First Author: J.Werner GERMANY
Co Author(s): C. Enders G. Lang G. Lang
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Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a white dot syndrome hence being a primary inflammatory capillaropathy (PICCP). Due to the predominant involvement of the choriocapillaris, APMPPE is also called multifocal ischaemic choroidopathy (AMIC) and can selectively be analyzed with optical coherence tomography angiography (OCT-A). Aim of this study is to provide novel insights into the pathogenesis of APMPPE by new imaging methods including OCT-A.
We included 4 patients (8 eyes) with APMPPE that were seen in our tertiary care hospital between December 2015 and May 2016. All patients gave their written informed consent to study participation, the study was conducted after the approval of the local ethics committee had been obtained.
The routine ophthalmologic work-up was followed by fundus photography, fundus autofluorescence (FAF), optical coherence tomography (OCT), OCT-A and fluorescein angiography (FA). FA was performed in every patient excluding patient 4 due to circulatory collapse. OCT examination was performed with the Zeiss Cirrus 5000 equipped with the AngioPlex module. In addition to structural OCT B-scans, en-face structure analysis of the inner and outer segment (IS/OS) of the ellipsoid zone and OCT-A were performed. In OCT-A superficial retina, deep retina, choriocapillaris and choroid slabs were analyzed. Fundus photography was performed with a Carl Zeiss FF450plus camera and FAF was recorded.
In acute and sub-acute phases of the disease, an early blockage and late staining in FA was found. Structural OCT revealed an intraretinal edema with thickening (4/6 eyes) and a defect in the IS/OS junction (8/8 eyes). Dark areas in the en-face structure analysis of IS/OS junction also indicated defects in this layer. OCT-A imaging showed no alteration of retinal perfusion. Indeed perfusion defects in choriocapillaris and choroid slabs could be observed and the pattern of reduced perfusion was similar to the reduced autofluorescence in FAF. In the course of disease, retinal or sub-RPE fluid resolved in structural OCT. IS/OS junction defects however were still present in structural OCT B-Scans and en-face structure analysis but not as pronounced as on day one after onset of symptoms. Perfusion deficits observed in OCT-A imaging resolved first in the choroid and then in the choriocapillaris slab whereas there were still no perfusion alterations in the retinal slabs. We were able to examine one patient on the first day after onset of symptoms. Already 6 days after onset of symptoms a decline of hypoperfusion areas in choriocapillaris and choroid was observed in OCT-A imaging.
To the best of our knowledge, this is the first time that improvement in choroidal perfusion beginning first in the choroid and subsequently in the choriocapillaris, has been documented by OCT-A in APMPPE. In conclusion, our findings could have an impact on diagnosis and therapy of APMPPE. Perfusion patterns of the retina, choriocapillaris and choroid are distinct in OCT-A. If a demarcation to other PICCP is possible, OCT-A could replace the invasive FA and indocyanine green angiography in diagnosis of APMPPE. In addition, our results support the thesis that APMPPE is an impairment of the choroidal circulation caused by an underlying inflammatory process, with secondary damage to the outer retina. This hypothesis needs to be validated by larger studies. If it holds true, clinical trials assessing the effectiveness of immunosuppressive and/or immunomodulating therapies in APMPPE are warranted.