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Show Journal of C1illiazl Neuro-ophthalmo!ogy 8(2): 131-135, 1988. :c;', 1988 Raven Press, Ltd., New York Carotid-Cavernous Fistulae Presenting as Painful Ophthalmoplegia Without External Ocular Signs Gregory S. Kosmorsky, D.O., Maurice R. Hanson, M.D., and Robert L. Tomsak, M.D., Ph.D. We examined two patients with carotid-cavernous fistulae who presented with painful ophthalmoplegia syndromes. Neither patient had conjunctival arterialization, chemosis, or exophthalmos. This unusual presentation of carotid-cavernous fistula is discussed in light of the anatomy of cavernous sinus venous drainage. Key Words: Carotid-cavernous fistula-Digital subtraction angiography-Diplopia-Painful ophthalmoplegia. From the Section of Neuro-ophthalmology, Departments of Ophthalmology and Neurology (CSK.) and the Department of Neurology, Section of Neuro-ophthalmology (M.R.H.), Cleveland Clinic Foundation; and the Section of Neuro-ophthalmology, University Hospitals of Cleveland and Case Western Reserve University School of Medicine (R. L. T.), Cleveland, Ohio. Address correspondence and reprint requests to Dr. R. L. Tomsak at Suite 3200A, 2074 Abington Rd., Cleveland, OH 44106, USA. 131 Carotid-cavernous fistulae most often follow head trauma (75%), are less often spontaneous (25%), and are rarely congenital (1). They can also occur following rupture of an intracavernous aneurysm, Sanders and Hoyt (2) found the following presenting symptoms: bruit (75%), proptosis (69%), redness and swelling of the conjunctiva (36%), diplopia (24%), ipsilateral blurred vision (16%), and orbital pain (16%). Thus, the diagnosis of carotid-cavernous fistula would not ordinarily be considered in the absence of external ocular signs. Herein, we present two patients with painful ophthalmoplegia who lack the classical external findings and discuss the apparent mechanism for the absence of these signs. CASE REPORTS Case 1 A 40-year-old man was examined because of a 6 week history of pain around the right eye associated with binocular vertical diplopia. He denied facial numbness and had no other neurological complaints. He suffered from adult onset diabetes mellitus with retinopathy, essential hypertension, and angiographically documented severe coronary artery disease. He denied significant head trauma. Best corrected visual acuity was 20/30 with each eye. The other pertinent findings were absence of ocular congestion, proptosis, or ptosis (Fig. 1). Motility examination showed two diopters of right hypertropia in the primary position, eight diopters of right hypertropia on left gaze, one diopter of right hypertropia on right gaze, and six diopters of right hypertropia on right head tilt (consistent with a right fourth nerve palsy). Corneal and facial 132 G. S. KOSMORSKY ET AL. FIG. 1. Case 1: Note absence of proptosis and ocular congestion. sensation were normal and a bruit could not be auscultated over the eyes or head. A contrast enhanced high resolution computed tomography (CT) scan of the orbits was normal, without evidence of superior ophthalmic vein enlargement or convexity of the cavernous sinus to indicate an infiltrative process, tumor, or aneurysm. An intravenous digital subtraction angiogram showed a right carotid-cavernous fistula with flow mainly directed into the inferior petrosal sinus on the right (Fig. 2). The patient refused further evaluation and went elsewhere for further care. Case 2 A 68-year-old woman was examined because of right-sided headache of 3 months duration and a FIG. 2. Case 1: Lateral intravenous digital subtraction angiogram showing posterior drainage of carotidcavernous fistulae Into Inferinr petrosal (IP) sinus recent 2 week history of horizontal diplopia. One week before examination, a subjective bruit was heard in her right ear. She denied having diabetes, hypertension, or head trauma. The pertinent examination showed normal visual acuities, equally reacting pupils, a normal fundus examination, and normal ocular pressures of 10 mm Hg in both eyes. However, wide pulse pressures were noted by applanation tonometry. She had mild ptosis bilaterally (believed to be aponeurogenic on the right and secondary to mild superior division third nerve palsy on the left) but no exophthalmos or excessive conjunctival hyperemia or chemosis. Forced ductions and a tensilon test were not performed. Corneal sensation was intact on both sides. A bruit was audible over the right orbital and right temporal areas. Motility examination showed mild bilateral abduction deficits and the inability to look up fully with the left eye, consistent with mild bilateral sixth nerves paresis and a mild superior rectus paresis of the left eye (Fig. 3). An intra-arterial digital subtraction angiogram showed a large carotid-cavernous fistula with prompt filling of the right cavernous sinus, right superior ophthalmic vein, and right superior and inferior petrosal sinuses. There was also prompt cross-filling of the left cavernous sinus, superior and inferior petrosal sinuses, and superior ophthalmic vein (Figs. 4 and 5). Because of extreme tortuosity of the carotids, further treatment was considered hazardous. DISCUSSION Although carotid-cavernous fistulae are included in the differential diagnosis of painful ophthalmoplegia, their diagnosis is usually straightforward. Prominent external signs dominate the clinical picture with some combination of CAROTID-CAVERNOUS FISTULAE 133 proptosis, conjunctival arterialization and chemosis, and ophthalmoplegia. Elevated intraocular pressure and retinal ischemic changes also occur (1,3-11). These signs are secondary to elevation in venous pressure, which causes impaired venous drainage from the orbit. The venous drainage of the cavernous sinus can be either anterior or posterior. Anteriorly and superiorly, drainage is via the superior ophthalmic veins to the angular veins, then into the facial veins. Anteriorly and inferiorly, drainage is from the inferior ophthalmic veins into the pterygoid plexuses and then to the facial veins (12). Posteriorly, the cavernous sinuses drain into the transverse sinuses via the superior petrosal sinuses, into the internal jugular vein via the inferior petrosal sinuses, and into the pterygoid plexuses via several small emissary veins (13). The posterior drainage of the carotid-cavernous fistula in our patients explains the lack of external ocular signs. This is a distinctly unusual occurrence. For example, Walsh and Hoyt (3) mention that posterior drainage of carotid-cavernous fistula can make orbital manifestations inconspicuous, although they do not mention a specific patient. Taniguchi and others (5) reported two patients with no external eye signs of carotidcavernous fistula although they did not show evidence of posterior drainage. Most series, however, document a very high incidence of ocular and periocular findings (4,6,9). Most recently, McKinna reported that all of his 63 patients with carotid-cavernous fistula had proptosis and vascular congestion of the globe and orbit. The exception to this is the dural shunt syndrome, in which a low flow shunt develops between dural arteries and the cavernous sinus and in which external signs may be less conspicuous (10). Both of our patients presented with painful ophthalmoplegia syndromes, the first with headaches and a fourth nerve palsy. A differential diagnosis of diabetic ophthalmoplegia, Tolosa-Hunt syndrome, or intracavernous aneurysm was initially considered. The second patient had bilateral ophthalmoplegia with pain. The late onset of a subjective bruit suggested the presence of an arteriovenous communication. Even so, a dural (low-flow) shunt was suspected because of the lack of periocular findings. In this case, the enlargement of the superior ophthalmic veins without episcleral arterialization may have been due to a rapid runoff of blood into the facial venous system combined with rapid posterior drainage with a "bypass" on the conjunctival vessels. Alternatively, there may have been just enough "forward pressure" (i.e., venous backup) exerted on the superior ophthalmic veins from the fistula being exactly counterbalanced by rapid posterior drainage, the net effect of which prevented conjunctival arterialization. The inability to measure venous pressures makes it impossible to differentiate these alternative possibilities. To minimize the chance of missing the diagnosis of carotid-cavernous fistula in cases like ours, we FIG. 3. Case 2: Note absence of proptosis and ocular congestion as well as mild inability to elevate left eye fully. I Clill Neuro-oplzlhallllOl. Vol. 8, No.2, 1988 134 G. S. KOSMORSKY ET AL. FIG. 4. Case 2: Anteroposterior right carotid intra-arterial digital subtraction angiogram showing cross-filling and bilateral dilation of cavernous sinuses (CS), superior ophthalmic veins (SV), and superior (SP) and inferior petrosal (IP) sinuses. suggest that auscultation and palpation of the orbits and cranium should be done routinely in cases of painful ophthalmoplegia. In addition, careful attention should be given to measurement of intraocular pressure, specifically looking for a wide pulse pressure when using the applanation tonometer. In selected cases, intra-arterial digital subtraction angiography is recommended with special attention directed to the pattern of venous drainage as well as to the arterial phases. Acknowledgment: The authors thank Benjamin Kaufman, M.D., for his helpful comments. FIG. 5. Case 2: Right lateral intra-arterial digital subtraction angiogram showing dilation of cavernous sinus (CS) and posterior drainage into inferior petrosal (IP) sinus. 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