Ophthalmic Implications of Ehlers-Danlos Syndrome

Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders with systemic manifestations, but its ophthalmic implications are poorly characterized. Existing studies suggest an increased prevalence of many ophthalmic conditions as well as a higher rate of surgical complications, but a comprehensive understanding is lacking.

An anonymous survey was distributed online through social media and patient advocacy organizations. Participants reported genetic and ophthalmic diagnoses, year of diagnosis, refractive error, and ophthalmic surgical history. Analyses focused on individuals with hypermobile, vascular, and classical EDS.

A total of 766 completed surveys were analyzed. Most respondents (83.3%) had hypermobile EDS. Dry eye, strabismus, amblyopia, blue sclera, and several other eye conditions were common and more prevalent compared to population estimates. Myopia was the most prevalent condition, with high myopia reported by approximately 40% of respondents, exceeding population estimates of 20-30%. Several conditions, including glaucoma, posterior vitreous detachment, and retinal tear/detachment, presented at younger ages than expected. Ophthalmic surgeries were overall well tolerated, although persistent postoperative pain was frequently reported.

EDS may be associated with an increased risk and earlier onset of many ophthalmic conditions. Several of these conditions, including glaucoma and retinal tear/detachment, could lead to significant morbidity if not detected and managed early. Ophthalmic procedures are well tolerated, but persistent pain is common. Enhanced screening and tailored surgical management could improve quality of life in individuals with EDS.