Multimodal Imaging of Pigmented Paravenous Retinochoroidal Atrophy in a Pediatric Patient with Cystoid Macular Edema

Turk J Ophthalmol. 2022 Dec 28;52(6):432-435. doi: 10.4274/tjo.galenos.2022.39032.


The aim of this case report is to present the multimodal imaging characteristics of pigmented paravenous retinochoroidal atrophy (PPRCA) in a pediatric patient with cystoid macular edema (CME). A 7-year-old girl was admitted to our clinic with complaints of mild blurred vision and poor night vision. Best corrected visual acuity was 10/10 in both eyes. Fundus examination showed atrophic areas around the optic nerve and along the retinal vessels in both eyes. A few small dot-shaped paravenous pigmentations were observed in the mid-peripheral retina. Fundus autofluorescence was consistent with PPRCA. Spectral-domain optical coherence tomography (OCT) revealed the presence of CME and loss of the outer retinal layers outside the macula, with intact retinal layers in the macula. OCT angiography revealed normal choriocapillaris vasculature and flow. The patient was followed up for 6 months but showed no change in CME or clinical appearance. CME without ocular inflammation is an unusual finding of PPRCA and may suggest the involvement of chronic or latent inflammation in the etiology of PPRCA.

Keywords: Cystoid macular edema; optical coherence tomography angiography; pigmented paravenous retinochoroidal atrophy.

Publication types

  • Case Reports

MeSH terms

  • Atrophy
  • Child
  • Female
  • Fluorescein Angiography
  • Humans
  • Inflammation
  • Macular Edema* / diagnosis
  • Macular Edema* / etiology
  • Multimodal Imaging

Supplementary concepts

  • Pigmented Paravenous Chorioretinal Atrophy