Author: Peggy Bouzika (Greece)
Co-authors: Maria-Evanthia Sotirianakou, Konstantinos Tyrlis, Stylianos Blounas, Aikaterini Mani, Tryfon Rotsos
Purpose
Peripapillary pachychoroid syndrome (PPS) is a relatively novel pathological entity within the pachychoroid disease spectrum; it is considered a distinct variant of central serous chorioretinopathy. It is characterized by pachychoroid features surrounding the optic nerve and is associated with intraretinal and/or subretinal fluid in the nasal macula. Optic nerve head edema has also been observed in some patients. The aim of our case report is to present the challenges in correctly diagnosing a patient with PPS.
Setting/Venue
Retinal Service, 1st Department of Ophthalmology, National and Kapodistrian University of Athens, Gennimatas General Hospital, Athens, Greece.
Methods
A 72-year old white male presented to our hospital complaining of painless, gradual visual loss. His visual acuity (VA) was 4/10 in the right eye and 5/10 in the left. Examination of the anterior segment showed cataract formation in the right eye, and was unremarkable in the left eye. Fundoscopy, optical coherence tomography (OCT), and indocyanine green angiography (ICGA) were also performed.
Results
Fundoscopy and OCT revealed cystoid macular edema (CME) in the left eye with no obvious pathology present. The patient was treated with a combination of dexamethasone and nepafenac eye drops with no clinical improvement. Intravitreal anti-VEGF treatment was also administered with similarly disappointing results. The patient received a combination of dorzolamide eye drops and acetazolamide tablets. CME persisted, and at this point the visual acuity in the left eye was counting fingers. The ICGA performed revealed optic disc edema, peripapillary choroidal thickening and diffuse choroidal leakage in the posterior pole, findings supportive of PPS in the left eye. Photodynamic therapy was applied peripherally at the leakage sites. The CME finally resolved and the patient’s vision significantly improved (VA=7/10).
Conlusions
It is important for ophthalmologists to be able to recognize and correctly treat this challenging and unique clinical entity. Its resemblance to disorders with similar features, such as posterior uveitis, and other neuro-ophthalmologic conditions, can lead to misdiagnoses and unnecessary, erroneous interventions. Further studies are warranted in order to gain more knowledge and experience in the management of patients with PPS.
Financial Disclosure
None
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