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Show ORIGINAL CONTRIBUTION Facial and Trigeminal Neuropathies in Cavernous Sinus Fistulas Robert W. Jensen, MD, JD, Hideki Chuman, MD, Jonathan D. Trobe, MD, and John P. Deveilus, MD Abstract: Three patients with carotid- cavernous fistulas had prominent ipsilateral facial nerve neuropathy. One patient also had ipsilateral third division trigeminal neuropathy, manifesting as painful trismus and lower facial numbness. Rarely reported in carotid- cavernous fistula, these neuropathies may occur when there is substantial drainage of the fistula into a dilated inferior petrosal sinus. Closure of the fistula in two cases resulted in full recovery of the neuropathies within weeks to months. The neuropathies may be caused by ischemia from an unfavorable arteriovenous flow gradient, venous compression, or secondary inflammation. ( JNeuro- Ophthalmol 2004; 24: 34- 38) Facial and trigeminal neuropathies have been rarely reported in carotid- cavernous fistulas ( CCFs) ( 1- 8). We describe three patients in whom ipsilateral facial palsy was a prominent feature, one of whom also had partial trigeminal neuropathy with painful trismus. CASE REPORTS Case 1 A 3 8- year- old man awoke with a painful, red OS, diplopia, left tongue numbness, left jaw pain, and left facial droop. Apart from the eye and jaw pain, his chief complaint was difficulty closing his mouth. Examination disclosed a proptotic OS with lid swelling and conjunctival chemosis. Visual acuities were 20/ 20 in OU and pupils were normal. Left ocular ductions were reduced by 50% in all directions. Intraocular pressures were 12 mm Hg OD and 23 mm Hg OS. He had profound left facial weakness involving all muscles, including those of From the Kellogg Eye Center, Departments of Ophthalmology ( RWJ, HC, JDT), Neurology ( RWJ, JDT), Radiology and Neurosurgery ( JPD), University of Michigan Medical Center, Ann Arbor, Michigan. Address correspondence to Jonathan D. Trobe, MD, Kellogg Eye Center, 1000 Wall Street, Ann Arbor, MI 48105- 1912. E- mail: jdtrobe@ umich. edu. the forehead. The mouth opening was limited by pain over the left masseter, which was enlarged and tender to palpation ( trismus). Hypesthesia was present on the left side of the tongue and left lower lip. No cephalic bruit was heard by the patient or examiners. The rest of the clinical examination was normal. Computed tomography ( CT) of the orbits revealed engorgement of the left superior ophthalmic vein, left proptosis, and relative enlargement of the left extraocular muscles. Enhanced Tl magnetic resonance imaging ( MRI) disclosed asymmetric thickening and enhancement of the left cavernous sinus and dura adjacent to the foramen ovale ( Fig. 1 A). A cerebral arteriogram ( Fig. IB) disclosed a left cavernous sinus dural arteriovenous fistula with extensive filling through the meningohypophyseal trunk of the left internal carotid artery. The inferior petrosal sinus appeared occluded at the level of the petrous temporal bone ( Fig. IB). The patient underwent attempted endovascular closure of the fistula, with coils deposited in the left cavernous sinus via the left inferior petrosal sinus. After the last cavernous coils were deposited, slow flow to the fistula was still evident through the distal left internal maxillary artery. Transarterial embolization through that vessel completely closed the fistula ( Fig. 1C). The patient's trigeminal pain disappeared within 2 days, the congestive orbitopathy within 1 week, the facial weakness and trismus within 2 weeks, and the ocular ductal deficits and misalignment within 6 weeks. Case 2 A 75- year- old hypertensive woman developed diplopia and a red OD. Examination showed right proptosis with tortuous, injected, and arterialized conjunctival vessels. There was a partial right abduction deficit and a right facial palsy involving all muscles, including those of the forehead. The rest of the examination was normal. MRI showed enlargement of the right cavernous and inferior petrosal sinuses ( Fig. 2A). No intervention took place. One month later, visual acuity deteriorated to hand motions OD and 20/ 200 OS. Pupils measured 5 mm in dim 34 J Neuro- Ophthalmol, Vol. 24, No. 1, 2004 Neuropathies in Cavernous Sinus Fistulas JNeuro- Ophthalmol, Vol. 24, No. 1, 2004 FIGURE 1. Case 1. A. Coronal enhanced T1 MRI shows asymmetric thickening of the left cavernous sinus ( arrow), with dural enhancement extending to the subjacent foramen ovale. B. Left internal carotid arteriogram ( lateral view, mid- arterial phase) shows early filling of the left superior ophthalmic vein ( SOV) and cavernous sinus, with prominent drainage into the left inferior petrous sinus, which appears occluded at the petrous apex ( arrow). C. Left external carotid arteriogram ( lateral view, early arterial phase) after placement of coils in the cavernous sinus. There is no filling of the fistula. FIGURE 2. Case 2. A. Axial T2 MRI shows a dilated right inferior petrosal sinus ( arrow). B. Right common carotid arteriogram ( lateral view, mid- arterial phase) shows a dural carotid- cavernous fistula draining into a dilated and truncated inferior petrosal sinus ( arrow). The superior petrosal sinus ( curved arrow) and superior ophthalmic vein ( open arrow) are dilated. 35 JNeuro- Ophthalmol, Vol. 24, No. 1, 2004 Jensen et al. illumination and were minimally reactive to light. Abduction, infraduction, and supraduction were 50% reduced in the OD. Abduction and supraduction were reduced 25% in the OS. The patient had proptosis of 26 mm on the right and 28 mm on the left. Applanation tonometry revealed intraocular pressures of 16 mm Hg OD and 24 mm Hg OS. She had complete right facial paralysis with exposure keratopathy and a large central corneal epithelial defect. Right carotid arteriography revealed a right dural CCFs, fed predominantly by branches of the right external carotid artery. The fistula drained through a markedly dilated right inferior petrosal sinus ( Fig. 2B). There was heavy drainage into the left cavernous sinus and ophthalmic veins. Transvenous embolization through the right inferior petrosal sinus failed because of multiple septations in that sinus. Transarterial embolization of the right ascending pharyngeal artery, using a dilute solution of polyvinyl alcohol emboli particles, was successful in greatly reducing flow to the fistula. One year after the procedure, the patient's visual acuity had improved to finger counting OD and 20/ 30 OS. She still had corkscrew conjunctival vessels in the OD and a complete right facial palsy. She declined further intervention. Case 3 A 5 8- year- old man developed sudden pain behind his OS, diplopia, and weakness of the left face. Examination that day disclosed normal visual function. There was 25% reduction in left adduction, supraduction, and infraduction; 2 mm of left upper lid ptosis; and left mydriasis with a sluggish pupillary reaction to light. Intraocular pressures were 15 mm Hg OU. The ocular adnexal tissues were otherwise normal, and the anterior and posterior segments of the globes appeared normal. He had incomplete paralysis of all facial muscles on the left side, with preservation of remaining cranial nerves ( including trigeminal). The rest of the neurologic examination was normal. Catheter cerebral arteriography demonstrated a direct left carotid- cavernous fistula with dilatation of the left cavernous sinus and drainage primarily into bilateral ophthalmic and inferior petrosal sinuses ( Figs. 3A and B). There was a relative stenosis in the ipsilateral inferior petrosal sinus ( Fig. 3A). Transvenous catheterization of the cavernous sinus via the inferior petrosal sinus, with deposition of platinum coils, closed the fistula ( Fig. 3C). Within hours of the procedure, periocular pain was gone. Six days later, his third nerve palsy had completely resolved. His facial palsy had disappeared 10 days later. DISCUSSION Our three patients had CCFs and ipsilateral facial nerve palsies. The patient in Case 1 had an ipsilateral sensory and motor third- division trigeminal neuropathy as well. All patients had prominent drainage through the ipsilateral inferior petrosal sinus and angiographic evidence of petrous sinus congestion. Two of the patients ( Cases 1 and 2) had dural fistulas fed primarily by external carotid artery branches; one had a direct carotid- cavernous fistula fed exclusively by the intracavernous internal carotid artery. Endovascular repair was successful in closing the fistula completely in Cases 1 and 3, and in rapidly eliminating all clinical stigmata. In Case 2, repair was only partially successful and clinical manifestations, including facial palsy, persisted. Facial and trigeminal neuropathies are rare manifestations of CCFs, except in head trauma ( 1- 4). In such cases, skull fractures are common; the neuropathies may be the result of the contusion injury rather than the fistula. There are three descriptions of seventh nerve palsies in nontraumatic fistulas ( 5- 7). The first ( 5) consisted of a red- eyed direct carotid- cavernous fistula ( 5) " with early filling of the jugular vein," but no other angiographic detail was reported. The authors ascribed the facial weakness to pressure from congested fallopian canal veins that drain into the inferior petrosal sinus and ultimately into the jugular vein. The second case ( 6) consisted of a red- eyed dural CCF that demonstrated " early filling of the venous plexus in the pet-roclival region and petrosal sinus." The authors proposed that the facial palsy resulted from compression by dilated or thrombosed veins, or from " steal" from the middle meningeal artery, a major supplier of the fistula and a major arterial supplier of the facial nerve before it enters the fallopian canal. The third case ( 7), reported in a textbook, consisted of a retroclival dural arteriovenous malformation draining forward into the cavernous sinus and backward into the petrosal sinuses. No vascular details or hypotheses were put forward. There is only one previous description of a trigeminal neuropathy in a patient with an intracranial fistula ( 8). In that report, a 38- year- old woman complained of unilateral pulsatile tinnitus and ipsilateral paresthesias of the left lip, tongue, and buccal mucosa, dating from a pregnancy 2 years earlier. Examination showed reduced touch and pinprick sensation over the second and third trigeminal divisions and an intracranial bruit that disappeared with ipsilateral carotid compression. Corneal sensation and other first division sensory signs were normal. Congestive ophthalmic signs and trismus were not described. Angiography showed " a dilated middle meningeal artery with early filling of the cavernous sinus. Early venous drainage was noted into the posterior venous sinus and internal jugular veins." During a 36 © 2004 Lippincott Williams & Wilkins Neuropathies in Cavernous Sinus Fistulas JNeuro- Ophthalmol, Vol. 24, No. 1, 2004 * V r i J * FIGURE 3. Case 3. A. Left internal carotid arteriogram ( frontal view, early arterial phase) shows a left direct carotid- cavernous fistula with drainage into contralateral cavernous and petrosal sinuses. There is relative stenosis of the left inferior petrosal sinus ( arrow). B. Left internal carotid arteriogram ( lateral view, early arterial phase) shows a left direct carotid- cavernous fistula with drainage into the inferior petrosal sinus ( arrow) and superior ophthalmic veins. C. Left common carotid arteriogram ( lateral view) after deposition of coils in the cavernous sinus shows no further arteriovenous shunting. subtemporal craniectomy, " the dilated middle meningeal artery was followed to the enlarged foramen spinosum, which had become contiguous with the foramen ovale." Ligation of that vessel led to immediate postoperative disappearance of the bruit. Two months later, the facial paresthesias had lessened. The authors attributed the trigeminal neuropathy either to steal from the inferior cavernous sinus artery, compression of the second and third division branches by a dilated middle meningeal artery, or " chronic venous congestion within the restrictive cave of Meckel due to high pressure posterior drainage into the petrosal sinus." The common thread in these few cases and ours is prominent drainage through the inferior petrosal sinus ( Fig. 4). The patient in our Case 1 had occlusion of the ipsilateral inferior petrosal sinus. The patient in Case 2 had multiple septations in that sinus, and the patient in Case 3 had a stenosis in that sinus. The Case 1 patient, with the combined seventh and fifth nerve palsies, had prominent enhancement . y&£^%'\ C¥' fr; L't; f. FIGURE 4. Schematic of dural venous plexuses at the cranial base illustrating pathologic flow initiated by a left carotid- cavernous fistula. The drawing shows dilation of the left cavernous sinus and left inferior petrosal sinus ( 1), which compresses the left facial nerve as it enters the acoustic foramen ( 4) ( Cases 1- 3); and dilation of the vein entering the left foramen ovale ( 2), which compresses the left third trigeminal branch ( 5) ( Case 1). The left superior petrosal sinus ( 3) is not dilated. ( Drawing by David Mur-rel, M. F. A.) 37 JNeuro- Ophthalmol, Vol. 24, No. 1, 2004 Jensen et al. of the dura subjacent to the cavernous sinus, which was likely related to venous congestion extending into the ipsi-lateral foramen ovale. These findings tempt us to invoke inferior petrous venous congestion as an etiology for the facial neuropathy ( Cases 1- 3), and foramen ovale congestion for the trigeminal neuropathy ( Case 1). However, we acknowledge having encountered other patients with similar drainage patterns who do not have these neuropathies. REFERENCES 1. Roland JT Jr, Hammerschlag PE, Lewis WS, et al. Management of traumatic facial nerve paralysis with carotid artery cavernous sinus fistula. Eur Arch Otorhinolaryngol 1994; 251: 57- 60. 2. German C, Leeb DC. Arteriovenous fistula and paralysis of the mandibular branch of the facial nerve following direct wiring of a jaw fracture: case report. Plastic Reconstruct Surg 1977; 60: 807- 808. 3. Kalina RE, Kelly WA. Proliferative retinopathy after treatment of carotid- cavernous fistulas. Arch Ophthalmol 1978; 96: 2058- 2060. 4. Walsh FB. Vascular lesions and circulatory disorders of the nervous system. In: Walsh FB, Clinical Neuro- Ophthalmology, Edn 2. Baltimore: Williams & Wilkins, 1957: 850. 5. Kapur A, Sanghavi NG, Parikh NK, et al. Spontaneous carotid-cavernous fistula with ophthalmoplegia and facial palsy. Postgrad Med J 1982; 58: 773- 775. 6. Moster ML, Sergott RC, Grossman RL. Dural carotid- cavernous sinus vascular malformation with facial nerve paralysis. Can J Ophthalmol 1988; 23: 27- 29. 7. Kupersmith MJ. Neurovascular Neuro- ophthalmology. New York: Springer- Verlag, 1993: 130. 8. Rizzo M, Bosch EP, Gross CE. Trigeminal sensory neuropathy due to dural external carotid cavernous sinus fistula. Neurology 1982: 32: 89- 91. 38 © 2004 Lippincott Williams & Wilkins |
