Case History: 72 year-old Black or African American male Chief complaint: Patient presented for routine eye exam with c/o mild distance and near blur. Patient denied any other changes to vision, flashes, new , , eye pain/ache/strain, or . Ocular history: * Physiological cupping vs. suspect * Choroidal Nevus * Peripheral hemes OD in the setting of stage 4 CKD * FOHx: (+)Glaucoma- mother Medications: - NO ocular meds Medical history: * Allopurinol * Clopidegrel Bisulfate * * Colonic polyps * Aspirin * Egocalciferol * Adrenal hyperplasia * Diverticulosis * Calcitriol * Ferrous sulfate * Possible CAD * CKD Stage 4 * Carvedilol * Nifedipine * Gout * Former smoker * Clonidine * Sodium bicarbonate

Pertinent Findings: Clinical: ▪ BCVA 20/20-2 OD and 20/20 OS, Ancillary Tests (, EOMs, CFF) all WNL ▪ Slit lamp exam: unremarkable OU, IOP: 13/14 ▪ BP: 220/106. HTN Crisis. His BP was measured for a clinical trial that he enrolled in after he was dilated with 1% tropicamide and 2.5% phenylephrine. ▪ ONH: OU: 0.55 cupping with healthy rim and distinct margin, Macula: OU: (-)elevation, (-)heme, (-)SRF ▪ Vessels: Mild arteriolar attenuation and reflex change with minimal AV nicking. ▪ Post. Pole: OD: 2 moderate SRDs, 2 small SRDs, 1 small focal PED; no heme/exudate/CWS OS: 4 small focal PEDs; no heme/exudate/CWS ▪ Periphery: unremarkable OU

Labs: Blood Urea Nitrogen: 64 mg/dl (high), Creatinine: 4.28 mg/dl (high), EGFR: 17 ml/min (low) Ophthalmic Imaging: SD-OCT, infrared imaging (IR), autofluorescence (FAF), and fundus photography were used to document the initial clinical presentation and subsequent resolution of SRDs with continued medical management/control of BP over the course of 5 visits. Salient and sequential images will be presented. ▪ Visit 1 (1/17/17): 4 SRDs + 1 PED OD, 4 PEDs OS, thickened subfoveally OU (in comparison to 2012 baseline – which already showed thicker than average looking choroid) were imaged. BP was dangerously elevated at 220/106 at this initial visit. ▪ Visit 2-5 (last seen on 8/15/17): Imaging shows gradual and complete resolution of all SRDs, and reduction in subfoveal choroidal thickness in conjunction with improving BP level (122/69 on 8/15/17). PEDs remained stable throughout the course.

Differential diagnoses: 1. Primary: Hypertensive choroidopathy OD > OS with SRD primarily caused by HTN crisis and complicated by pre-existing CSCR 2. Chronic CSCR OD > OS primarily causing SRD, complicated by sudden increase in BP resulting in a hypertensive choroidopathy component 3. HTN choroidopathy OD>OS, with SRD from HTN process only. 4. CSCR OD > OS with SRD from CSCR process only

Diagnosis and discussion: ▪ Hypertensive crisis is defined as a systolic BP >180 mmHg and or diastolic BP >110 mmHg. Our patient’s presenting BP was 220/106. Hypertensive choroidopathy is a pathological response of the choroid from excessively elevated BP causing damage to the choriocapillaris and RPE cell resulting in SRD, Elchnig spots (focal damage), and Siegrist’s streaks.. Although HTN choroidopathy is an uncommon complication of HTN, if occurs, it often associates with hypertensive crisis, and more so if there is renal disease (my patient presented with stage 4 CKD), pheochromocytoma, or toxemia of pregnancy. Sudden, excessive rises in will impact choroidal circulation much more than retinal vasculature due to configuration of its vascular network. Choroidal arteries supply the choriocapillaris at a 90 degree angle, making the choriocapillaris more susceptible to acute rises in BP. This can lead to fibrinoid necrosis of the choriocapillaris, resulting in choroidal and damage to the RPE with subsequent serous retinal detachments. HTN tends to respond to chronic hypertension via changes in the smooth muscle in the retinal arteriole wall with early manifestations of arteriolar attenuation, reflex change, and AV nicking. ▪ Untreated or improperly managed hypertension is also a risk factor for activating central serous chorioretinopathy (CSCR); a condition defined as a serous detachment of the neurosensory that occurs over an area of choriocapillaris fluid leakage through the RPE. This choroidal vascular hyperpermeability can lead to PEDs, disruption of RPE barrier, and sensory retinal detachments. This similar mechanism of fluid extension into the subretinal space is also seen in eyes with hypertensive choroidopathy, suggesting a similar pathogenesis between choroidal permeability changes and SRF accumulation. However, eyes with true active CSCR do not have signs of accelerated hypertension. An increase in choroidal thickness is also attributable to choroidal hyperpermeability, seen in both hypertensive choroidopathy and CSCR. Studies have shown that choroidal thickness and SRDs can increase significantly with BP spikes and decreases as BP normalizes in HTN choroidopathy. In CSCR, the increased choroidal thickness often remains unchanged despite resolution of the SRD, which suggest a different pathophysiologic process ▪ Our patient’s clinical findings of SRDs, PEDs, and thickened subfoveal choroid are consistent with both HTN choroidopathy and CSCR It is our opinion that his clinical manifestations are most consistent with HTN choroidopathy caused by his hypertensive crisis, and secondarily complicated by the patient’s preexisting CSCR. This is further supported by the subsequent resolution of the SRDs and general and overall reduction in subfoveal choroidal thickness corresponding to his ongoing improvement in BP control. At his most recent visit, his BP was 122/69, with complete resolution of his SRDs and significant reduction in choroidal thickness.

Treatment and Management: ▪ On the day of initial encounter with hypertensive crisis, patient was subsequently admitted for in-patient management of hypertensive urgency. Patient has been following up with primary care and renal clinic every 2-3 mo. with consistent monitoring of BP. ▪ Patient has been followed closely by with DFE, SD-OCT (with EDI imaging), infrared imaging (IR), fundus autofluorescence (FAF), and fundus photography until complete resolution of HTN choroidopathy with 5 visits in the past 7 months.

Conclusion(s): ▪ Hypertensive choroidopathy may cause permanent damage to vision if left untreated; therefore it is crucial to accurately diagnose and properly manage in order to reduce the risks of systemic and ocular comorbidities. This case highlights the importance of understanding choroidal pathophysiology, as well as recognizing key clinical features on multimodal imaging, in order to provide optimal care to our patients. It is essential for optometrists to be competent in diagnosing and managing all potential ocular complications of HTN. ▪ Similar to hypertensive choroidopathy, CSCR also results from choroidal dysfunction. Therefore, hypertensive choroidopathy may mimic findings of CSCR and it is essential to differentiate between the two to avoid potentially devastating consequences on a patient’s health. This is especially important in a VA setting, as coexistence of HTN and CSCR is not uncommon. In those cases, it will be prudent to measure patient’s BP in- office and evaluate the choroid with multimodal imaging in order to enhance clinical care.

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