Pigmented Paravenous Chorioretinal Atrophy: Clinical Spectrum and Multimodal Imaging Characteristics

Posterior vitreous cortex hyalocytes visualization in asymmetric pigmented paravenous chorioretinal atrophy (PPCRA) using en face OCT

“…Lee et al describe the OCT-A imaging characteristics of eyes affected by PPCRA. 12 In their studies, they noted relative preservation of retinal perfusion with significant choriocapillaris hypoperfusion, which suggests that PPCRA initially affects the choroidal vasculature. On the other hand, Battaglia et al found that there is reduced vessel density in the deep capillary plexus, and suggest that the initial insult occurs at the retinal circulation in this disease.…”

“… 4 , 8 , 9 , 11 Published reports involving fundus autofluorescence (FAF) and spectral domain optical coherence tomography (SD-OCT) have documented the presence of well-demarcated areas of hypoautofluorescence surrounded by a ring of hyperautofluorescence, thinning of the entire outer retina, and reduced choroidal thickness. 12 Optical coherence tomography angiography (OCT-A) has been recently utilized to investigate pathological alterations in vessel density and choriocapillaris porosity in patients with PPCRA. 12 , 13 , 14 , 15 , 16 These reports provide evidence of choriocapillaris hypoperfusion and reduced vessel density in the deep capillary plexus of the retina in this condition.…”

“… 12 Optical coherence tomography angiography (OCT-A) has been recently utilized to investigate pathological alterations in vessel density and choriocapillaris porosity in patients with PPCRA. 12 , 13 , 14 , 15 , 16 These reports provide evidence of choriocapillaris hypoperfusion and reduced vessel density in the deep capillary plexus of the retina in this condition. 13 , 16 …”

Elevated levels of Merkel cell polyoma virus in the anophthalmic conjunctiva

“…Multiple mechanisms have been hypothesized to account for the paucity of bacteria on the ocular surface, including the mechanical action of eyelid blinking, the presence of lysozyme and lactoferrin in tears, and the abundance of IgA in tears 8 , 9 . Recent research has also demonstrated a resident conjunctival virome including torque teno virus (TTV), a small single stranded DNA anellovirus 3 , 10 , 11 and Merkel cell polyoma virus (MCPyV), a small double stranded DNA polyomavirus 3 . Both TTV and MCPyV have also recently been described in intraocular fluids from post-operative endophthalmitis cases 10 , 12 .…”

“…Recent research has also demonstrated a resident conjunctival virome including torque teno virus (TTV), a small single stranded DNA anellovirus 3 , 10 , 11 and Merkel cell polyoma virus (MCPyV), a small double stranded DNA polyomavirus 3 . Both TTV and MCPyV have also recently been described in intraocular fluids from post-operative endophthalmitis cases 10 , 12 .…”