Imaging in a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with foveal involvement and impressive response to treatment with systemic steroids

Poster Details

First Author: G.Siasou UK

Co Author(s):    S. Stoyanov   A. Tin-U   D. Dragnev                       

Abstract Details


To present a case of APMPPE with foveal involvement and impressive response to treatment with systemic steroids. APMPPE is an inflammatory chorioretinopathy characterized by multiple yellow-white placoid subretinal lesions of the posterior pole, usually bilateral, which tend to resolve over weeks or months leaving circumscribed areas of retinal pigment epithelial (RPE) disturbance. Although the condition is self-limited with 80% of affected eyes achieving a visual aquity of 6/12 or better, foveal involvement confers a worse visual prognosis. There is no current consensus on treatment, however steroids have been reported to be beneficial.


Ophthalmology Department, St Helen's Knowsley and Teaching Hospitals NHS Trust, UK.


A 31-year-old man with clear past medical history presented with severe headaches and vomitting followed by bilateral visual loss and central scotomas four days later. Best corrected visual aquity (BCVA) was 6/36 in each eye. There was no inflammatory reaction in the anterior chamber or in the vitreous. Fundoscopy revealed yellow-white placoid lesions at the level of the RPE involving the fovea. Optical coherence tomography (OCT) presented hyperreflectivity from the outer plexiform layer to the RPE with normal retinal thickness. Fluorescein angiography revealed early hypofluorescence corresponding to the placoid lesions followed by late irregular staining. Brain MRI and lumbar puncture were normal. Blood tests were also performed to exclude other infectious uveitis. The diagnosis of APMPPE was set and the patient was immediately started on intravenous methylprednisolone 1g/day followed by oral prednisolone 80 mg/day, which was reduced by 10 mg every week.


Gradual visual recovery began within the first two weeks of commencing treatment and after four weeks BCVA was 6/9 in the right eye and 6/6 in the left eye. OCT showed resolution of hyperreflectivity of outer layers with residual discontinuation of inner segment/outer segment layer and RPE disturbance.


APMPPE with bilateral foveal involvement can present significant visual recovery within the first month, however further studies are needed to evaluate whether treatment with systemic steroids is beneficial in altering the visual outcome or preventing possible recurrence.

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