Grand Rounds Eddie Apenbrinck M.D. University of Louisville School of Medicine Department of Ophthalmology & Visual Sciences 4/1/2016 1 1

Subjective CC: sudden painless loss of vision OD HPI: 80 year old white male referred to Retina clinic for sudden painless loss of vision in the right eye. Loss of vision occurred over seconds and was not associated with any pain, flashes or floaters.

POH: Pseudophakia OU (2006) PMHx: HTN, NIDDM, Hyperlipidemia ROS: denies any temporal artery tenderness, headaches, jaw claudication, fatigue, weight loss, appetite changes Meds: Metformin, Simvastatin, Lisinopril Allergies: NKDA

Exam VA(cc,n): 20/25 20/20 -0.50 sphere -1.00+0.50x150 OD OS VA(cc,n): 20/25 20/20 -0.50 sphere -1.00+0.50x150 Pupils: 4 2 4 2 no APD IOP: 13 12 CVF: Very Constricted Full EOM: Full OU 4

Exam Anterior Segment L/L: WNL OU C/S: WNL OU Cornea: WNL OU OD OS Anterior Segment L/L: WNL OU C/S: WNL OU Cornea: WNL OU AC: No cell or flare OU I/L: PCIOL OU Vitreous: WNL OU 5

HVF 30-2 OS OD

Fundus Photos OD OS Normal

BAF OD OS

OCT: Fovea OD OS

OCT: OD Fovea

OCT: OD

Fluorescein Angiogram OD

FA OD OS

Assessment and Plan Assessment Plan 80 year old male with CRAO with patent cilioretinal artery Plan Observe Obtain Carotid, Echocardiogram

Work-up Echocardiogram: unremarkable Carotid ultrasound Right ICA: 50-69% stenosis based on peak systolic velocity Left ICA: poor study, recommend CTA CTA: significant atherosclerotic calcifications involving left carotid bulb and proximal internal carotid artery indicating high grade stenosis vs occlusion

1 month follow-up No significant changes in vision per patient BCVA OD: 20/25 OS: 20/20 IOP: OD 11 OS 12 DFE OD: New NVD and VH (inferiorly)

FA OD

Assessment/Plan 80yo with CRAO with patent cilioretinal OD and new NVD and VH OD Plan PRP OD: #1000 burns, 360 degrees in periphery

2 Month follow-up 4 Month follow-up BCVA OD: 20/25 DFE OD: NVD with resolving VH OCT OD: VMT Plan: Observe 4 Month follow-up BCVA OD: 20/25 DFE OD: Optic Disc Collaterals and resolving VH OCT OD: VMT Plan: Observe

OCT OD: 2 month F/U OCT OD: 4 month F/U

Central Retinal Artery Occlusion Characterized by unilateral, sudden, painless vision loss Visual acuity at the time of initial presentation ranges from counting fingers to light perception in 74-90% of eyes A patent cilioretinal artery is seen in approximately one- third of cases.

Epidemiology The estimated incidence of CRAO is 1 in 10,000 cases at tertiary referral centers. The average age at presentation is in the early sixties Men are affected more frequently than women 1-2% of cases may manifest bilaterally

Central Retinal Artery Occlusion CRAO is often caused by arthrosclerosis-related thrombosis occurring at the level of the lamina cribrosa Evaluation of embolic source Carotid Doppler Imaging (presence or absence of plaque) Echocardiography Hypercoagulability work-up in patients <50 yo

CRAO Treatment NaturalCourse Current conventional therapy Less than 10% of cases report improvement in VA Current conventional therapy Dislodging emboli: ocular massage, Yag laser embolectomy Reducing IOP: anterior chamber paracentesis Vasodilation: Carbogen (95% O2 & 5% CO2), nitrates Maintaining retinal oxygenation: hyperbaric oxygen Thrombolytics: streptokinase, urokinase, and tissue plasminogen activator None of these treatments have proven effective and their use is largely based on anecdotal reports and small case series.

Retrospective case series of 168 cases of CRAO NVD developed in three patients after CRAO, an occurrence rate of 1.8%. Rubeosis iridis also developed in two of the three patients. All three eyes received panretinal laser photocoagulation, with eventual resolution of the NVD

Lit Search Retrospective case series of consecutive patients (286 total eyes: 83 CRAOs and 203 BRAOs) Twelve (14.5%) of the 83 eyes with a CRAO developed ocular neovascularization (91.7%) had iris neovascularization (83.3%) had neovascular glaucoma (16.7%) had neovascularization of the optic disc. 2 of the 203 eyes with a BRAO developed iris neovascularization Diabetes mellitus type 2 was a risk factor for ONV development following a CRAO with an adjusted odds ratio of 5.2

References BCSC: Retina and Vitreous. Central Retinal Artery Occlusion. Pgs 138-140 Ryan SJ. Retina. 4th ed. Philadelphia: Elsevier/Mosby; 2013. Rumelt S, Dorenboim Y, Rehany U. Aggressive systematic treatment for central retinal artery occlusion. Am J Ophthalmol 1999;128:733–8. Brown GC, Magargal LE. Central retinal artery obstruction and visual acuity. Ophthalmology 1982;89:14–9. Hayreh SS, Zimmerman MB. Central retinal artery occlusion: visual outcome. Am J Ophthalmol 2005;140:376–91. Biousse V, Calvetti O, Bruce BB, et al. Thrombolysis for central retinal artery occlusion. J Neuroophthalmol 2007;27:215–30. Park SJ, Choi NK, Seo KH, Park KH, Woo SJ. Nationwide Incidence of Clinically Diagnosed Central Retinal Artery Occlusion in Korea, 2008 to 2011. Ophthalmology. 2014 Jun 7. pii: S0161-6420(14)00383-2. doi: 10.1016/j.ophtha.2014.04.029. [Epub ahead of print] Tang WM, Topping TM, Vitreous surgery for central retinal artery occlusion. Arch Ophthalmol 2000 Nov;118(11):1586-7. Hayreh SS, Jonas JB. Optic disk and retinal nerve fiber layer damage after transient central retinal artery occlusion: an experimental study in rhesus monkeys. Am J Ophthalmol 2000;129:786–95.