Clinical Case of Pigmented Paravenous Retinochoroidal Atrophy Complicated by Cystoid Macular Edema
DOI: 10.32364/2311-7729-2026-26-2-12
Clinical Case of Pigmented Paravenous Retinochoroidal Atrophy Complicated by Cystoid Macular Edema
V.G. Gvazava1, I.E. Panova1,2,3, K.K. Shefer1,2
1St. Petersburg Branch of the S. Fyodorov Eye Microsurgery Federal State Institution,
St. Petersburg, Russian Federation
2North-Western State Medical University named after I.I. Mechnikov, St. Petersburg, Russian Federation
3St. Petersburg State University, St. Petersburg, Russian Federation
Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare condition characterized by perivenous pigment clumping associated with peripapillary and radially oriented areas of retinochoroidal atrophy distributed along the retinal veins. The etiology remains uncertain. However, associations with inflammatory and infectious diseases, including sarcoidosis, Behcet disease, syphilis, measles, rubella, and tuberculosis, have been reported. Genetic associations involving variants in the CRB1, HK1, CRX, and RPGRIP1 genes have also been described. A clinical case of PPRCA complicated by bilateral cystoid macular edema in a 54-year-old patient is presented. The diagnosis was established based on characteristic clinical findings supported by multimodal imaging, including perimetry, fundus autofluorescence, fluorescein angiography, electroretinography, and optical coherence tomography. Treatment with local glucocorticosteroids (sub-Tenon injection of betamethasone) resulted in complete resolution of macular edema and improvement in best-corrected visual acuity in both eyes. This case demonstrates the effectiveness of local glucocorticosteroid therapy in managing cystoid macular edema in PPRCA, suggesting that sub-Tenon periocular injections may represent a viable treatment option. Multimodal imaging plays a key role in improving understanding of disease mechanisms, confirming the diagnosis, and assessing prognosis in this condition.
Keywords: pigmented paravenous retinochoroidal atrophy, cystoid macular edema, multimodal imaging, perimetry, autofluorescence, fluorescein angiography, electroretinogram, optical coherence tomography, topical glucocorticosteroids.
For citation: Gvazava V.G., Panova I.E., Shefer K.K. Clinical Case of Pigmented Paravenous Retinochoroidal Atrophy Complicated by Cystoid Macular Edema. Russian Journal of Clinical Ophthalmology. 2026;26(2):156–163 (in Russ.). DOI: 10.32364/2311-7729-2026-26-2-12
About the authors:
Viktoria G. Gvazava — Ophthalmologist, St. Petersburg Branch of S. Fyodorov Eye Microsurgery Federal State Institution; 21, Yaroslava Gasheka str., St. Petersburg, 192283, Russian Federation; ORCID iD 0000-0002-2765-0332
Irina E. Panova — Dr. Sc. (Med.), Professor, Deputy Director for Research, St. Petersburg Branch of S. Fyodorov Eye Microsurgery Federal State Institution; 21, Yaroslava Gasheka str., St. Petersburg, 192283, Russian Federation; Professor of the Department of Ophthalmology, North-Western State Medical University named after I.I. Mechnikov; 41, Kirochnaya str., St. Petersburg, 191015, Russian Federation; Professor of the Department of Otorhinolaryngology and Ophthalmology, St. Petersburg State University; 7–9, Universitetskaya emb., St. Petersburg, 199034, Russian Federation; ORCID iD 0000-0001-7443-4555
Kristina K. Shefer — C. Sc. (Med.), Associate Professor, Head of Pediatric Department, St. Petersburg Branch of S. Fyodorov Eye Microsurgery Federal State Institution; 21, Yaroslava Gasheka str., St. Petersburg, 192283, Russian Federation; Associate Professor of the Department of Ophthalmology, North-Western State Medical University named after I.I. Mechnikov; 41, Kirochnaya str., St. Petersburg, 191015, Russian Federation; ORCID iD 0000-0003-0568-6593
Contact information: Viktoria G. Gvazava, e-mail: gvazava-sochi@mail.ru
Financial Disclosure: no authors have a financial or property interest in any material or method mentioned.
There is no conflict of interest.
Received 19.03.2025.
Revised 10.04.2025.
Accepted 05.05.2025.
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