Joanna Gołębiewska, Katarzyna Paczwa, Maciej Gawęcki
{"title":"Presentation and Course of Pachyvitelliform Maculopathy: A Case Series and Mini-Review.","authors":"Joanna Gołębiewska, Katarzyna Paczwa, Maciej Gawęcki","doi":"10.1159/000552616","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Pachyvitelliform maculopathy (PVM) is a novel phenotype within the spectrum of pachychoroid disorders featuring subretinal fluid and yellowish, hyperreflective, and hyperautofluorescent deposits. This study aimed to characterize PVM through multimodal imaging and evaluate therapeutic responses to guide clinical management.</p><p><strong>Case presentations: </strong>This retrospective study analyzed patient records from 2020 to 2025 at two ophthalmic centers. PVM was diagnosed based on subretinal hyperreflective material on optical coherence tomography (OCT), hyperautofluorescence, and increased choroidal thickness (>350 μm). Macular neovascularization was excluded via OCT angiography, fluorescein angiography (FA), and OCT. Outcomes following half-dose photodynamic therapy (hd-PDT) and subthreshold micropulse laser (SML) were evaluated. Seven patients with PVM were identified. At baseline, visual acuity did not exceed 0.5 Snellen in any case. Two eyes received hd-PDT (including one treated with combined SML and PDT), four received SML exclusively, and one was managed with observation only. Improvement in best corrected visual acuity occurred in 2 cases. While significant morphological restoration was observed in two eyes, it was also accompanied by atrophy of the outer retinal layers and retinal pigment epithelium. Only one patient achieved significant functional improvement following hd-PDT. One patient demonstrated the progressive evolution of vitelliform-like lesions from a simple form of central serous chorioretinopathy (CSC) over 60 months in the absence of prompt treatment.</p><p><strong>Conclusions: </strong>PVM represents a diagnostic challenge, often necessitating multimodal imaging to reliably distinguish it from macular dystrophies. Further large-scale studies are required to better establish the efficacy of available therapeutic approaches. Nevertheless, treatment modalities commonly employed in the management of CSC, such as PDT and SML, currently remain among the most viable options.</p>","PeriodicalId":9635,"journal":{"name":"Case Reports in Ophthalmology","volume":"17 1","pages":"728-738"},"PeriodicalIF":0.6000,"publicationDate":"2026-06-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13427032/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Ophthalmology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1159/000552616","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2026/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"OPHTHALMOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Introduction: Pachyvitelliform maculopathy (PVM) is a novel phenotype within the spectrum of pachychoroid disorders featuring subretinal fluid and yellowish, hyperreflective, and hyperautofluorescent deposits. This study aimed to characterize PVM through multimodal imaging and evaluate therapeutic responses to guide clinical management.
Case presentations: This retrospective study analyzed patient records from 2020 to 2025 at two ophthalmic centers. PVM was diagnosed based on subretinal hyperreflective material on optical coherence tomography (OCT), hyperautofluorescence, and increased choroidal thickness (>350 μm). Macular neovascularization was excluded via OCT angiography, fluorescein angiography (FA), and OCT. Outcomes following half-dose photodynamic therapy (hd-PDT) and subthreshold micropulse laser (SML) were evaluated. Seven patients with PVM were identified. At baseline, visual acuity did not exceed 0.5 Snellen in any case. Two eyes received hd-PDT (including one treated with combined SML and PDT), four received SML exclusively, and one was managed with observation only. Improvement in best corrected visual acuity occurred in 2 cases. While significant morphological restoration was observed in two eyes, it was also accompanied by atrophy of the outer retinal layers and retinal pigment epithelium. Only one patient achieved significant functional improvement following hd-PDT. One patient demonstrated the progressive evolution of vitelliform-like lesions from a simple form of central serous chorioretinopathy (CSC) over 60 months in the absence of prompt treatment.
Conclusions: PVM represents a diagnostic challenge, often necessitating multimodal imaging to reliably distinguish it from macular dystrophies. Further large-scale studies are required to better establish the efficacy of available therapeutic approaches. Nevertheless, treatment modalities commonly employed in the management of CSC, such as PDT and SML, currently remain among the most viable options.
期刊介绍:
This peer-reviewed online-only journal publishes original case reports covering the entire spectrum of ophthalmology, including prevention, diagnosis, treatment, toxicities of therapy, supportive care, quality-of-life, and survivorship issues. The submission of negative results is strongly encouraged. The journal will also accept case reports dealing with the use of novel technologies, both in the arena of diagnosis and treatment. Supplementary material is welcomed. The intent of the journal is to provide clinicians and researchers with a tool to disseminate their personal experiences to a wider public as well as to review interesting cases encountered by colleagues all over the world. Universally used terms can be searched across the entire growing collection of case reports, further facilitating the retrieval of specific information. Following the open access principle, the entire contents can be retrieved at no charge, guaranteeing easy access to this valuable source of anecdotal information at all times.