A 73-year-old male patient complained of a dark area in the visual field in both his eyes. He has a history of IM steroid injection (2 months ago) and primary open-angle glaucoma. Intraocular pressures were undercontrol with topical medications. A cataract was detected in the right eye. In the anterior segment and vitreous examination, there is no sign of inflammation in both eyes. His visual acuity was 20/40 in the right eye and 20/25 in the left eye. OCT scans reveal that both eyes have larger cysts in the outer nuclear layer and small cysts in the inner nuclear layer at the nasal side of the macula. Both eyes have enlarged Haller’s layer vessels at the nasal side. Fluorescein angiography (FA) shows bilateral window defect hyperfluorescense around the optic disc secondary to peripapillary atrophy. However, there is no sign of cystoid macular edema or choroidal neovascularization in both eyes. The mid phase of indocyanine green angiography (ICG) shows a significant choroidal hyperpermeability between the fovea and optic disk and punctate hyperfluorescence spots at the upper field. Negative staining of the large choroidal vessels in late-phase indocyanine green angiography is another finding indicating choroidal hyperpermeability. These ICG features are characteristics of pachychoroid diseases.
Why do we see cysts in a pachychoroid patient without any sign of chronicity in autofluorescence image?
This condition occurs due to peripapillary atrophy. Note that there is no photoreceptor or external limiting membrane and RPE is atrophic adjacent to the optic disk. In classic central serous, the fluid arising from the choroid accummulates under the photoreceptor layer. However, due to the absence of these preventive tissues, the fluid arising from the choroid passes directly to the intraretinal area in these cases.
Old age, bilateral involvement, all pachychoroid features, peripapillary atrophy, intraretinal fluid, and absence of CME in FA are characteristics of Peripapillary Pachychoroid Syndrome. Subretinal fluid, short axial length, choroidal folds, and disk leakage in FA can also be present.
You will find the images of this case below. You can also watch the video of the comparative presentation of two cases: peripapillary pachychoroid syndrome and acquired optic pit secondary to glaucoma.
Credit: M. Giray Ersoz, MD, FEBO
Biruni University School of Medicine, Department of Ophthalmology, Istanbul, Turkey
Instagram accounts: @retina.review and @retina.dr.girayersoz
Multimodal Imaging
OCT of peripapillary pachychoroid syndrome (OD and OS)
FA of peripapillary pachychoroid syndrome (OD and OS)
ICG of peripapillary pachychoroid syndrome (OD, mid and late phase)
ICG of peripapillary pachychoroid syndrome (OS, mid and late phase)
