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Abstract: A 68-year-old male presents with unilateral engorged episcleral vessels and elevated intraocular pressure of 37 mmHg. After making a diagnosis of elevated episcleral venous pressure, one must rule out cavernous sinus fistula. I. Case History 67 year old white male CC: Unilateral red eye and near blur Hx: hyperlipidemia, hypertension, peripheral vascular disease, coronary artery disease, polycythemia vera Ocular hx: narrow angles with LPI OU about 10 years ago II. Pertinent findings Engorged episcleral vessels OS IOP 20/37 OD/OS on initial visit Gonioscopy: open angles with red tinge to TM OS C/D: OD: 0.20 OS: 0.35 Pachymetry 566/590 OD/OS FDT: repeatable superior nasal and inferior cluster OS; clean field OD CTA did not reveal AV abnormality III. Differential diagnosis Carotid cavernous sinus fistula Idiopathic increased episcleral venous pressure Sturge-Weber IV. Diagnosis and discussion Carotid cavernous sinus fistula can lead to a mixing of arterial and venous blood leading to a decrease in arterial pressure and an increase in episcleral venous pressure on the side of the fistula. This is due to the flow of aqueous beginning at the trabecular meshwork and proceeding through Schlemm’s canal, the aqueous veins, the anterior ciliary veins, the episcleral veins, and then the inferior and superior ophthalmic veins to the cavernous sinus. Blood seen in Schlemm’s canal while performing gonioscopy is an important clinical sign of increased episcleral venous pressure. The IOP increases reflexly in accordance to the Goldmann equation: IOP = (F/C) + EVP where F = aqueous formation rate, C = aqueous outflow rate, and EVP = episcleral venous pressure. This condition can lead to unilateral glaucoma and often responds poorly to topical medication. V. Treatment and Management On initial visit, when IOP was 20/37 OD/OS, Iopidine 0.5% was instilled OS and reduced the IOP to 35 (after dilation). Began timolol 0.5% 1 gtt BID OS. On week follow up IOP was 20/37, added Simbrinza and referred for possible SLT or trabeculectomy Increased episcleral venous pressure typically responds poorly to topical medication because these drops only reduce the gap between IOP and episcleral venous pressure. The only medication family that works independently of episcleral venous pressure is prostaglandins. SLT is only mildly effective due to the outflow facility being normal. Surgical intervention with trabeculectomy is often necessary to stabilize patients, however, there is an increased risk of choroidal effusion with incisional surgery due to sudden decompression if preoperative IOP is very high. VI. Conclusion Management of glaucoma caused by elevated episcleral venous pressure is challenging. Clinicians should consider elevated episcleral venous pressure as a cause of unilateral glaucoma and be aware of the potential treatment challenges in order to prevent vision-threatening complications.