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

Abstract

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.

WITHDRAWN: Pigmented paravenous retinochoroidal atrophy caused by VPS13B gene mutations: a case report

“…Pigmented paravenous retinochoroidal atrophy (PPRCA) is a rare retinal degenerative disorder, characterized by the presence of areas of retinochoroidal atrophy originating at the optic disc and extending along the retinal veins [1,2]. The etiology and mechanisms underlying this condition are yet to be fully understood.…”

Pigmented paravenous chorioretinal atrophy: Updated scenario

“…Electrooculography (EOG) may display normal, subnormal or significantly reduced Arden ratio. 3 , 8 , 38 , 48 In a recent study, EOG findings were considerably proportionate with scotopic ERG findings, which, together with the observation that many cases of unilateral PPCRA have been described, suggests a generalized disfunction of the photoreceptor/RPE interface. 8 Finally, in some patients dark adaptation is delayed with an increased rod-treshold.…”

Unilateral pigmented paravenous retinochoroidal atrophy with acute angle-closure glaucoma: a case report

“…Comparison between the two patients are summarized in Table 1 . They hypothesized that PPRCA triggered RPE and choroidal atrophy, making eyes less tolerant to high IOP than average glaucomatous eyes and speculated that PPRCA might be an incomplete manifestation of RP, and the mechanisms of co-occurrence of AACG was similar to that of RP complicated by the later disease [ 18 ]. Xu et al found patients with primary angle-closure glaucoma (PACG) associated with RP had the same biometric parameter characteristic as the patients with chronic PACG and acute PACG, suggesting that RP may have a coincidental relationship with angle-closure glaucoma [ 19 ].…”