Case of the Month I November 2018

Case of the Month
November 27, 2018

The Case

The patient was an 82-year-old man with diabetes, hypertension, and lung cancer diagnosed two weeks previously who presented with a complaint of a sudden, recent onset of a shadow in the nasal visual field in the right eye associated with new floaters and blurriness. The visual acuity was 20/40 OD and 20/30 OS. The anterior chamber examination was remarkable for pseudophakia without posterior capsular opacity. The posterior chamber examination revealed 2+ vitreous cells in the right eye and a clear vitreous in the left eye. The optic nerves looked healthy. Both maculas had RPE disturbances, a few drusen, and rare hemorrhages. A montage of the fundus periphery of the right eye can be viewed, and the periphery of the left eye is unremarkable except for a few scattered hemorrhages. What is the most likely diagnosis? Is any treatment needed?

This patient had a large subretinal hemorrhage due to peripheral exudative hemorrhage chorioretinopathy (PEHCR), also known as an ectopic choroidal neovascular membrane, in the right eye. Our patient also had mild age-related macular degeneration and diabetic retinopathy. PEHCR patients have choroidal neovascular membranes that can result in loss of vision from massive subretinal hemorrhage or vitreous hemorrhage. The average age of onset is about 80 years, most patients are female, and the vast majority of patients with PEHCR are white. The condition is associated with hypertension as well as age-related macular degeneration and polypoidal vasculopathy. Many patients with PEHCR have been using anticoagulant medications, such as aspirin or Plavix.

As with our patient, over 75% of PEHCR lesions are in the temporal quadrant. Breakthrough bleeding into the vitreous is common, and there was some vitreous hemorrhage in our patient. The value of anti-VEGF treatment in this setting is unclear. Our patient had three Avastin treatments in an attempt to reduce the risk of further bleeding. Our patient ultimately did well, with resolution of the hemorrhage, fibrotic involution of the choroidal neovascular membrane, and improvement in vision.

Case Photos

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This patient had a large subretinal hemorrhage due to peripheral exudative hemorrhage chorioretinopathy (PEHCR), also known as an ectopic choroidal neovascular membrane, in the right eye. Our patient also had mild age-related macular degeneration and diabetic retinopathy. PEHCR patients have choroidal neovascular membranes that can result in loss of vision from massive subretinal hemorrhage or vitreous hemorrhage. The average age of onset is about 80 years, most patients are female, and the vast majority of patients with PEHCR are white. The condition is associated with hypertension as well as age-related macular degeneration and polypoidal vasculopathy. Many patients with PEHCR have been using anticoagulant medications, such as aspirin or Plavix.

As with our patient, over 75% of PEHCR lesions are in the temporal quadrant. Breakthrough bleeding into the vitreous is common, and there was some vitreous hemorrhage in our patient. The value of anti-VEGF treatment in this setting is unclear. Our patient had three Avastin treatments in an attempt to reduce the risk of further bleeding. Our patient ultimately did well, with resolution of the hemorrhage, fibrotic involution of the choroidal neovascular membrane, and improvement in vision.

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