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Ocular manifestations in Ehlers-Danlos syndrome

Kim SB, Shaia JK, Kaelber DC, Singh RP, Talcott KE

Eye (London, England), 2025

99 participants

Abstract

BACKGROUND/OBJECTIVE: To provide a large-scale analysis on the demographics and ocular comorbidities in Ehlers-Danlos Syndrome (EDS) patients in the US.

SUBJECTS/METHODS: This is an exploratory cross-sectional study comparing medical records of EDS patients to the general population on demographic variables and ICD-10 ocular diagnoses. A research platform with de-identified EHR data of over 99 million patients across 60 healthcare organizations was utilized. Groups were stratified by 30-year age groups. Patients aged 0-61+ with an ICD-10 diagnosis of EDS (76,526), the general platform population aged 0-61+ (99,836,639), and patients with a concurrent ICD-10 ocular diagnosis were queried to determine the prevalence of EDS across demographic variables, ocular disease, and odds of ocular disease. Statistical analysis was conducted using Microsoft Excel and R studio, using p < 0.01 and 95% confidence intervals (CI).

RESULTS: An EDS diagnosis was most prevalent in white females aged 0-30 years old (259.6 per 100,000). The majority of ocular diagnoses were more prevalent in the 0-60-year-old EDS population compared to the general population including myopia (5227.0 per 100,000) and dry eye (4211.6 per 100,000). Overall, diagnoses of angioid streaks (POR 18.72, 95% CI 10.32, 33.94) and idiopathic intracranial hypertension (IIH) (POR 18.43, 95% CI 17.51, 19.39) showed the highest increased odds in patients with EDS while significantly decreased odds were shown for type 2 diabetic…

How strong is the evidence?

Evidence Hub reads this as a cross-sectional, done in people. Its evidence level is 4 of 5 (Low · observational). Observational designs can show an association but not prove cause. Reported size: 99 participants or records.

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