Frequency and Distribution of Ophthalmic Surgical Procedures among Patients with Inherited Retinal Diseases

Abstract

Objective or PurposeIn this study, we aimed to characterize the frequency and distribution of ocular surgeries in patients with inherited retinal diseases (IRDs) and evaluate associated patient and disease factors. DesignRetrospective cohort. ParticipantsSubjects 18 years and older who were followed at the Johns Hopkins Genetic Eye Disease (GEDi) Center. MethodsWe studied a retrospective cohort of patients with an IRD diagnosis to analyze the occurrence of laser and incisional surgeries. Subjects were categorized into two groups: central dysfunction (macular/cone/cone-rod dystrophy, "MCCRD group") and panretinal or peripheral dysfunction (retinitis pigmentosa-like, "RP group"). Genetic testing status was recorded. The association of patient and disease factors on the frequency, distribution, and timing of surgeries was analyzed. Main outcome MeasurePrevalence, prevalence odds ratio (POR), hazard ratio (HR) of ophthalmic procedures by phenotype. ResultsA total of 1472 eyes of 736 subjects were evaluated. Among them, 31.3% (n = 230) had undergone ocular surgery, and 78.3% of those (n=180/230) had a history of more than one surgery. A total of 602 surgical procedures were analyzed. Cataract extraction with intraocular lens implantation (CEIOL) was the most common (51.2%), followed by YAG capsulotomy, refractive surgery, retinal surgery, and others. CEIOL occurred more frequently in RP than in MCCRD subjects (POR 2.59, p = 0.002). RP subjects underwent CEIOL at a younger age than MCCRD patients (HR = 2.11, p < 0.001). ConclusionApproximately one-third of IRD patients had a history of laser or incisional surgery. CEIOL was the most common surgery; its frequency and timing may be associated with IRD phenotype. This data may inform the design of prospective research. Such efforts may illuminate routine clinical decision-making and contribute to surgical strategy development for cell and gene therapy delivery.