Author: Diogo Filipe Maleita (Portugal)
Co-authors: Diogo Maleita, Rita Serras-Pereira, Miguel Vieira, Ricardo Figueiredo, Rita Anjos, Rita Flores
Peripapillary pachychoroid syndrome (PPS) is a newly described entity from the pachychoroid disease spectrum (PDS). Distinctively, in PPS, the pachychoroid features are centered in the optic nerve, with peripapillary choroidal thickening, intraretinal and/or subretinal fluid and optic nerve head edema in some eyes. We report a case of an asymptomatic 60-year-old woman with PPS.
Ophthalmology department of Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
A 60 year’s old woman presented in a routine ophthalmology appointment for glaucoma screening due to positive family history. Complete ophthalmic examination was performed, which included assessment of best corrected visual acuity, biomicroscopy gonioscopy and fundoscopy. Optical coherence tomography (OCT) and Fluorescein Angiography (FA) images were obtained using the Spectralis®.
Upon examination, best corrected visual acuity (BCVA) was 10/10 in the right eye (OD) and left eye (OS). Slit-lamp biomicroscopy gonioscopy, intraocular pressures were all normal, and fundoscopy revealed minor peripapillary atrophy with normal optic discs in both eyes. Optical coherence tomography (OCT) showed no changes in the macula with a decrease in RNFL thickness in the nasal sector that warranted follow-up. An OCT was repeated 8 months later revealing de novo intraretinal fluid extending from the temporal margin of the optic. Six months later, another OCT showed, in both eyes, intraretinal fluid in the nasal macular region extending from the temporal margin of the optic disc, multiple small serous pigment epithelial detachments, peripapillary choroidal thickening and pachyvessels. Fluorescein Angiography demonstrated peripapillary granular transmission hyperfluorescence without leakage. Indocyanine green angiography revealed multiple peripapillary punctate hyperfluorescence spot lesions in mid-to late phase.
Peripapillary pachychoroid syndrome has a relatively benign course, with visual acuity stability, decrease in subfoveal subretinal fluid and nasal macular intraretinal fluid over time. For this reason, watchful waiting is usually the option. When progression is detected, treatments such as PDT and anti-VEGF may be considered. We report a well-documented initial case of this newly described entity. This patient was followed from normal baseline exams to the development of bilateral peripapillary pachychoroid syndrome in one year. Despite its good prognosis, diagnosis and awareness of this entity are in the patients' best interest.