Grand Rounds Conference Juan P. Fernandez de Castro, MD University of Louisville Department of Ophthalmology and Visual Sciences Sept 18, 2015

Subjective CC: Blurred vision HPI: 32 year old female presents to general clinic complaining of poor vision OS since she had a hypertensive emergency 7 months ago.

Past Medical History HTN since early 20s Hypertensive stroke, 7 months ago Stage IV Kidney disease Meds: ASA, Amlodipine, Metoprolol, Hydralazine, Clonidine, Vitamin D

Past Medical History Family Hx: HTN mother, father Social Hx: former smoker (Quit 3 years ago) No Known Drug Allergies TIA/stroke - MRI scan shows 3 very small areas of lacunar infarction, 2 in the left frontal head region and one in the right frontal head region. It is unclear if these had anything to do with her symptoms or are purely incidental. MRA suggests that the left middle cerebral artery is completely occluded. The fact that she does not have a significant stroke means that there are probably adequate collaterals to provide blood flow to the area. It is impossible to tell this point whether the middle cerebral artery blockage is old or new.).

Hypertensive emergency, 7 months ago BP 260/200 TIA Bilateral renal atrophy with severe HTN nephrosclerosis, no glomerulonephritis Left Ventricular Hypertrophy Serologies neg: ANA, ANCA, HBV/HCV

Objective OD OS VA (sc): 20/20 CF@2ft BCVA (-0.50+0.75x135) 20/125 Pupils: 54 + rAPD OS 43 Color Brightness OD>OS 10% IOP: 19mmHg 18mmHg EOM: Full Full

Objective SLE: External/Lids WNL OU Conjunctiva/Sclera WNL OU Cornea Clear OU A/C Deep and Quiet OU Iris WNL OU Lens Clear OU Vitreous Clear OU

Fundus Photos Elsching spots OU Attenuated vessels

OCT OD

OCT OS Inner and outer retina

HVF 24-2

Assessment 32 year old female with a history of hypertensive emergency, presenting with a left inferior visual field defect OS, resolving exudative retinopathy and retinal atrophy compatible with Choroidal and Retinal Ischemia OU, with vision loss OS, secondary to hypertensive crisis.

Hypertensive retinopathy  Acute effects are a result of vasospasm to autoregulate perfusion in the retina. Autoregulation is absent in the choroidal vessels Chronic effects are caused by arteriosclerosis Predisposes to visual loss Vascular occlusions Macroaneurysms The sympathetic nerves of the choroid are probably involved in a protective mechanism, preventing overperfusion in acute increments in BP. The facial nerve contains parasympathetic vasodilator fibers to the choroid. Unknown physiological significance

Pathophysiology Retinal hemorrhages: Damaged vessels bleed into either the nerve fiber layer (flame shaped) or the inner retina (dot blot). Cotton wool spots: Ischemia to the ganglion cells in the nerve fiber layer secondary to fibrinous necrosis and luminal narrowing. Exudates: Lipid accumulation. Papilledema: Leakage (heme and disc edema) and ischemia (ON swelling and blurred margins) of arterioles supplying the optic disc

Choroidopathy Poor perfusion of the choriocapillaris Elschnig spots: hyperpigmented patches in the choroid surrounded by a ring of hypopigmentation. Siegrist streaks: linear hyperpigmented lesions over choroidal arteries. Focal pigment epithelium detachment, can lead to exudative retinal detachment

Elschnig Spots www.RetinaGallery.com

Siegrist streaks Retina Image Bank, HJ Kaplan http://imagebank.asrs.org/file/5310/siegrist-streaks

Keith-Wagener-Barker (KWB) HTN Retinopathy Grades (1939) Grade 1: Arteriolar constriction/attenuation/sclerosis silver wiring and vascular tortuosities Grade 2: As G1 + AV nicking Grade 3: As G2 + Retinal edema, cotton wool spots and flame-hemorrhages Grade 4: As G3 + swelling of the optic disc+ macular star

Modified Scheie Classification (1953)

To investigate retinal and choroidal changes using spectral-domain optical coherence tomography (SD-OCT) and to evaluate visual outcome in patients with severe hypertension

Based on fundoscopic and OCT features, eyes were classified as: Mild to moderate retinopathy Malignant retinopathy without SRF Malignant retinopathy with SRF. Unlike KWB grades (P=0.077), the OCT-based retinopathy grades were significantly correlated to final BCVA, as shown by linear regression analyses (P=0.025). Retinal and Choroidal Changes With Severe Hypertension and Their Association With Visual Outcome. SJ Ahn et al.

KWB Classification not corresponding with VA Fundus photographs and optical coherence tomography (OCT) images taken at baseline in (A) a 30-year-old man with BP of 186/120 mm Hg (systolic/diastolic) and (B) a 57-year-old woman with BP of 206/106 mm Hg. Keith-Wagener-Barker (KWB) grades are indicated in the upper right corner of each fundus photograph, and best-corrected visual acuities (VA) are noted in the lower right corner. Right-column OCT images demonstrate retinal changes, including irregular thickening/reflectance of the retinal nerve fiber layer (white arrows) and intraretinal hyperreflective dots (black arrows). The eyes shown in (B) had greater subretinal fluid height (white numbers), thicker subfoveal choroids, and worse visual acuities than those shown in (A), although KWB grades and fundoscopic features were less severe. Retinal and Choroidal Changes With Severe Hypertension and Their Association With Visual Outcome. SJ Ahn et al.

Modified 3-step classification of HTN retinopathy in patients with severe HTN Retinal and Choroidal Changes With Severe Hypertension and Their Association With Visual Outcome. SJ Ahn et al.

Choroidal Changes Enhanced Depth Imaging OCT Choroidal changes identified with enhanced depth imaging optical coherence tomography in two patients with severe hypertension before and after BP control. Left: One month after BP control, subfoveal choroidal thickness decreased from 282 lm at baseline to 222 lm. Right: One month after BP control, choroidal thickness decreased from 241 lm at baseline to 166 lm. In both cases, subretinal fluid height also remarkably decreased following BP control. Retinal and Choroidal Changes With Severe Hypertension and Their Association With Visual Outcome. SJ Ahn et al.

Spectral-domain optical coherence tomography (SD-OCT) images comparing the clinical courses of malignant hypertensive retinopathy between patients with poor (A) and good (B) visual outcomes. Both eyes showed resolution of subretinal fluid, intraretinal fluid, and intraretinal hyperreflective dots. However, the eye with poor final visual acuity (20/50) had incomplete photoreceptor recovery, as indicated by interdigitation zone (IZ) loss and focal inner segment ellipsoid zone (EZ) loss, at the final visit. In contrast, the eye with good final visual acuity (20/15) had complete photoreceptor recovery by the final visit. In the eye with a poor visual outcome (left), the posterior choroidal border was indefinite and choroidal thickness could not be measured. In the eye with a good visual outcome (right), baseline choroidal thickness at week 1, week 2, month 1, month 2, and the final visit was 182, 170, 163, 154, 157, and 158 lm, respectively. ELM, external limiting membrane; VA, visual acuity

Conclusions Severe hypertension resulted in characteristic peripapillary and periarteriolar hypertensive retinopathy features, sub-retinal fluid (SRF) accumulation, and increased choroid thickness. The presence of SRF was associated with choroidal thickening and with poor visual outcome in patients with severe hypertension. OCT may be useful to document hypertensive retinopathy and choroidopathy severity, and predicting visual outcomes

References Retinal and choroidal changes with severe hypertension and their association with visual outcome. Ahn SJ, Woo SJ, Park KH. 2014-2015 Basic and Clinical Science Course (BCSC): Section 12: Retina and Vitreous Paperback. American Academy of Ophthalmology Malignant hypertension: clinical manifestations of 7 cases. Steinegger K, Bergin C, Guex-Crosier Y.