Peripheral exudative-hemorrhagic chorioretinopathy, formalization of forms and features of the course

Abstract

Introduction: Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a rare retinal vasculopathic disease, similar to age-related macular degeneration, primarily afecting Caucasians. This degenerative process is characterized by hemorrhage and/or exudation under the retina, leading to potential vision deterioration. Risk factors include age (66–83 years), hypertension, and use of anticoagulants. Despite its prevalence and impact, there is no established management or classifcation for PEHCR, highlighting the need for research. Objectives: Our study aimed to formalize the forms and features of PEHCR and study its morphological structure to develop a treatment strategy. Methods: We examined 13 patients with PEHCR (16 eyes) using standard ophthalmological methods. All patients had hypertension. Diagnostic tools included OCT, FA, ultrasound, and long-wave infrared fundusography. Lesions were typically found temporally, with visual acuity ranging from 0.005 to 0.7. The main causes of visual impairment were hemorrhage and exudative detachment. Results: We developed a formalization based on lesion forms and course features, considering form, activity level, macular condition, lesion length, hemorrhage severity, and treatment. The main morphological characteristics of PEHCR included subretinal hemorrhage, lipid exudation, RPE hyperplasia, and peripheral RPE atrophy. Treatment involved intravitreal afibercept injections, with or without triamcinolone acetonide, and vitrectomy. Formalization of PEHCR: Shape: dome-shaped, plateau-shaped Activity level: active form, spontaneous regression with signs of chorioretinal atrophy or fbrosis Regarding the macula: without involvement, with involvement Extent: 1 quadrant, 2 or more quadrants Mono- and bilateral lesion Presence and severity of hemorrhages: in the PEHCR zone, in the vitreous body Stage of the process: initial single lesions, developed lesions of 2 or more quadrants with signs of varying activity, severe with spread to the macula or vitreous body Conducted management: after intravitreal anti-VEGF injection, after vitrectomy Presence of AMD: present, absent. Conclusions: We established a formalization of PEHCR forms and features, providing a foundation for treatment strategy development. The morphological similarities between PEHCR and AMD suggest the potential efectiveness of anti-VEGF therapy. Further research is needed to validate the efectiveness and safety of the proposed treatments.