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

Lukas Mees; Mingyi Li; Bani Antonio-Aguirre; Tin Yan Alvin Liu; Adela Wu; Xiangrong Kong; Mandeep S Singh

doi:10.1016/j.oret.2024.03.005

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

Ophthalmol Retina. 2024 Mar 12:S2468-6530(24)00109-X. doi: 10.1016/j.oret.2024.03.005. Online ahead of print.

Authors

Lukas Mees¹, Mingyi Li², Bani Antonio-Aguirre¹, Tin Yan Alvin Liu¹, Adela Wu³, Xiangrong Kong⁴, Mandeep S Singh⁵

Affiliations

¹ Retina Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland.
² Department of Epidemiology and Population Health, School of Medicine, Stanford University, Stanford, California; Department of Biostatistics, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland.
³ Retina Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland; Department of Neurosurgery, Stanford Health Care, Stanford, California.
⁴ Retina Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland; Department of Biostatistics, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, Maryland.
⁵ Retina Division, Wilmer Eye Institute, Johns Hopkins University, Baltimore, Maryland; Department of Genetic Medicine, Johns Hopkins University, Baltimore, Maryland. Electronic address: singhcorrespauth@gmail.com.

PMID: 38485090
DOI: 10.1016/j.oret.2024.03.005

Abstract

Objective: In 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.

Design: Retrospective cohort.

Participants: Subjects aged ≥ 18 years who were followed at the Johns Hopkins Genetic Eye Disease Center.

Methods: We studied a retrospective cohort of patients with an IRD diagnosis to analyze the occurrence of laser and incisional surgeries. Subjects were categorized into 2 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 measures: Prevalence, prevalence odds ratio (POR), hazard ratio (HR) of ophthalmic procedures by phenotype.

Results: A 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 1 surgery. A total of 602 surgical procedures were analyzed. Cataract extraction with intraocular lens implantation (CEIOL) was the most common (51.2%), followed by yttrium aluminum garnet capsulotomy, refractive surgery, retinal surgery, and others. Cataract extraction with intraocular lens implantation occurred more frequently in RP than in MCCRD subjects (POR, 2.59; P = 0.002). Retinitis pigmentosa subjects underwent CEIOL at a younger age than patients with MCCRD (HR, 2.11; P < 0.001).

Conclusions: Approximately one-third of patients with IRD had a history of laser or incisional surgery. Cataract extraction with intraocular lens implantation was the most common surgery; its frequency and timing may be associated with the 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.

Financial disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Keywords: Cataract extraction; Retinal dystrophies; Vitrectomy; cell therapy; gene therapy.