Pigmented paravenous retinochoroidal atrophy: a case report

Abstract

BackgroundPigmented paravenous retinochoroidal atrophy (PPRCA) is an unusual retinal degeneration, and its performance on optical coherence tomography angiography (OCTA) is unclear. We report a Chinese female case of PPRCA and her OCTA features.Case presentationA 66-year-old female patient was referred to the author’s center for gradual progressive loss of vision in both eyes and photophobia of 2 years duration. She reported having no family history of inherited ocular diseases. The funduscopic examination revealed bone-spicule pigmentation and retinochoroidal atrophy along the retinal veins. This patient was diagnosed with PPRCA which is a rare disease, uncommon in females, more commonly affecting the paravascular fundus. Noninvasive imaging techniques features of this patient was described, including ultra-wide field fundus autofluorescence, spectral domain optical coherence tomography (SD-OCT), OCTA (SSADA), etc. The en face OCTA images demonstrated areas of flow void beneath the retinal pigment epithelium-Bruch membrane layer suggestive of choriocapillaris hypoperfusion that corresponded with indocyanine green angiography (ICGA). Further studies should be conducted to clarify the relationship between choriocapillaris hypoperfusion and the development of PPRCA.ConclusionsThe OCTA features in patients with PPRCA has not been described previously in the literature. This case might provide preliminary information regarding the pathophysiology of PPRCA and improve our understanding of the nature of this disease.

Multimodal imaging of pigmented paravenous retinochoroidal atrophy

“…The diagnosis of PPRCA is usually based on typical fundus appearance, the findings of multimodal imaging help us to confirm the diagnosis. 4 FAF and SS-OCT findings seen in our patient were similar to the cases described in literature. FAF showed hypo-fluorescent areas correlating with the atrophy of RPE.…”

“…3 Recently multimodal imaging techniques have been used to better understand its pathogenesis. 2,4,5…”

Pigmented paravenous retinochoroidal atrophy associated with Vogt-Koyanagi-Harada disease: a case report

“…This observation has been reported only in one case [9]. This singular SD-OCT feature is of physiopathological interest; in fact, it was hypothesized that choroidal atrophy resulting in decreased choroidal perfusion and subsequent insufficient metabolic supply for the outer retinal layers preceded the photoreceptors degeneration and then the RPE loss [10]. Perivascular thickening of the RNFL may support an additional vessel-based etiology for PPRCA rather than a RPE-based mechanism only.…”

Clinic-based ultra-wide field retinal imaging in a pediatric population

“…Increased or attenuated autofluorescence patterns involved the periphery in 23 of 24 (96%) patients highlighting the usefulness of capturing the peripheral retina with UWF imaging. UWF-FAF has been described in case reports previously in Stargardt disease, pigmented paravenous chorioretinal atrophy, and long-chain 3-hydroxyacyl-CoA dehydrogenase retinal dystrophy [19–21]. The peripheral extent of lesions and autofluorescence patterns may be useful to diagnose heredodegenerative disorders and monitor disease activity, particularly in inflammatory conditions [22].…”