Peripheral exudative hemorrhagic chorioretinopathy as a variant of polypoidal choroidal vasculopathy in a case series of Chinese patients

Abstract:

AIM: To evaluate the clinical features, diagnosis, treatment, and outcome of peripheral exudative hemorrhagic chorioretinopathy (PEHCR), a variant of polypoidal choroidal vasculopathy (PCV), in a case series of Chinese patients. METHODS: This study was retrospectively conducted from September 2018 to March 2025. Clinical examinations included color fundus photography, B-scan ultrasonography, fluorescein angiography (FA), indocyanine green angiography (ICGA), swept-source optical coherence tomography (SS-OCT), and optical coherence tomography angiography (OCTA), and two active or inactive subgroups and misdiagnosed cases were analyzed. RESULTS: Totally 19 patients (21 eyes) with a mean age of 54.3±9.4 (range, 36–68)y were included, with a majority of women (n=13, 68.4%). The mean follow-up period was 13±1.4 (range: 1–57)mo. Decreased visual acuity was the most frequent initial manifestation (17 eyes, 84.2%), and lesions were mainly distributed in the inferotemporal or temporal quadrant (14 eyes, 66.7%), with choroidal polyps and branching neovascular networks revealed by OCTA and ICGA. Nine patients had been previously misdiagnosed with choroidal melanoma, and 6 of them had massive vitreous hemorrhage (VH). PEHCR manifested along a spectrum ranging from active or inactive subretinal hemorrhagic forms to chronic fibrotic or atrophic forms. One patient experienced natural regression. Ten eyes received a mean of 4.7±1.1 (range: 3–7) intravitreal anti-vascular endothelial growth factor (VEGF) injections, two eyes underwent vitrectomy, and six eyes were treated with vitrectomy combined with anti-VEGF therapy. Best-corrected visual acuity (logMAR) in treated eyes (18 eyes) improved to 0.31±0.25 from the baseline of 1.50 ± 0.75 (P<0.001). CONCLUSION: PEHCR is a variant of PCV. Chinese patients with PEHCR have a relatively younger age of onset. Anti-VEGF injections and/or vitrectomy are treatment options for lesion regression or dense VH to gain better visual outcomes.