Abstract
Purpose::
To describe clinical characteristics and visual and anatomic outcomes of a syndrome clinically similar to acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in older patients.
Methods::
We retrospectively reviewed medical records, color fundus photographs, and fluorescein angiograms of consecutive patients over the age of 50 who presented with acute-onset scotomata associated with flat, gray-white lesions at the level of the retinal pigment epithelium (RPE) reminiscent of APMPPE.
Results::
The cohort included four men and two women with a mean age of 70.6 (range, 58 to 82) years. The acute gray-white lesions were accompanied by vitritis in 3 (50%) patients and by retinal vasculitis in 1 (17%) patient. The disease course was characterized by recurrent episodes in 4 (67%) patients, with initial or eventual bilaterality in all 5 binocular patients (one patient was monocular due to childhood trauma). Acute lesions faded with or without anti-inflammatory therapy, leaving patches of geographic atrophy of the RPE and inner choroid. Laboratory work-ups were unrevealing. Five of six patients were treated with corticosteroids with significant short-term improvement in visual acuity. However, all patients developed progressive geographic atrophy of the RPE and 5 (83%) developed choroidal neovascularization. With a mean (± S.D.) follow-up time of 6.5±5.5 years, the final visual acuity was 20/200 or worse in 9 (82%) of 11 involved eyes.
Conclusions::
APMPPE-like syndrome in an older individual carries a potentially poor visual prognosis. In the short-term, aggressive immunosuppression is likely to improve visual functioning, but progression to geographic atrophy and choroidal neovascular membrane formation is the usual outcome.
Keywords: chorioretinitis • aging • visual acuity