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78-year-old woman presents with left upper lid droop, binocular diplopia
The patient's ocular history included POAG and previous cataract surgery.
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A 78-year-old woman came to the New England Eye Center 2 months after she first began to notice her left upper lid drooping. A few weeks later, she began to have binocular diplopia that was horizontal in nature and seemed to worsen when looking to the left and at distance. These symptoms were variable but did not seem to have progressed. She denied having any recent headaches or head trauma. The clarity of her vision was unchanged.
The patient’s past medical history was significant for hypothyroidism, Barrett’s esophagus and hiatal hernia. She had a diagnosis of primary open-angle glaucoma for which she had been using timolol, brimonidine and dorzolamide. She had cataract surgery in each eye with IOL implants several years before.
The exam
Uncorrected visual acuity at distance was 20/30+ and 20/20−. The pupils were equal at 3 mm in dim light reactive to 2 mm in bright light without any afferent defect. There was mild limitation of abduction of her left eye. She was esotropic by 14 prism diopters in primary gaze. This improved to 8 D in right gaze and was 18 D in left gaze.
She had a variable hypertropia that measured 4 D in primary gaze. The right hypertropia was as large as 8 D in left upward gaze and as low as 2 D in right downward gaze. She showed mild proptosis of the left eye and 2+ ptosis of the left upper lid. The ptosis seemed to be fatigable and improved some after ice testing. Her facial sensation was intact to light and sharp touch, and her facial strength was full and symmetric. Her IOP was 12 mm Hg and 18 mm Hg.
The patient’s left eye was diffusely mildly injected with some corkscrew episcleral vessels. Her corneas were clear, anterior chambers were deep and quiet, and she had posterior chamber IOLs bilaterally. Dilated fundus exam was unremarkable.
What is your diagnosis?
Upper lid droop
The differential diagnosis of unilateral ophthalmoplegia and ptosis with proptosis includes ischemic cranial nerve palsy, thyroid eye disease, idiopathic orbital inflammation, orbital tumor with restrictive myopathy, myasthenia gravis and a dural-cavernous sinus fistula.
In the case of our patient, her clinical features of abduction deficit, esotropia and ptosis do not fit the pattern of single cranial nerve palsy. Thyroid orbitopathy would more commonly produce eyelid retraction than ptosis. Idiopathic orbital inflammation would be unlikely without any pain. An orbital mass was not seen on orbital computed tomography (CT) scan.
Myasthenia gravis would not explain her injection or proptosis. Highest on the differential was a dural-cavernous sinus fistula which could cause proptosis, injection, ophthalmoplegia by extraocular muscle congestion in addition to nerve palsy and ptosis (Figure 1). No bruit was heard on auscultation of the orbit, and the patient initially denied any pulsatile tinnitus.
Figure 1a. Photo showing mild left upper lid ptosis and injection of left eye.
Images: Sitko K, Hedges TR
Figure 1b. Photo showing mild limitation of abduction of the left eye.
Figure 1c. Photo showing proptosis of the left eye.
Figure 2. Orbital computed tomography scan demonstrating enlarged left superior ophthalmic vein.
Figure 3. 3-D reconstruction from cerebral angiogram demonstrating filling of the left cavernous sinus from a dural branch of the right internal carotid artery.
A CT scan of the orbits clearly demonstrated a dilated left superior ophthalmic vein (Figure 2). Follow-up cerebral angiography proved a connection between a dural branch of the right internal carotid artery and the left cavernous sinus, confirming the diagnosis (Figure 3).
Discussion
While a carotid-cavernous sinus fistula induced by trauma causes an unmistakable clinical picture of severe proptosis, pain and redness with an audible orbital bruit that is apparent to both clinician and patient, the more subtle findings of a low-flow dural shunt can be more challenging diagnostically. A dural shunt can arise spontaneously, and the clinical findings can be caused by a combination of orbital venous congestion and/or thrombosis, cranial nerve compression within the cavernous sinus, and vascular steal causing ischemia.
Findings include proptosis, dilated episcleral vessels, chemosis, increased IOP and limitation of ocular motility all from the effects of venous congestion from increased venous pressure. Ptosis, limitation of ocular motility and anisocoria can occur as a result of cranial nerve compression in the cavernous sinus or due to vascular steal causing an ischemic palsy. Visual loss can also occur as a result of retinopathy or choroidal effusion when there is severe thrombosis of a superior ophthalmic vein. Optic neuropathy can be caused by glaucoma or expansion of the cavernous sinus to involve the optic nerve.
The findings can be highly variable. Most of the effects of these fistulas are not serious, and many dural shunts spontaneously regress. A small minority of cases can have a cerebral thrombosis and intracerebral hemorrhage. In such cases, and in cases of severe headache, intractable diplopia or uncontrollable IOP elevation, treatment is warranted. The treatment that has shown the best outcomes and least complications is transvenous endovascular obliteration of the cavernous sinus and feeding veins with platinum coils. Given our patient’s advanced glaucoma and increased IOP on maximal medical therapy, it was decided to proceed with treatment.
Clinical course
Our patient underwent transvenous endovascular coiling of parts of her left cavernous sinus as well as her left superior and inferior ophthalmic veins. Follow-up angiography showed resolution of the fistula. Her IOP improved to 12 mm Hg immediately after the procedure. Over the following weeks her injection, proptosis, ophthalmoplegia and ptosis all improved.