Foveal involvement and lack of visual recovery in APMPPE associated with uncommon features

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is commonly believed to be a benign disease with excellent visual prognosis. Identification of cases with poor visual outcome prompted this retrospective study of 33 eyes of 18 patients with this disorder. Loss of visual acuity at presentation was recorded in 25 eyes (76%), 22 of which had lesions at the fovea. Visual acuity quickly returned to normal or near normal levels (even when it was as poor as counting fingers at entry) in all but 7 eyes of 7 patients, in which visual acuity failed to recover to better than 6/24 over a period of several months. All these eyes had poor acuity and foveal involvement when first seen, and at least one of the following atypical features: age older than 60 years, unilaterality, an interval before involvement of the second eye of at least 6 months, recurrence of the disease, leakage from choroidal vein. One additional patient whose foveae were initially not involved lost vision in one eye because of the development of choroidal neovascularisation. Caution should be exercised in giving a prognosis in cases when the fovea is involved and the acuity markedly reduced, particularly if one or more atypical features is present.