Pigmented paravenous retinochoroidal atrophy with acute angle-closure glaucoma and posterior subcapsular cataract: a case report

• Main Authors: Li, J. (Author), Sun, Y. (Author), Yu, L. (Author), Zheng, Y. (Author)
• Format: Article
• Language: English
• Published: BioMed Central Ltd 2022
• Subjects: Acute angle-closure glaucoma; atrophy; Atrophy; case report; cataract; Cataract; choroid; Choroid; closed angle glaucoma; eye disease; Eye Diseases, Hereditary; female; Female; Fluorescein Angiography; fluorescence angiography; genetics; Glaucoma, Angle-Closure; human; Humans; middle aged; Middle Aged; optical coherence tomography; pathology; Pigmented paravenous retinochoroidal atrophy; Posterior subcapsular cataract; procedures; retina degeneration; Retinal Degeneration; retinitis pigmentosa; Retinitis pigmentosa; Retinitis Pigmentosa; Tomography, Optical Coherence
• Online Access: View Fulltext in Publisher

 Background: Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare fundus disease characterized by the presence of osteoblast-like pigment, atrophy of retinal pigment epithelium (RPE), and choroid deposition along the large retinal veins. Case presentation: A 55-year-old Chinese female presented with right eye distention and bilateral vision loss. Osteocyte-like pigmentation and retinal choroidal atrophy distributed along the large retinal veins were seen in the fundus of bilateral eyes. The atrophy in the left eye was more severe compared to the right eye. The patient also presented with bilateral acute angle-closure glaucoma (AACG) and posterior subcapsular cataract (PSC) accompanied with anterior segmental manifestations, similar to the complications of retinitis pigmentosa (RP). The patient underwent ultrasound biomicroscopy (UBM), Humphrey field analyser (HFA), optical coherence tomography (OCT), fundus autofluorescence (FAF), fluorescein fundus angiography (FFA), electroretinogram (ERG), and electrooculography (EOG), all of which confirmed the aforementioned diagnose. Conclusion: PPRCA is a rare disease of unknown etiology. The patient in this case presented with complications similar to those of RP, and the two conditions may share a genetic basis. Further studies are needed to confirm this relationship. © 2022, The Author(s).