinical features in our patients are sum at admission showed right exophthalmos, chemosis. and sixth nerve marized in table 2. The classic signs of a carotid-cavernous palsy. fistula, including sudden onset of pulsating exophthalmos, CT scanning demonstrated nontumoral right eye proptosis and a prominent bruit behind the affected eye, marked venous prominent enhancement of the right cavernous sinus. Bilateral congestion and chemosis, impaired eye movement, and carotid angiographies revealed a right-sided dural-cavernous fis reduced visual acuity were present only in the patient with tula draining exclusively into the right inferiour petrosal sinus (fig. a traumatic direct internal carotid-cavernous sinus fistula 1 A). Slow flow, puddling, and layering of the contrast agent in the (case 8). The patient with a direct fistula due to a ruptured right cavernous sinus (fig. 1 B) were interpreted as reflecting partial right cavernous sinus thrombosis. A few hours afler angiography, aneurysm (case 7) had only diplopia and bilateral sixth nerve increased swelling and chemosis developed about the right eye. palsies. This had improved by the following morning, and the subjective The five spontaneous dural-cavernous shunts all oc bruit had disappeared. One month later, despite mild residual right curred in patients 65 years old or older; four were women. proptosis, chemosis, and sixth nerve palsy, repeat angiography They usually were insidious in onset and had features similar showed disappearance of the fistula (figs. 1 C and 1 D). Clinical to those observed by Newton and Hoyt [1]. The most com examination 4 months later revealed complete resolution of the mon in order of frequency were ipsilateral headache, prop proptosis and chemosis, and only diplopia on far right lateral gaze tosis, conjunctival injection, elevated intraocular pressure, persisted. sixth nerve palsy, and a subjective or objective bruit. Classic findings of aseptic cavernous sinus thrombosis Case 2 include headache; sixth and occasionally