Grand Rounds Peripheral Exudative Hemorrhagic Chorioretinopathy Mark Sherman MD University of Louisville Department of Ophthalmology and Visual Sciences 08/01/2014

Subjective CC: Decreased vision in the right eye for four months HPI: 70 year old white female presented to the retina clinic with a four month history of painless decreased vision in her right eye. Patient denied any other ocular symptoms. No history of trauma. No recent illness. POH: None PMH: IDDM, HTN

Exam OD OS BCVA: CF @ Face 20/30 Pupils: 53 53 No APD IOP: 14 14 EOM: Full OU

Anterior Segment Exam SLE: OD OS L/L WNL WNL Conjunctiva WNL WNL K WNL WNL AC WNL WNL I/L 2+ NS 2+ NS (No NVI OU)

DFE OD: Dense vitreous hemorrhage, unable to view disc or fundus OS: Disc WNL, attenuated vessels, few scattered microaneurysms (No NVD/NVE)

B-Scan OD Dense vitreous hemorrhage; retina flat

Assessment/Differential Diagnosis Assessment: 70 year old diabetic white female with a dense vitreous hemorrhage OD x ~4 months DDx: 1) Proliferative Diabetic Retinopathy 2) Retinal Tear/Break 3) Exudate age-related macular degeneration Plan: Pars Plana Vitrectomy OD

Surgical Video

1 Week Follow-Up S: Vision markedly improved, no complaints O: BCVA: 20/30 OD, Anterior segment: Unchanged DFE OD: retina flat, ~25% air bubble, large sub-retinal clot inferior to inferior arcade surrounded by laser, peripheral laser 360 degrees A: Peripheral Exudative Hemorrhagic Chorioretinopathy

Peripheral Exudative Hemorrhagic Chorioretinopathy Thought to be a variant of age related macular degeneration Characterized by blood in the subretinal or sub-retinal pigment epithelial space in the peripheral retina More common in older (70-80) Caucasians (~90%) Often confused on presentation for vitreous hemorrhage secondary to retinal detachment or break, choroidal melanoma, or retinal artery macroaneurysm

Peripheral Exudative Hemorrhagic Chorioretinopathy Three characteristic lesion types Hemorrhagic: subretinal or sub-pigment epithelium blood present in the absence of exudate (60%) Exudative: subretinal exudates in the absence of clinically detectable blood (10%) Exudative-Hemorrhagic: both blood and exudates present (30%) Treatment is usually not required unless secondary complications occur (persistent vitreous hemorrhage, retinal detachment, extension into the macula)

Retrospective chart review of 173 eyes (146 patients) Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal melanoma in 173 eyes Retrospective chart review of 173 eyes (146 patients) Mean age: 80 145 (99%) Caucasian 98 (67%) Female 171 (99%) were referred for the diagnosis of choroidal melanoma Shields CL, Salazar PF, et al. Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal Melanoma in 173 eyes. Ophthalmology. 2009;116:529-35.

Common clinical features: Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal melanoma in 173 eyes Common clinical features: Subretinal hemorrhage: 78% Serous RPE detachment: 28% Vitreous hemorrhage: 24% Retinal exudation: 21% Associate macular changes: RPE changes: 23% Drusen: 17% Choroidal neovascularization: 8% Shields CL, Salazar PF, et al. Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal Melanoma in 173 eyes. Ophthalmology. 2009;116:529-35.

Features found to differentiate from melanoma: Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal melanoma in 173 eyes 15 months of observation 90% stabilized or regressed 10% progressed 7% of patients developed exudative macular changes Features found to differentiate from melanoma: Retinal exudation Hypofluorescence on FA Lack of sentinel vessels Cleft separating clot from from choroid on ultrasound Shields CL, Salazar PF, et al. Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal Melanoma in 173 eyes. Ophthalmology. 2009;116:529-35.

Intravitreal Bevacizumab Injection for Peripheral Exudative Hemorrhagic Chorioretinopathy Case report of 74 year female referred for suspicion of choroidal melanoma OD Patient presented with 20/50 vision OD, WNL anterior segment, mild vitreous hemorrhage, and a serous retinal detachment with retinal and subretinal hemorrhages in the inferior periphery B-Scan: 8mm x 3mm infero-temporal lesion Socorro, MA, Sabater N, et al. Intravitreal Bevacizumab Injection for Peripheral Exudative Hemorrhagic Chorioretinopathy. Jpn J Ophthalmol. 2011;55(4)425-7

Intravitreal Bevacizumab Injection for Peripheral Exudative Hemorrhagic Chorioretinopathy Patient was given a diagnosis of peripheral exudative hemorrhagic chorioretinopathy and was observed 2 months later the patient returned with decreased vision (20/100) and complaints of metamorphopsia Exam showed increased vitreous hemorrhage and extension of the serous retinal detachment into the macula Patient was given an intravitreal injection of bevacizumab One week follow-up: vision had improved to 20/40, improvement of vitreous heme and complete resolution of serous detachment Socorro, MA, Sabater N, et al. Intravitreal Bevacizumab Injection for Peripheral Exudative Hemorrhagic Chorioretinopathy. Jpn J Ophthalmol. 2011;55(4)425-7

References Annesley, WH JR: Peripheral exudative hemorrhagic retinopathy. Trans Am Ophthalmol Soc 78:321-364, 1980 BCSC: Retina and Vitreous. Peripheral Retinal Neovascularization. Pgs: 121-22 Mantel I, Uffer S, Zografos. Peripheral Exudative Hemorrhagic Chorioretinopathy: A Clinical, Angiographic, and Histologic Study. Am J Ophthalmol. 2009;148:932-38 Pinarci EY, Kilic, et al. Clinical Characteristics of Peripheral Exudative Chorioretinopathy and its Response to Bevacizumab Therapy. Eye. 2013;27:111-12 Shields CL, Salazar PF, et al. Peripheral Exudative Hemorrhagic Chorioretinopathy Simulating Choroidal Melanoma in 173 eyes. Ophthalmology. 2009;116:529-35. Socorro, MA, Sabater N, et al. Intravitreal Bevacizumab Injection for Peripheral Exudative Hemorrhagic Chorioreintopathy. Jpn J Ophthalmol. 2011;55(4)425-7