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Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity

Two cases with peripapillary pachychoroid syndrome (PPS) along with the challenges concerning correct diagnosis and treatment are presented. In the first case, the patient presented with painless unilateral gradual visual loss. Fundoscopy and optical coherence tomography (OCT) revealed cystoid macul...

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Autores principales: Bouzika, Peggy, Georgalas, Ilias, Sotirianakou, Maria-Evanthia, Karamaounas, Aristotelis, Symeonidis, Chrysanthos, Tyrlis, Konstantinos, Blounas, Stylianos, Mani, Ekaterini, Rotsos, Tryfon
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9167143/
https://www.ncbi.nlm.nih.gov/pubmed/35669885
http://dx.doi.org/10.1155/2022/9124630
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author Bouzika, Peggy
Georgalas, Ilias
Sotirianakou, Maria-Evanthia
Karamaounas, Aristotelis
Symeonidis, Chrysanthos
Tyrlis, Konstantinos
Blounas, Stylianos
Mani, Ekaterini
Rotsos, Tryfon
author_facet Bouzika, Peggy
Georgalas, Ilias
Sotirianakou, Maria-Evanthia
Karamaounas, Aristotelis
Symeonidis, Chrysanthos
Tyrlis, Konstantinos
Blounas, Stylianos
Mani, Ekaterini
Rotsos, Tryfon
author_sort Bouzika, Peggy
collection PubMed
description Two cases with peripapillary pachychoroid syndrome (PPS) along with the challenges concerning correct diagnosis and treatment are presented. In the first case, the patient presented with painless unilateral gradual visual loss. Fundoscopy and optical coherence tomography (OCT) revealed cystoid macular edema (CME) in the left eye (LE), extending from the temporal optic disc margin towards the fovea, with no additional findings. Enhanced-depth imaging- (EDI-) OCT provided additional information and increased choroidal thickness nasally to the macula and pachyvessels in the outer choroidal layer, findings supportive of PPS. Photodynamic therapy (PDT) was applied at the leakage sites. Two months later, CME and subretinal fluid (SRF) had resolved, and VA had significantly improved. In the second case, a patient presented with reduced vision and metamorphopsia bilaterally over the previous 5 days. Fundoscopy revealed CME in both eyes. OCT confirmed the presence of CME in the papillomacular area in the right eye; similarly, CME was recorded in the macula of the LE with SRF located subfoveally. EDI-OCT showed increased choroidal thickness in both eyes. Treatment was administered, originally with dorzolamide eye drops along with eplerenone tablets, and then dexamethasone eye drops that eventually led to significant anatomic and functional improvement. It is important for ophthalmologists to be able to recognize the unique clinical entity of PPS, as its resemblance to disorders with similar features may lead to misdiagnoses and unnecessary, or even incorrect, interventions.
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spelling pubmed-91671432022-06-05 Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity Bouzika, Peggy Georgalas, Ilias Sotirianakou, Maria-Evanthia Karamaounas, Aristotelis Symeonidis, Chrysanthos Tyrlis, Konstantinos Blounas, Stylianos Mani, Ekaterini Rotsos, Tryfon Case Rep Ophthalmol Med Case Report Two cases with peripapillary pachychoroid syndrome (PPS) along with the challenges concerning correct diagnosis and treatment are presented. In the first case, the patient presented with painless unilateral gradual visual loss. Fundoscopy and optical coherence tomography (OCT) revealed cystoid macular edema (CME) in the left eye (LE), extending from the temporal optic disc margin towards the fovea, with no additional findings. Enhanced-depth imaging- (EDI-) OCT provided additional information and increased choroidal thickness nasally to the macula and pachyvessels in the outer choroidal layer, findings supportive of PPS. Photodynamic therapy (PDT) was applied at the leakage sites. Two months later, CME and subretinal fluid (SRF) had resolved, and VA had significantly improved. In the second case, a patient presented with reduced vision and metamorphopsia bilaterally over the previous 5 days. Fundoscopy revealed CME in both eyes. OCT confirmed the presence of CME in the papillomacular area in the right eye; similarly, CME was recorded in the macula of the LE with SRF located subfoveally. EDI-OCT showed increased choroidal thickness in both eyes. Treatment was administered, originally with dorzolamide eye drops along with eplerenone tablets, and then dexamethasone eye drops that eventually led to significant anatomic and functional improvement. It is important for ophthalmologists to be able to recognize the unique clinical entity of PPS, as its resemblance to disorders with similar features may lead to misdiagnoses and unnecessary, or even incorrect, interventions. Hindawi 2022-05-28 /pmc/articles/PMC9167143/ /pubmed/35669885 http://dx.doi.org/10.1155/2022/9124630 Text en Copyright © 2022 Peggy Bouzika et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Bouzika, Peggy
Georgalas, Ilias
Sotirianakou, Maria-Evanthia
Karamaounas, Aristotelis
Symeonidis, Chrysanthos
Tyrlis, Konstantinos
Blounas, Stylianos
Mani, Ekaterini
Rotsos, Tryfon
Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity
title Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity
title_full Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity
title_fullStr Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity
title_full_unstemmed Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity
title_short Peripapillary Pachychoroid Syndrome (PPS): Diagnosing and Treating a Rare Entity
title_sort peripapillary pachychoroid syndrome (pps): diagnosing and treating a rare entity
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9167143/
https://www.ncbi.nlm.nih.gov/pubmed/35669885
http://dx.doi.org/10.1155/2022/9124630
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