Survey of Ehlers‒Danlos Patients’ ophthalmic surgery experiences

Abstract

Background: Ehlers-Danlos Syndrome (EDS) is a rare disease affecting approximately 1 in 5,000 people. Although ophthalmic conditions associated with EDS have been described, little data exist concerning ophthalmic surgical outcomes experienced by EDS patients. Methods: Patients with EDS were surveyed via the EDS Society and asked about their ophthalmic surgical experiences including procedure, complications, and the timing with respect to receiving the EDS diagnosis. Complications were confirmed as such by subspecialists. Results: Of 579 respondents, 467 reported confirmed EDS, and 112 of those had an ophthalmic procedure, including refractive surgery, cataract/lens surgery, retinal surgery, strabismus surgery, oculoplastic surgery, corneal surgery, and laser surgery for glaucoma. The rate of confirmed complications was: 23%-refractive, 33%-lens/ cataract, 33%-retina, 59%-strabismus, 23%-oculoplastics, 0%-cornea, and 25%-glaucoma laser. In addition, 76% of patients underwent surgery prior to the EDS diagnosis. Conclusions: Patients with EDS may have elevated risk of postoperative ophthalmic surgical complications. It would seem reasonable to systemically and prospectively explore how patients with EDS respond to ophthalmic surgery. Furthermore, it would seem circumspect to ask surgical candidates patients about whether they carry a diagnosis of EDS or have signs and symptoms of EDS prior to surgery.

Outcomes and Complications in Patients With Hypermobile Ehlers–Danlos Syndrome Who Have Undergone Laser In Situ Keratomileusis Surgery

“…17 Two patients with EDS self-reported corneal ectasia after LASIK in a survey study, but this is difficult to confirm without Scheimpflug imaging. 16 The only other case report of a patient with EDS who had LASIK surgery occurred in a patient with vascular EDS, and he developed myopic regression but not kerectasia. 18 In our case series of 12 patients with hypermobile EDS who underwent LASIK, 1 patient developed bilateral corneal ectasia, showing that patients with EDS still may be at higher risk of developing this complication.…”

“…Invitations to participate in our study were sent to participants of the aforementioned EDS survey, 16 members of the EDS Society, and patients of Dr. Singman who had LASIK surgery and EDS. Patients were seen for a full dilated eye examination by one of the study authors (Gauthier).…”

Controversy and Consideration of Refractive Surgery in Patients with Heritable Disorders of Connective Tissue

“…A literature search revealed only one study surveying patients with EDS about their ophthalmic surgical experiences, showing 43% of patients had undergone radial keratotomy, PRK, LASIK, or LASEK. Of those, 23.3% reported complications, including under correction/regression (18.6%), postoperative pain (9.3%), impaired night vision (7%), dry eye (4.7%), induced astigmatism (7%) and corneal ectasia (4.7%) [19]. The survey demonstrates that refractive surgery on an EDS patient does not make any complication a foregone conclusion.…”

Ehlers-Danlos syndromes and their manifestations in the visual system

“…Further progression of lenticular opacification can be visually symptomatic from decreased visual acuity. It must be mentioned that hEDS may be at higher risk for complications of ophthalmic surgery, adding to the complexity of treating these patients ( 52 ).…”