Goals Retinal Cases: Not rare if it’s in your chair  Review cases of uncommon retinal diseases  Discuss differential diagnoses  Evaluate retinal diagnostic test and how they can KELLEN KASHIWA, OD BENNETT EYE INSTITUTE help with diagnosis

Case 1 Fundus Photo

 19 yo AF – CC: Blurry vision at near, annual visit  VA: 20/20 OD, OS, OU  IOP: 12, 13mmHg  Anterior segment: Unremarkable  Posterior segment: Next Slide

Differential Diagnosis Roth Spots

 Diabetic Retinopathy  White centered round, flame-shaped retinal heme  Hypertensive Retinopathy  First seen in patient with subacute bacterial  Vein occlusion endocarditis  Vasculitis  Lab testing if suspicion for endocarditis includes  CBC with differential  HIV Retinopathy  Erythrocyte sedimentation rate  Leukemia  C-reactive protein  Anemia  Blood cultures  Preeclampsia Lab Results Leukemia Retinopathy

Patient 1 Normal Normal Male  ESR – Normal Female  Leukemia Retinopathy ESRC-Reactive Protein 4 mL per hour 0-20 ml 0-15 ml per hr  Most case diagnosed prior to eye exam per hr  In a 2020 review, 25% of leukemia cases were diagnosed after  CBC CRP eye exam

CBC RBC– 65g/L  Posterior segment manifestations WBC – 109 x 10^9  Secondary to direct invasion of leukemic cells Platelet – 40.2 x 10^9/L  Serum lactate 950 U/L <250 Retinal grayish white nodules surrounded by hemorrhage dehydrogenase U/L  Roth spots – retinal hemorrhage with white-center  Pale gray swelling of the optic nerve head

Case 2 Fundus Photos

 32 yoAF  Sudden vision loss OD  Vision: OD: 20/40; OS: 20/30  IOP: OD: 13; OS: 13mmHg OU  Anterior segment: Unremarkable, NSC OU  Posterior segment: Next Slide

Differentials Purtscher Retinopathy

 Diabetic Retinopathy  Similar findings to DR  Hypertensive Retinopathy  Retinal hemorrhage (65%)  Retinal whitening  BRVO  Cotton wool spots (93%)  Purtscher retinopathy  Optic disc edema  Purtscher flecken (areas of inner retinal whitening  Mostly associated with trauma  Typically bilaterally seen Purtscher-like Retinopathy Case 3

 In absence of trauma other causes:  43 year old Hispanic female  Acute pancreatitis  CC: sudden vision loss OU  Renal failure  Hx: Hypertension  Preeclampsia and childbirth  BCVA: OD: 20/400 OS: CF @1’  Connective tissue disorder  Valsalva maneuver  IOP: 12, 13mmHg  Anterior segment: MGD, s/p LASIK, NSC trace OU  Posterior segment: Next Slide

Fundus Photos OCT

Findings Differential Diagnosis

 Bilateral optic nerve  Papilledema head edema grade 4+  CRVO  Scattered Exudates  Subretinal fluid  Optic neuritis  Diabetic papillopathy  Hypertensive disc edema  NAION  AION  Compressive optic neuropathy  Posterior scleritis Additional Testing

 Blood pressure  Hemoglobin A1C/Serum glucose  Fasting lipid profile

 If secondary hypertension suspected:  Urinary cortisol – Cushing’s  Plasma renin to aldosterone ration – hyperaldosteronism  Angiograpy – renal vascular disease

Results Posterior Reversible Encephalopathy Syndrome

 In office blood pressure  Rapid onset of symptoms  210/104 mmHg  Headaches  Sent to Emergency room  Seizures for immediate  Vision loss/disturbance hypertensive crisis treatment  Often due to acute hypertension  Chronic/Acute kidney disease/injury  Pre-eclampsia

Treatment Case 3

 Hypertensive control -> Stent to circumflex artery  54 year old Brazilian female  Blood pressure next visit 128/82mmHg  CC: blurred reading vision  Optic nerve head neuroprotection  20/20 OD, OS, OU  Alphagan BID OU for optic nerve head protection  IOP: 13, 14  Macular edema  Recent diagnosed with pneumonia and recovered  Subtenon kenalog for macular edema  Anterior Segment: Pterygium, mild bleph  VA responded  2 wks post from 20/400 -> 20/60  Posterior segment:  4 wks post 20/60 -> 20/40  8 wks post 20/40 ->20/20! Cotton Wool Spots

 Not a normal finding  In a series of patients with CWS  Diastolic BP was >90mmHg  Elevated blood sugar in 20%  Secondary to ischemia from retinal arteriole obstruction  Eosinophilic segments of ganglion cell axons that are swollen – defective axoplasmic flow

Etiology COVID

 Ischemic: HTN, Diabetes, RVO  COVID-19 caused by SARS-CoV-2 uses the ACE2  Infectious: HIV retinopathy, Cat-Scratch receptor to gain entry into cells  Idiopathic  ACE2 receptor is widely expressed in multiple organs – including the retina  Trauma  Coronavirus have been shown to cause optic neuritis  Medication: Interferon Retinopathy and retinitis  Inflammatory: Lupus, GCA

Conjunctivits

 Hyperreflectivity at the Ganglion cell layer and Inner  7% of COVID-19 patients had viral RNA in the plexiform layers conjunctival secretions  Cotton-wool spots and microhemorrhages seen  Autoimmune response  Animal model suggest retinitis and optic neuritis can  Possible macrophage activation syndrome be seen Case 4

 18 yoAM complains of sudden decreased vision and photophobia. BCVA IOPs  OD: 20/50 OD: 11mmHg  OS: 20/400 OS: 12mmHg

 Ant Seg: 3+ cells/flare in the anterior chamber and vitreous OU. Keratic percipitates OU and Koeppe nodules OU.

 Post Seg: See next slides.

 General Health is excellent. No recent illness/malaise, systemic changes or medications.

Case One - OCT Case One – Fundus photos

Case one - FA Case One – Thoughts?

 Differentials???  Findings:  Panuveitis  Bilateral serous RDs  Multiple hyperfluorescent dots at RPE Differential Diagnosis Vogt-koyanagi-Harada

 Syphilis  Sypathetic ophthalmia  Autoimmune Disease Treatment  Tuberculosis  Vogt-Koyanagi-Harada  Higher Rate in Asian,  Systemic Corticosteroids Syndrome  Behcet’s disease Latin, Mediterranean  Multiple White Dot descent  InVit / SubTenon  Posterior sclertis Corticosteroids Syndrome  Chronic, bilateral  Acute Posterior granulomatous uveitis  Surgery  Lupus Multifocal Placoid  Disease vs. Syndrome Epitheliopathy  Sarcoidosis  Triad (APMPPE)  Ocular Lyme Disease  Alopecia  Poliosis  Vitilgo

Case 5 OPTOS

 39 year old Filipino male  CC: Cloudy, FB sensation OS, chef – rubbed his eyes with gloves and may have got something in it  VA: OD: 20/20 OS: 20/60  Anterior segment:  OS: Arcus, 3+ KPs, 3+ cells / 2+flare  Posterior segment:  OS: Vitreal cells

Differential Diagnosis Findings

 Sarcoidosis  Panuveitis  Focal chorioretinitis  RPE tracks with white  Toxoplasmosis lesions  Histoplasmosis  Peripheral subretinal FB  Multifocal choroiditis  Acute multifocal placoid  Further questioning pigment epitheliopathy reveals  MEWDs  Often consumes raw meats and poltry!  DUSN  Ophthalmomyiasis Ophthalmomyiasis Interna Outcome

 Parasitic disease  Treatment  Followed closely and affecting the outer retinal  Immediate treated with oral and RPE photocoagulation antihelminthic for  Nematode  Patient is at risk for a months significant inflammatory  Strongyloides stercoralis  Vision returned to 20/20 response once larva dies OS!  Careful monitoring and prompt treatment of inflammation is essential  Albendazole 400mg  Pred forte QID

CASE 6 CASE 6

 72 YOWF CC: Sudden, painless vision loss OD  BCVA IOP  OD: 20/200 14mmHg  OS: 20/30 15mmHg

 Ant Seg: Unremarkable other than 2+ NSC OU  Post Seg: See following slides

 Medical Hx: Thyroid cancer 20+; Treated 1980s  Medication: None

CASE 6 Optos and RNFL OD Optos and RNFL OS Findings

Findings Differentials???

 Optic nerve head  AION edema OD  NAION  Optic nerve pallor OS  Papillitis  Slight Attenuation  Multiple Sclerosis

Differential Diagnosis? Diagnosis of Exclusion

Differentials??? Additional Testing? PSEUDO – FOSTER KENNEDY SYNDROME

 Foster Kennedy  Imaging – CT/MRI  Optic nerve head  Treatment Syndrome  CSF edema in one eye  Similar to GCA  Papilledema  CRP vs Sed Rate  Optic nerve head pallor  High level IV steroids  Pseudotumor  Carotid Dissection in the fellow eye  Followed by oral steroids  AION  No mass / tumor  Carotid dissection  NAION underlying cause  Active NAION with old NAION in fellow eye

Case 7 Case 7

 65 year old male with  Mp1 shows central loss, reduced VAs decreased sensitivity  OD 20/20->20/50  OCT shows RPE changes  OS 20/20->20/60  ERG shows reduced high  Retinal exam and OCT contrast function show no intraocular fluid (photopic)  MP1 shows significant reduced central sensitivity What’s your differentials? Cone Dystrophy

 Cone-Rod dystrophies  Vision loss ranges from  Treatment  Leber’s congenital 20/40 to CF  Increased anti-oxidant amaurosis  Color vision loss supplementation  Beta-carotenoids  Stargardt disease  RPE loss  Increase omega 3 fatty  Bardet-Biedl syndrome  ERG best test for acid consumption diagnosis  Refsum disease  Electrical current  Batten disease stimulation?  Spinocerebellar axtaia type 7

Electrical Current Therapy MiraGel Implant

 Frequency specific microcurrent electrical stimulation  RPE function

Coat’s Disease Juvenile Retinoschisis

 Splitting of OPL  Typically in the macula  VA ranges 20/200 – CF  Responds very well to magnification  No Treatment Von Hippel Lindau Choroidal Melanoma

Pars Planitis Histoplasmosis

Histoplasmosis Mahalo For Coming!

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