Pigment Epithelium Detachment in Severe Acute Posterior Multifocal Placoid Pigment Epitheliopathy

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PURPOSE: To describe the spectrum of optical coherence tomography (OCT) findings in Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), with a specific focus on the prevalence of pigment epithelium detachments (PEDs). METHODS: Multicenter, retrospective, longitudinal study including patients diagnosed with APMPPE based on color fundus photography, OCT and fluorescein angiography. Demographics, visual acuity, and the following OCT findings were assessed: outer retinal hyperreflectivity (ORH), serous retinal detachment (SRD), bacillary layer detachment (BALAD), and PED. RESULTS: Forty-six eyes of 27 patients were included. The median age at presentation was 23 years (range: 18-36 years), and 17 patients (62.9%) were men. At baseline, ORH was found in all eyes, while BALAD, SRD, and PED were observed in 22 (47.8%), 14 (30.4%), and 26 eyes (56.5%), respectively. A univariate logistic regression showed a significant association between the presence of PED and a worse baseline visual acuity (odds ratio [OR]: 5.3; 95% confidence interval [CI]: 1.5-18.6; p=0.01), the presence of BALAD (OR: 9.0; 95% CI: 2.3-35.6; p=0.002), and the presence of SRD (OR: 7.3; 95% CI: 1.4-38.3; p=0.02). CONCLUSION: PEDs were significantly associated with a poorer initial visual acuity, as well as with the presence of BALAD and SRD upon presentation. These findings suggest that PEDs could be used as a biomarker of disease severity and choriocapillaris ischemia in APMPPE.

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