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Fovea centralis, located at the center of the macula, is packed with cone photoreceptors and is responsible for central visual acuity. Isolated foveal photoreceptor disruption may occur in a variety of hereditary, degenerative, traumatic, and toxic chorioretinal diseases. These have been known previously by multiple synonyms including macular microhole, foveal spot, and outer foveal microdefects. A common clinical feature underlying these conditions is the presence of apparently normal fovea or subtle hypopigmented lesion at the foveal or juxtafoveal area. A detailed history along with high-resolution optical coherence tomography is often helpful to derive a conclusive diagnosis in majority of these cases. Focal photoreceptor disruption usually involves loss or rarefaction of ellipsoid/interdigitation zone, either in isolation or associated with external limiting membrane or retinal pigment epithelium disruption in the fovea. Vitreomacular interface (VMI) disorders including vitreomacular traction, posterior vitreous detachment, epiretinal membrane, and impending macular hole possibly remain the most common cause. Retinal dystrophies such as cone dystrophy, occult macular dystrophy, and achromatopsia may present with diminution of vision and normal appearing fundus in a younger age group. Other causes include photic retinopathy (e.g., from a history of sun gazing or laser pointer exposure), blunt trauma, drug exposure (e.g., poppers maculopathy or tamoxifen retinopathy), and acute retinal pigment epitheliopathy (ARPE). Visual prognosis depends on the underlying etiology with complete recovery common in the subset of patients with VMI, and ARPE, whereas persistent outer retinal defects are the rule in other conditions. We discuss the differential diagnoses that lead to isolated foveal photoreceptor defects. Identifying and understanding the underlying disease processes that cause foveal photoreceptor disruption may help predict visual prognosis., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: The aim of this systematic review and meta-analysis was to provide an overview of reported cases of poppers maculopathy and a statistical analysis of the clinical presentations, anatomical changes and treatment and follow-up of the rare disease., Methods: A systematic search of CINAHL, Embase and PubMed databases was conducted, including a search of the grey literature. Data were pooled to provide a summary of the clinical characteristics and outcomes of poppers maculopathy. Studies were critically appraised using the Joanna Briggs Institute or the Authority, Accuracy, Coverage, Objectivity, Date, Significance checklists., Results: Of the 493 articles obtained, 32 were included, consisting of 113 participants with a mean age of 40.2. Most patients were male with bilateral maculopathy and presented with either vision loss or a visual disturbance. Cessation of popper use was the most common form of management prescribed. The mean VA at baseline was 0.22 (logMAR units). For those followed-up, the mean VA significantly improved from 0.24 to 0.11 (Z = -5.800, p = <0.001). Bilateral yellow foveal spots were viewed on fundoscopy in 53 patients, and a sub-foveal disturbance of the ellipsoid layer was reported on spectral domain optical coherence tomography in 89 patients. By 6 months, SD-OCT anatomical changes mostly improved or resolved., Conclusion: Recreational users of poppers who develop maculopathy generally present with vision loss or a visual disturbance secondary to foveal disruption. Patients have a good visual prognosis, with cessation of poppers potentially having a role in improved outcomes. With the high prevalence of use among gay and bisexual men, awareness of related maculopathy and presenting symptoms by clinicians is important to mitigate side effects.

Purpose: To describe a case of poppers maculopathy by means of multimodal imaging., Methods: A 26-year-old male presented to our Department in April 2018 with a history of visual disturbances. He underwent a complete ophthalmologic examination, including optical coherence tomography, fundus autofluorescence, and ultra-widefield fundus photograph., Results: At baseline, the patient presented a visual acuity of 20/25 Snellen bilaterally, with an altered foveal reflex on funduscopic examination. Structural optical coherence tomography showed disruption of the outer retinal layers in both eyes. After 6-month observation, the status of the outer retinal layers on optical coherence tomography and his vision (20/20) appeared improved. In this occasion, we performed optical coherence tomography angiography that revealed clear and persisting choriocapillaris alterations. Upon further interrogation, he admitted the episodic use of poppers, hence leading to the diagnosis., Conclusion: Our findings suggest that microvascular toxicity at the level of choriocapillaris may act as primary pathogenetic insult in poppers maculopathy.

The article presents a clinical case of poppers-associated maculopathy - a maculopathy of toxic genesis, insufficiently represented in Russian scientific literature. The diagnosis was based on anamnestic data (long-term use of poppers), specific structural disorders of the outer layers of the retina in the foveolar zone according to spectral optical coherence tomography, as well as changes in multifocal and ganzfeld electroretinography readings. The main strategy for patients with this disorder is complete rejection of poppers.

Background: "Poppers" is a slang term for a group of alkyl nitrites that are used as recreational drugs. Their inhalative intoxication leads to muscle relaxation, analgesia, and euphoria. Maculopathy is a rare but serious side-effect., Patients/methods: Clinical, imaging, and electrophysiological findings of seven patients with maculopathy after consumption of poppers were presented., Results: All seven patients were male with a median age of 35 years (range 28-45 years), the median duration of periodical poppers use until the onset of symptoms was 9.8 years (one day to 25 years). Five of seven patients were HIV-positive, one patient was negative, and the HIV-status of one patient was unknown. Median average of visual acuity at presentation was 20/30 in each eye. In all patients, optical coherence tomography (OCT) showed pathognomonic alterations of the outer foveal retina. One patient showed an almost complete restitution of the maculopathy six months after cessation of drug use and following the oral intake of Lutein. Imaging alterations returned to normal and visual acuity recovered from 20/50 and 20/30 (right and left eye, respectively) to 20/20 on both eyes. Follow up of two other cases showed no relevant functional decline or improvement., Discussion: Toxic maculopathy due to the consumption of poppers is an important differential diagnosis in acute visual loss without clinico-morphological correlate. Optical coherence tomography is the only reliable diagnostic tool in these cases. Complete recovery of visual function and macular morphology is rare, even after cessation of drug abuse. Oral lutein therapy may have a beneficial effect.