Pachychoroid Spectrum Disorder Findings in Patients with Coronavirus Disease 2019

Purpose. To report the occurrence of acute, bilateral, central serous chorioretinopathy (CSC), and pachychoroid spectrum disorder findings in patients with coronavirus disease 2019 (COVID-19). Methods. In recovered cases of COVID-19 with visual disturbances, complete ocular examinations with multimodal retinal and choroidal evaluation, including enhanced depth imaging optical coherence tomography, fluorescein or indocyanine green angiography, and blue autofluorescence, were obtained. Results. Four COVID-19 recovered patients presented with bilateral blurred vision. Ocular examination and imaging revealed pachychoroid and pachyvessels associated with choroidal hyperpermeability without any obvious intraocular inflammation. Bilateral localized serous retinal detachment was obvious in three cases compatible with pachychoroid associated with CSC manifestation and pachychoroid pigment epitheliopathy in one patient. CSC was resolved with treatment by steroidal antimineralocorticoid (Eplerenone) in two patients and by photodynamic therapy in one patient. None of the patients reported emotional stress and history of corticosteroid consumption. Conclusion. Hyperpermeability of the choroid, pachychoroidopathy, or choroidal vessel congestion can be observed or exacerbated in association with COVID-19.

Pachychorioidealis kórképek

Összefoglaló. Célunk, hogy közleményünkben összefoglaljuk a pachychorioidealis kórképekkel kapcsolatos ismereteket egy-egy saját esettel illusztrálva. Az irodalmi adatok és a saját klinikai tapasztalatok alapján összegeztük a pachychorioidealis kórképekkel kapcsolatos ismereteinket, az alcsoportok kezelési lehetőségeiről összefoglaló folyamatábrát készítettünk. A pachychorioidealis kórképekbe a következő betegségek tartoznak: centrális serosus chorioretinopathia (CSCR), pachychorioidealis pigmentepitheliopathia (PPE), pachychorioidealis neovasculopathia (PNV), polypoid chorioidealis vasculopathia (PCV), peripapillaris pachychorioidealis syndroma (PPS), focalis chorioideaexcavatio (FCE). A pachychorioidealis kórképek közös jellemzője a chorioidea kvantitatív vagy kvalitatív eltérései, melyekhez gyakran subretinalis folyadékgyülem társul. A betegségcsoportnak jelenleg nincs standard kezelési protokollja; a többféle kezelési mód közül néhány hatékonyabbnak bizonyul, az alcsoportok között azonban lényeges különbségek mutatkoznak. Összegezzük, hogy melyik alcsoportban érdemes eplerenonetablettás kezeléssel, mikropulzuslézer-kezeléssel, verteporfinos fotodinámiás kezeléssel (PDT) vagy intravitrealis anti-VEGF-injekciós kezeléssel kezdeni. Orv Hetil. 2020; 162(20): 770–781. Summary. The aim of this study is to present our knowledge about pachychoroid diseases using case reports, literature review and our own clinical experiences. A summary flow chart of treatment options for the subgroups was prepared, too. Pachychoroid diseases include the following: central serous chorioretinopathy (CSCR), pachychoroid pigment epitheliopathy (PPE), pachychoroid neovasculopathy (PNV), polypoidal choroidal vasculopathy (PCV), peripapillary pachychoroid syndrome (PPS), focal choroidal excavation (FCE). A common feature of pachychoroid diseases is the quantitative or qualitative abnormality of the choroidea, which is often associated with subretinal fluid accumulation. The disease group does not currently have a standard treatment protocol; some of the multiple treatments prove to be more effective, however, there are significant differences between the subgroups. We summarize which subgroup benefits from eplerenone tablet therapy, micropulse laser therapy, verteporfin photodynamic therapy or intravitreal anti-VEGF injection therapy. Orv Hetil. 2020; 162(20): 770–781.

Pachychoroid: current concepts on clinical features and pathogenesis

Purpose The term “pachychoroid” refers to a newly described phenotype in which functional and structural choroidal changes are thought to play a key pathogenic role in a spectrum of related retinal disorders. A more detailed understanding of how the choroid is involved within this spectrum and a better knowledge of the most relevant clinical signs of the pachychoroid phenotype are important to differentiate these disorders from other retinal conditions. Our objectives are to provide a literature review of pachychoroid and the commonalities that may be present across pathologies included in the spectrum, and to provide details on the examination, monitoring, and management of these disorders. Methods We searched the PubMed web platform to identify relevant studies using the following keywords: pachychoroid, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, aneurysmal type 1 neovascularization, focal choroidal excavation, peripapillary pachychoroid syndrome, vasculopathy pachysclera, pachychoroid geographic atrophy, and pachydrusen. We selected 157 publications and identified the most important features related to pachychoroid. Results The presence of hypertrophic or congested vessels in the choroid, not thickened choroid per se, under an area of reduced or absent choriocapillaris in the posterior pole seems to be the most salient feature of pachychoroid. However, other qualitative/quantitative features are needed to differentiate the uncomplicated pachychoroid from the pathological pachychoroid clinical spectrum, which may be associated with exudation, neovascularization, and/or retinal pigment epithelium and photoreceptor atrophy. Conclusions The most salient feature of pachychoroid appears to be the presence of large vessels under an area of reduced or absent choriocapillaris. Knowledge of the features and pathogenesis of the different disorders in the pachychoroid spectrum may assist in the management of patients.

Overview and Classification of Pachycoroid Spectrum Diseases

Pachychoroid spectrum has been defined recently and the diseases of this spectrum share some common choroidal features. The aim of the current review is to provide an overview of characteristic features and classification of pachychoroid spectrum including uncomplicated pachychoroid, pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polipoidal choroidal vasculopathy, peripapillary pachychoroid syndrome, and focal choroidal excavation.

Epidemiology, Prevalence And Incidence in Pachychoroid Disease Spectrum

Pachychoroid is a relatively novel concept describing a phenotype characterized by increased choroidal thickening, pathologically dilated veins in the Haller’s layer (pachy-veins), thinning in Sattler’s and choriocapillaris layers. The spectrum comprises the following disease groups: Pachychoroid Pigment Epitheliopathy, Central Serous Chorioretinopathy, Pachychoroid Neovasculopathy, Polypoidal Choroidal Vasculopathy, Focal choroidal excavation, Peripapillary pachychoroid syndrome. These conditions all exhibit the characteristic choroidal alterations and are believed to represent different manifestations of a common pathogenic process. The aim of this review is to exhibit the epidemiology, risk factors, incidence and prevalence of the pachychoroid diseases respectively.

Genetics, Risk Factors and Pathogenesis in the Spectrum of Pachychoroid Diseases

The spectrum of pachychoroid disease was first described by Warrow et al. in 2013. The characteristics of pachyoroid phenotype are dilatation of the vessels in the Haller layer (pachyvessels), thinning of the layers of Sattler, and choriocapillaris. Dilated choroidal vessels, choroidal hyperpermeability, diffuse or focal choroidal thickness and associated with progressive retinal pigment epithelium (RPE) dysfunction are thought to be in the pathogenesis. Angiographic findings such as choroidal vascular hyperpermeability, especially in the region of RPE changes, and delay in choroidal filling suggest that the primary pathology is associated with choroidal vascular disturbance. However, the exact pathophysiological mechanisms of the disease are not fully known. Pakikoroid-related diseases include pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy, focal choroidal excavation, and peripapillary pachychoroid syndrome. These diseases are thought to be reveal different manifestations of common pathological mechanisms. This review highlights the current understanding of genetics, risk factors, and pathogenesis in the spectrum of pachychoroid disease based on the current literature.

Pachychoroid Pigment Epitheliopathy; Signs, Diagnosis, and Management

Pachychoroid (ancient Greek pachy: thick) means thick choroid. Pachychoroid pigment epitheliopathy (PPE) has been recently defined as a forme fruste of central serous chorioretinopathy (CSC). Its characteristic findings are pachychoroid phenotype, a variety of RPE abnormalities, absence of subretinal fluid, and absence of a history of subretinal fluid. PPE may progress to CSC or pachychoroid neovasculopathy. The aim of the current review is to provide an overview of signs, diagnosis, and management of PPE.