Cavernous sinus syndrome produced by communication between the external carotid artery and cavernous sinus MICHAEL S. EDWARDS, M . D . , AND EDWARD S. CONNOLLY, M . D .
Department of Neurosurgery, Ochsner Medical Institutions, New Orleans, Louisiana The authors present two cases of cavernous sinus syndrome with spontaneous onset secondary to arteriovenous malformations and review the cases reported previously. These malformations enlarge slowly and produce symptoms only in adult life. Diagnosis may be difficult when there is no associated bruit. Adequate evaluation necessitates selective angiography of both the internal and external carotid artery circulation and the vertebral circulation. Conservative treatment is recommended unless symptoms worsen or there is progressive loss of vision. KEY WORDS 9 angiography, selective cavernous sinus
AVERNOUS sinus syndrome has commonly been associated with fistulous communications between the cavernous sinus and the internal carotid artery. Only 24 cases have been reported in which the major supply was through the external carotid artery 1-6'8'1~176 or the vertebral artery. 4 We wish to report two cases in which the major blood supply was through the external carotid artery with a main contribution from the vertebral artery in one case. Case Reports
Case 1 A 42-year-old left-handed man developed a pulse-synchronous noise in his left ear in 1971, which he described as like the sound of whining dogs. H e was examined by an otolaryngologist who found that compression of the left c o m m o n carotid artery eliminated this noise. H e underwent a carotid angiogram that was reported as normal. 92
9 artery, external carotid
In October, 1973, he noticed proptosis of his left eye, more pronounced on awakening, and a pulsatile vein below his left eye. He denied diplopia and headache at any time since the onset of symptoms. Visual acuity was 20/20 in both eyes. He exhibited 2 m m of non-pulsatile exophthalmos and an intraocular pressure of 3.5 on the right and 7.5 on the left. Extraocular muscle strength was intact and the fundi appeared normal. A bruit, audible over both eyes, the left side of the face and head, and below the left ear, could also be heard over the left carotid artery but not over the right. It was abolished by carotid compression. A left selective angiogram with external carotid injection revealed filling of the cavernous sinus t h r o u g h multiple dural branches of the left internal maxillary artery (anterior meningeal branches and the middle meningeal artery). Multiple tortuous vessels beneath the petrous bone in the cervical region were opacified with retrograde filling J. Neurosurg. / Volume 46 / January, 1977
External carotid-cavernous sinus fistula
FIG. 1. Case 1. Left: Left common carotid injection shows early filling jugular system with retrograde flow into the cavernous sinus (upper arrow) and opacification of the arteriovenous malformation on the petrous bone (lower arrow). Right." Selective left vertebral injection shows filling of the left cavernous sinus through its anterior meningeal branch, and also opacities tortuous vessels on the clivus and petrous bone.
of the cavernous sinus (Fig. 1 left). Drainage from the left cavernous sinus was in three directions: posteriorly into the petrosaljugular system, anteriorly into the superior and inferior ophthalmic veins, and medially to a prominent vein on the margin of the tentorium, draining into the great vein of Galen and straight sinus. Injection into the right common carotid artery revealed filling of the left cavernous sinus from meningeal branches of the right internal maxillary artery. A left vertebral injection revealed filling of the left cavernous sinus through its anterior meningeal branch, and also opacified tortuous vessels on the clivus and petrous bone (Fig. 1 right). No treatment was instituted, and his clinical signs and symptoms were unchanged when he was last examined in 1976.
Case 2 A 58-year-old right-handed man noticed a pulse-synchronous "buzzing noise" in his head in 1971. In March, 1973, he complained of redness of both eyes and was treated with Blephamide ophthalmic ointment by a local ophthalmologist. Following treatment, there was slight improvement on the right; however, the left eye became more severely involved. He was evaluated again in July,
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1973, at which time he exhibited marked conjunctival injection on the left with moderately dilated conjunctival veins. Examination revealed a visual acuity of 20/25 on the right and 20/20 on the left. Exophthalmometer readings were 10 on the right and 22 on the left with applanation tensions of 12 on the right and 32 on the left. He exhibited mild left lateral rectus weakness. The fundi revealed sharp discs bilaterally and no evidence of venous congestion. On auscultation of the orbits, a bruit was heard bilaterally, louder on the left and loudest over the left mastoid and temporal area. A selective left external carotid angiogram revealed a vascular malformation of both the petrous bone and the extracranial structures at the anterior-inferior margin of the petrous pyramid, that drained into the jugular system directly. There was filling of the cavernous sinus through the middle meningeal and anterior dural branches of the internal maxillary artery. Drainage occurred via the petrosal jugular system, anteriorly into the superior and inferior ophthalmic veins, and medially to a prominent vein in the margin of the tentorium that drained into the great vein of Galen/straight sinus (Fig. 2 left). There also appeared to be a contribution to the cavernous sinus via meningeal branches from 93
M. S. Edwards and E. S. Connolly
FIG. 2. Case 2. Left: Left selective external carotid injection shows filling of the cavernous sinus via multiple dural branches of the internal maxillary artery (anterior branches and middle meningeal artery) (upper arrow). Drainage into the petrosal jugular system can be seen (lower arrow) anteriorly into the superior and inferior ophthalmic veins, and medially to a prominent vein on the margin of the tentorium that drains via the great vein of Galen and straight sinus. Right: Left common carotid injection shows opacification of left cavernous sinus via meningeal branches of the internal maxillary artery.
the left internal maxillary artery (Fig. 2 right). A p p r o x i m a t e l y 1 year later the patient's bruit spontaneously ceased with clearing of his conjunctival injection. Discussion In a cooperative study o f 545 cases of arteriovenous malformation (AVM's) Perret and Nishioka TM found 39 carotid-cavernous fistulas; four were extracranial angiomatous malformations and seven combined intraextracranial malformations. Newton and Cronqvist TM reviewed 129 cases of AVM's and found 15 cases limited to the dura and supplied by meningeal arteries alone. O f their patients studied by external carotid angiography, 52% had dural contributions from one or both of the external carotid arteries. In addition to our two cases, we found 36 reported cases of direct filling of the cavernous sinus from the external carotid arteries (Table 1). In 17 cases, the blood supply was solely through the external carotid artery;1.~,a,1o,la,1719 in nine cases, via the external and internal carotid artery; 2,3,s,1~ in seven
cases by the external carotid artery and meningeal branches o f the internal carotid; T M in two cases by the meningeal branches of the internal carotid artery; TM and in one case by the meningeal branches of the vertebral artery.' L i d postulated, and we agree, that cavernous sinus syndrome of spontaneous onset is the result o f congenital dural AVM's that enlarge slowly and present symptoms only in adult life? 8 Almost all cases with blood supply chiefly from the external carotid artery were of spontaneous onset. These appear to be low pressure, low flow shunts that differ from the direct type of internal carotid-cavernous sinus fistula. Eight of 22 patients with cavernous sinus syndrome of spontaneous onset had no bruit, which m a k e s diagnosis difficult. These patients often seek treatment for ophthalmic disorders, but in m a n y cases the dilatation of conjunctival veins is absent or mild; the proptosis is mild, and in only two cases was pulsatile proptosis present. In assessing patients with a cavernous sinus syndrome, selective arteriography of the in-
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External carotid-cavernous sinus fistula TABLE 1 Cavernous sinus syndrome produced by direct filling from external carotid arteries: summary of 38 cases
Authors Markham, 1961 Hayes, 1963 Pakarinen,* 1965 (11 patients) Castaigne, et aL, 1966 Clemens & Lodin, 1968 Newton, 1968 Rosenbaum & Schechter, 1969 Newton & Hoyt, 1970 Taniguchi, et al., 1971 Katsiotis, et al., 1974 (nine patients) Koshik, et al., 1974
Waga, et al., 1974 Edwards & Connolly, 1977
Age, Sex 31 F M M M 30-78 M (8) F (3) 22 F 30 F 67 F 62 F 62 F 49 F 20 45 23 53 64 28 42 58
M F M F M F M M
Bruit + + + + + (6) 0 (5) + + 0 + + +
Exophthalmos Non-puls Puls + + + + proptosis (10) + + + + + proptosis
0 0 + + +
+ + + +
Onset ? traumatic traumatic traumatic traumatic (1) spontaneous (10) spontaneous spontaneous spontaneous spontaneous spontaneous spontaneous spontaneous traumatic traumatic traumatic spontaneous spontaneous spontaneous spontaneous spontaneous
*Individual case reports not given.
ternal and external carotid arteries and the vertebral artery should be done. Our patient (Case l) exemplifies the necessity of a vertebral study. In his case the anterior meningeal branch of the left vertebral artery fed the dural venous plexus around the foramen m a g n u m with retrograde drainage to the cavernous sinus. Fistulas fed by the external carotid artery appear to have a benign clinical course. Newton and H o y t 13 presented one patient who had developed h y p o x i c orbital c o m p l i c a t i o n s related directly to increased orbital venous pressure and subsequently reduced arterial perfusion pressure. He also reported five patients who had experienced spontaneous resolution of these fistulas. Taniguchi, et al.,ig suggested that these patients be managed conservatively unless there is exacerbation of symptoms or visual loss. Mahaley and Boone 8 treated their patients with embolization and achieved good results. Ligation of the external carotid artery has relieved symptoms in some cases; however, if there is a blood supply via the contralateral carotid or vertebral artery, the symptoms may recur. J. Neurosurg. / Volume 46 / January, 1977
References 1. Castaigne P, Laplane D, Djindjian R, et al: Communication art6rio-veineuse spontan6e entre la carotide externe et le sinus caverneux. Rev Neurol 114:5-14, 1966 2. Clemens F, Lodin H: Non-traumatic external carotid-cavernous sinus fistula. Clin Radiol 19:201-203, 1968 3. Hayes G J: External carotid-cavernous sinus fistulas. J Neurosurg 20:692-700, 1963 4. Houser OW, Baker HL Jr, Rhoton AL Jr, et al: Intracranial dural arteriovenous malformations. Radiology 105:55-64, 1972 5. Katsiotis P, Kiriakopoulos C, Taptas J: Carotid-cavernous sinus fistulae and dural arteriovenous shunts. Vasc Surg 8:60-69, 1974 6. Kosnik EJ, Hunt WE, Miller CA: Dural arteriovenous malformations. J Neurosurg 40:322-329, 1974 7. Lie TA: Congenital Anomalies of the Carotid Arteries Including the Carotid-Basilar and Carotid-Vertebral Anastomoses; an Angiographic Study and Review of the Literature. Amsterdam: Excerpta Medica, 1968 8. Mahaley MS Jr, Boone SC: External carotidcavernous fistula treated by arterial embolization. Case report. J Neurosurg 40:110-114, 1974 95
M. S. Edwards and E. S. Connolly 9. Markham JW: Arteriovenous fistula of the middle meningeal artery and the greater petrosal sinus. J Neurosurg 18:847-848, 1961 10. Mingrinos S, Moro F: Fistula between the external carotid artery and cavernous sinus. Case report. J Neurosurg 27:157-160, 1967 11. Newton TH: The anterior and posterior meningeal branches of the vertebral artery. Radiology 91:271-279, 1968 12. Newton TH, Cronqvist S: Involvement of dural arteries in intracranial arteriovenous malformations. Radiology 93:1071-1078, 1969 13. Newton TH, Hoyt WF: Dural arteriovenous shunts in the region of the cavernous sinus. Neuroradiology 1:71-81, 1970 14. Newton TH, Hoyt WF: Spontaneous arteriovenous fistula between dural branches of the internal maxillary artery and the posterior cavernous sinus. Radiology 91:1147-1150, 1968 15. Pakarinen S: Arteriovenous fistula between the middle meningeal artery and the sphenoparietal sinus. A case report. J Neurosurg 23:438-439, 1965 16. Perret GE, Nishioka H: Report of the Cooperative Study of Intracranial Aneurysms
and Subarachnoid Hemorrhage. IV. Cerebral angiography. An analysis of the diagnostic value and complications of carotid and vertebral angiography in 5484 patients. J Neurosurg 25:98-114, 1966 Rosenbaum AE, Schechter MM: External carotid-cavernous fistulae. Acta Radiol [Diagn] 9:440--444, 1969 Takekawa SD, Holman CB: Roentgenologic diagnosis of anomalous communications between the external carotid artery and intracranial veins. Am J Roentgenol Radium Ther Nucl Med 95:822-825, 1965 Taniguchi RM, Goree JA, Odom GL: Spontaneous carotid-cavernous shunts presenting diagnostic problems. J Neurosurg 35:384-391, 1971 Waga S, Ohtsubo K, Handa J, et al: Extracranial congenital arterio-venous malformations. Surg Neurol 2:241-245, 1974
Address reprint requests to: Edward S. Connolly, M.D., Ochsner Clinic, 1516 Jefferson Highway, New Orleans, Louisiana 70121.
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