“Young Female with Painless Blurry Vision” Mohammad Ali Sadiq, MD – PGY2 March 01, 2019

Patient Presentation CC “Blurry vision in right eye” HPI 25 year old white female presented with Sudden, painless, blurry vision OD for the past one week.

History Past Ocular Hx: None Past Medical Hx: Sinus tachycardia Fam Hx: Mom had a history of a retinal illness when she was 55 years old. Meds: None

History Allergies: Penicillin Social Hx: No smoking/alcohol ROS: Negative except as in the HPI

External Exam OD OS VA 20/25+2 20/20-1 Pupils Pharmacologically dilated IOP 21 mmHg EOM Full CVF

Anterior Segment Exam SLE OD OS External/Lids WNL Conj/Sclera White and quiet Cornea Clear Ant Chamber Deep and quiet Iris Flat Lens

Posterior Segment Exam OD OS Nerve Few disc hemorrhages, no edema, no NVD Optic disc pink and sharp Macula Scattered dot blot hemorrhages, flame shaped hemorrhages Normal macula, good foveal reflex Vessels Dilated tortuous veins all quadrants Normal Caliber Vitreous Clear Periphery Scattered hemorrhages in all quadrants. No Holes or Tears. Attached 360 Degrees.

Fundus Photographs Optic Nerve c/d 0.2, pink and sharp, Mild elevation inferotemporal Macula Foveal/Parafoveal multiple fine yellow/orange granular focal dots Vessels normal caliber Subretinal hypopigmented dots (100-200 µm

OCT Optic Nerve c/d 0.2, pink and sharp, Mild elevation inferotemporal Macula Foveal/Parafoveal multiple fine yellow/orange granular focal dots Vessels normal caliber Subretinal hypopigmented dots (100-200 µm

Assessment and Plan A 25 year old white female with who presented with sudden onset, painless decreased vision OD. Differential Diagnosis: Impending CRVO Papillophlebitis Hyperviscosity retinopathy (waldenstrom, multiple myeloma or blood dyscrasias) Ocular Ischemic syndrome Plan: Ordered extensive coagulopathy/infectious work up. Re-assess in 2 weeks.

Labs CBC ANA Protein-C Factor V Leiden Homocysteine Antithrombin Activity Anticardiolipin RPR, Lyme Quantiferon Lipid panel Punctate hyperfluorescence

Update No further visual changes VA 20/25 and 20/20 Work up was negative Mild macular edema in OD on OCT Plan: Continue to observe Follow up in 3 weeks

OCT Optic Nerve c/d 0.2, pink and sharp, Mild elevation inferotemporal Macula Foveal/Parafoveal multiple fine yellow/orange granular focal dots Vessels normal caliber Subretinal hypopigmented dots (100-200 µm

Central Retinal Vein Occlusion Second most common vascular cause of visual loss after DR Most commonly associated with advancing age and hypertension. Vision loss is sudden and painless Ranging along a spectrum from non-ischemic (mild) to ischemic (severe). Choroidal changes Hypoautofluorescence corresponding to sub retinal fluid Punctate hyperautofluorescence corresponding to elongated photoreceptor outer segments

Non-Ischemic CRVO Also known as partial, perfused or venous stasis retinopathy. Vision >20/200 Mild or no RAPD Minimal areas of non-perfusion on FA Anterior segment neovascularization is rare Choroidal changes Hypoautofluorescence corresponding to sub retinal fluid Punctate hyperautofluorescence corresponding to elongated photoreceptor outer segments

Ischemic CRVO Also known as complete, non-perfused or hemorrhagic retinopathy Defined as having ≥10 DD of retinal capillary non-perfusion. Usually associated with poor vision. Associated with anterior segment neovascularization Presence of RAPD More extensive venous dilation/tortuosity and hemorrhages Only 10 percent having >20/400 vision

Anterior Segment Neovascularization High rate in ischemic CRVO (up to 60%) Occurs on average 3-5 months after onset of symptoms. Poor VA is the risk factor most predictive of iris NV in these patients. Other risk factors include area of non-perfusion and presence of intraretinal blood (CVOS study). Choroidal changes Hypoautofluorescence corresponding to sub retinal fluid Punctate hyperautofluorescence corresponding to elongated photoreceptor outer segments

Risk Factors Most important risk factor is age (>90% are older than 50). Hypertension Open-angle glaucoma Diabetes mellitus Hyperlipidemia Hypercoagulability Choroidal changes Hypoautofluorescence corresponding to sub retinal fluid Punctate hyperautofluorescence corresponding to elongated photoreceptor outer segments

Hypercoagulability Hyperhomocystinemia Protein S deficiency Protein C deficiency Factor V leiden mutation SLE Sarcoidosis

Management

Management Multimodal imaging Initial evaluation with monthly visits x 6 months In the CVOS study 16% of initially non ischemic CRVOs converted to ischemic by 4 months. By 36 months, the percentage increased to 34%

IMAGING

Collateralization

CRVO and Cilioretinal Artery Occlusion

CRVO and Carotid Cavernous Fistula

Fluorescein Angiography

OCT-Angiography

Management Laser: Grid laser not recommended for CRVO with macular edema. Prophylactic PRP did not result in significant reduction in incidence of iris NV. PRP when iris neovascularization is observed.

Pharmacological Management Anti VEGF agents have been well-studied for CRVO. CRUISE – Ranibizumab COPERNICUS – Aflibercept SCORE – Intravitreal Triamcinolone SCORE 2 – Bevacizumab and Aflibercept Systemic anticoagulation not recommended

Other Surgical Approaches Peripheral laser anastomosis between retinal vein and choroidal circulation Radial relaxing incision of optic nerve scleral ring Retinal vein cannulation with infusion of TPA.

Conclusions CRVO is a common vascular cause of visual loss. Mostly seen in patients above the age of 50. Important to rule out precipitating causes of CRVO in the younger population. Need to differentiate between ischemic and non-ischemic CRVO. Anti VEGF therapy plays a critical role PRP if there is anterior segment neovascularization. RESTORE trial

“If you can’t find a cause for something…. blame it on a VIRUS “If you can’t find a cause for something…. blame it on a VIRUS !” – Charles Barr, MD

Acknowledgements Charles Barr, MD Efrat Fleissig, MD