1B
Chronic Painless Ptosis, Complete Ophthalmoplegia with a Red Eye
CASE NO. 1B
A 69-year-old man presented with a 12-week history of a red eye OS which was initially felt to be “conjunctivitis” by an outside eye doctor and was treated with topical antibiotics for two weeks, then with topical antihistamines for two weeks, following which topical antivirals were added, and finally topical steroids without improvement. His symptoms worsened and he developed double vision which was worse on upgaze. He also noticed a “whooshing” sound in his ears, more noticeable just before going to sleep. He denied any periocular pain, headache, or visual blurring. There was no history of trauma as well as no history of thyroid eye disease, and he did not smoke cigarettes. The remainder of his medical history was non-contributory.
On examination, visual acuity was 20/20 OU. There was no relative afferent pupillary defect or anisocoria. There was 2 mm of ptosis in the left upper lid (Fig. 1B.1) and mild to moderate lid edema OS. Slit lamp examination showed diffuse subconjunctival hemorrhage OS with dilated and tortuous episcleral and conjunctival vessels extending to the limbus and looping back (Fig. 1B.2). The intraocular pressure (IOP) was 12 mm Hg OD and 28 mm Hg OS. Optic disc examination showed a cup-to-disc ratio of 0.3 OU and the remainder of the fundus exam was normal. Hertel measurement showed mild proptosis OS of 2 mm. Goldmann perimetry and OCT were normal OU. The left eye had a –2 underaction of elevation with a 15 prism diopter left hypotropia in upgaze.
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Emergencies in Neuro-Ophthalmology: A Case Based Approach
Fig. 1B.1. External photograph showing a left ptosis.
Fig. 1B.2. The left eye had a –2 underaction of elevation with a 15 prism diopter left hypotropia in upgaze. The left eye showed a left diffuse subconjunctival hemorrhage with tortuous vessels suggestive of “arterialization” of the episcleral vessels.
Dr. Lee. In a patient with a chronic “red eye” unresponsive to multiple different topical therapies, the general ophthalmologist should consider alternative etiologies to the typical “red eye” list. In light of this patient’s additional symptoms (e.g. diplopia, ptosis, and
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Chronic Painless Ptosis, Complete Ophthalmoplegia with a Red Eye
proptosis), the clinical localization of the problem shifts from the anterior segment to the orbit. The distinctive clinical sign here is the “arterialization” of the conjunctival and episcleral vessels (Fig. 1B.2), with the classic dilated and tortuous vessels extending to and from the limbus. Interestingly, the intervening conjunctiva is often white, also giving the clinician a clue that the etiology is not a typical allergic, inflammatory, or infectious “conjunctivitis.” The additional symptom of a subjective bruit in this case suggests a vascular etiology and the next most appropriate step is imaging. Elevated IOP and secondary glaucoma can occur due to orbital congestion or increased episcleral venous pressure. Rarely, patients may have angle closure from anterior rotation of a swollen ciliary body. Treating the IOP with glaucoma drops may be useful as a temporizing measure but the underlying etiology needs to be identified and treated. I would consider CCF to be the leading clinical diagnosis in a case like this one. The applanation tonometry mires sometimes give a clue to an increased pulse pressure in the measurement of the IOP. Orbital ultrasound might show a dilated superior ophthalmic vein and arterialization of flow can be obtained using orbital Doppler flow studies. CT scan or MR scan typically shows the dilated superior ophthalmic vein and might show enlargement or even flow voids in the cavernous sinus. Clinical or radiographic evidence for cortical venous drainage should be sought and might be an indication for more aggressive treatment for a carotid cavernous fistula.
Dr. Brazis. The above clinical scenario is most consistent with a CCF. The differentiating features for direct and high flow versus indirect and slow flow fistula have been discussed previously.
Course. Cranial MRI/MRA (Fig. 1B.3) showed a dilated left superior ophthalmic vein with arterialized flow. There was no clinical or radiographic evidence for cortical venous drainage. The patient was observed initially but continued to worsen and underwent a diagnostic catheter angiogram that confirmed the diagnosis of an indirect CCF with internal carotid artery feeders and this was followed by
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Emergencies in Neuro-Ophthalmology: A Case Based Approach
Fig. 1B.3. MRI shows dilated left superior ophthalmic vein.
Fig. 1B.4. Color photograph of the left eye (after dilation) following successful obliteration of the CCF, and with endovascular coiling procedure shows marked resolution of the arterialization of the conjunctival and episcleral vessels.
endovascular closure of the fistula. The subjective bruit immediately resolved after the procedure and over the next two months the ptosis, lid edema, proptosis, ophthalmoplegia, and red eye all completely resolved (Fig. 1B.4).
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