Management of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE): Insights from Multimodal Imaging with OCTA

Abstract

A 28-year-old man presented to the emergency room with blurred vision in the right eye for two days. He reported a preceding flu-like illness one week earlier. His best-corrected visual acuity (BCVA) was 20/40 in the right eye and 20/25 in the left eye. There was no anterior chamber inflammation or vitritis in either eye. He presented multiple yellowish-white placoid lesions in the posterior pole, some involving the foveal area, bilaterally. General examination and systemic investigation were unremarkable. Multimodal evaluation with fluorescein angiography, indocyanine green angiography, and spectral domain and optical coherence tomography angiography (OCTA) were consistent with the diagnosis of acute posterior multifocal placoid pigment epitheliopathy. Due to centromacular involvement with decreased BCVA, treatment with oral methylprednisolone was started after infectious causes were ruled out. After two weeks, the patient presented functional and anatomical improvement. OCTA showed partial reperfusion of the choriocapillaris in the affected areas, in both eyes.

Case Report: Acute Posterior Multifocal Placoid Pigment Epitheliopathy after SARS-CoV-2 Vaccination

“…This case is unique for its unilateral presentation that occurred 2 weeks after administration of the first dose of the Pfizer-BioNTech (Pfizer, New York, NY; BioNTech, Mainz, Germany) vaccine against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a healthy adolescent boy without a history of the novel coronavirus disease (COVID-19) infection that was discovered in December 2019. The typical fundus lesions along with a mild vitritis that developed during the disease are most consistent with the diagnosis of acute posterior multifocal placoid pigment epitheliopathy 1 – 4 . The authors strive to bring awareness about this possible ocular sequela of the novel SARS-CoV-2 messenger RNA (mRNA) vaccine to the eye care community.…”

“…Acute posterior multifocal placoid pigment epitheliopathy is an uncommon idiopathic inflammatory chorioretinopathy characterized by multiple creamy subretinal lesions. It typically occurs in young adults between the ages of 20 and 40 years with equal predilection for sex and race 1 – 4 . Although the incidence of the disease is unclear, it has been reported to occur in 0.15 individuals per 100,000, and there may be an increased risk in individuals possessing the human leukocyte antigens B27 and DR2 5 .…”

“… 3 Although treatment is often unnecessary because the disease is self-limiting, some patients may benefit from oral steroids, particularly if macular lesions are present. 3 , 4 Although the presentation is typically bilateral in 75% of patients, acute posterior multifocal placoid pigment epitheliopathy may initially begin in one eye, with the fellow eye demonstrating lesions days to weeks after the first eye is affected. 6 This case is unique for its unilateral presentation that occurred 2 weeks after administration of the first dose of the Pfizer-BioNTech (Pfizer, New York, NY; BioNTech, Mainz, Germany) vaccine against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in a healthy adolescent boy without a history of the novel coronavirus disease (COVID-19) infection that was discovered in December 2019.…”

Acute posterior multifocal placoid pigment epitheliopathy (APMPPE)

“…On spectral-domain optical coherence tomography (SD-OCT), the placoid lesions in the acute stage appear as disruption of the outer retinal and ellipsoid zone, with the presence of hyperreflective material at the level of the outer retinal layers and RPE (Fig. 2 A) [24][25][26]40]. Over the course of resolution of the placoid lesions, the hyperreflectivity of the outer retina disappears, resulting in either partial restoration of its appearance or focal areas of photoreceptors/RPE atrophy (Fig.…”

Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) after Influenza Vaccine followed by a Facial Palsy: Case Report

“…As a result, occurs an atrophy of the choriocapillaris, RPE, and photoreceptors. So actually, the choroidopathy may be a more appropriate name than epitheliopathy [5].…”