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Multimodal Imaging of Pigmented Paravenous Retinochoroidal Atrophy in a Pediatric Patient with Cystoid Macular Edema
Jale Menteş1, Cumali Değirmenci1
1Ege University Faculty of Medicine, Department of Ophthalmology, İzmir, Turkey.
Insights
This case report details a pediatric patient with pigmented paravenous retinochoroidal atrophy (PPRCA) and unusual cystoid macular edema (CME). Findings suggest potential chronic inflammation in the etiology of PPRCA.
Area of Science:
- Ophthalmology
- Medical Imaging
- Retinal Diseases
Background:
- Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare condition.
- Cystoid macular edema (CME) is an uncommon complication of PPRCA, especially in pediatric cases.
Observation:
- A 7-year-old girl presented with blurred vision and poor night vision.
- Multimodal imaging, including OCT and OCT angiography, revealed PPRCA with CME and outer retinal layer loss.
- Fundus autofluorescence confirmed PPRCA findings.
Findings:
- The pediatric patient exhibited typical PPRCA features alongside unusual CME.
- Spectral-domain OCT showed CME and outer retinal atrophy, with macular sparing.
- OCT angiography demonstrated normal choriocapillaris, ruling out significant vascular compromise.
Implications:
- CME in PPRCA, particularly without overt inflammation, is an unusual presentation.
- This finding may indicate a role for chronic or latent inflammation in PPRCA pathogenesis.
- Further research is needed to understand the link between inflammation and PPRCA development.
Abstract:
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.
