Peripapillary Pachychoroid Syndrome (PPS)

Optomap (1), red separation (2) and green separation (3) images - left eye

Ultra widefield imaging shows linear areas of hypo-pigmentation in the peripapillary retina and scattered focal hypo-pigmented areas scattered through the posterior pole. The optic disc has a small, crowded appearance.

Fundus autofluorescence image (left eye)

FAF shows mixed hyper and hypo-fluorescence in the areas of pigmentary change.

Spectralis OCT line scans - superior right macula (1) and right optic disc (2)

OCT shows the presence of peripapillary intra-retinal fluid.

Spectralis OCT line scans (left optic disc)

OCT shows the presence of peripapillary sub- and intra-retinal fluid.

Spectralis OCT line scans (left macula)

Macular line scans show intra- and sub-retinal fluid in the peripapillary retina. Shallow pigment epithelial detachments and disruption of the RPE may also be noted in each image.

Colour fundus photography

Posterior pole photography shows an area of hypo-pigmentation nasal to the right optic disc, likely due to the presence of SRF. There are also hypopigmentary changes at both maculae, more prominent in the right.

Red-free images

Fundus autofluoresence (FAF) imaging

FAF imaging shows scattered points of hyper-AF in the posterior pole of both eyes, mostly located adjacent to the optic disc. There are subtle hypo-FAF changes surrounding both maculae

Cirrus OCT radial scan (right macula)

A radial scan at the right optic disc shows a large PED at the infero-temporal edge of the scan with smaller one adjacent temporal to the disc. SRF adjacent to the disc and intra-retinal fluid adjacent to the PEDs can be noted . Large diameter choroidal vessels and compression of the choriocapillaris can also be noted.

Cirrus OCT macular line scan (Left macula)

An OCT line scan through the left macula shows multiple serous PEDs.

Cirrus OCT volume scan (right macula)

An OCT volume scan around the right macula shows multiple PEDs with peripapillary SRF visible. A small FIPED is visible in the middle scan. OCT angiography showed no signs consistent with choroidal neovascularisation.