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A woman in her early 70s with a history of hypertension, Cushing disease status post–pituitary radiotherapy (1973), left sphenoid wing meningioma involving the left optic nerve and cavernous sinus status postsurgical debulking (2009), radiotherapy with concurrent temozolomide followed by intravenous bevacizumab, and a single prior episode of deep venous thrombosis presented with 2 months of eye redness and decreased vision in her right eye. She previously received a diagnosis of viral conjunctivitis at an outside institution and was treated with topical prednisolone acetate without improvement before presenting to our institution. On examination, visual acuity was 20/25 OD and hand motions OS. A relative afferent pupillary defect was present on the left in the setting of left optic atrophy from the patient’s locally invasive meningioma. Confrontational visual fields were otherwise full in the right eye. Intraocular pressures were within normal limits in both eyes. Hertel exophthalmometry was notable for 3 mm of proptosis on the right. Slitlamp examination demonstrated 3 to 4+ meibomian gland dysfunction in both eyes, but with prominent conjunctival injection in the right eye only. Dilated funduscopy revealed scattered intraretinal hemorrhages (IRHs) and cystoid macular edema (CME) in the right eye, which was also seen on optical coherence tomography (Figure 1). Vital signs, complete blood cell count, and the results of thyroid function tests were within reference ranges. Magnetic resonance imaging and magnetic resonance angiography of the brain and orbits with and without contrast showed a stable size of the meningioma and were otherwise normal.
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Venous stasis retinopathy in the right eye associated with a right carotid-cavernous sinus fistula
D. Order cerebral angiography
The differential diagnosis for CME with IRHs includes retinal vein occlusion, microvascular disease (eg, diabetic retinopathy, radiation retinopathy), hyperviscosity syndrome (eg, plasma cell dyscrasias), and orbital diseases resulting in impaired venous outflow. This patient initially presented elsewhere with eye redness and decreased vision in the right eye, but by the time she presented to our institution, there was greater evidence of inadequate venous outflow (ie, unilateral proptosis, corkscrew vessels extending to the limbus, blood in Schlemm canal, IRHs, and CME). These findings can classically result from venous congestion (eg, thyroid eye disease, retrobulbar mass), arteriovenous fistulization (eg, carotid cavernous sinus fistula [CCF], Sturge-Weber syndrome), or an idiopathic disease (eg, Radius-Maumenee syndrome).1 The patient’s neuroimaging was unrevealing, but given the strong clinical suspicion for CCF, a cerebral angiogram was obtained, which revealed an indirect CCF supplied by dural branches of the right cavernous internal carotid artery. Cerebral angiography is considered to be the diagnostic standard and can detect a CCF missed by less-invasive neuroimaging methods (eg, magnetic resonance angiography). In this case, use of an antivascular endothelial growth factor agent would be inappropriate because doing so would delay the diagnosis and timely treatment of the underlying CCF. Systemic therapy with corticosteroids and a laboratory workup for uveitis are also unwarranted because there was no evidence of concurrent intraocular or orbital inflammation.
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JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC
Corresponding Author: Jane S. Kim, MD, Department of Ophthalmology, Duke University, 2351 Erwin Rd, Durham, NC 27705 (firstname.lastname@example.org).
Published Online: March 21, 2019. doi:10.1001/jamaophthalmol.2019.0069
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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