Ocular Motility Abnormalities in Ehlers-Danlos Syndrome: An Observational Study

Abstract

Purpose: To evaluate ocular motility (OM) abnormalities associated with Ehlers-Danlos Syndrome (EDS). Materials and methods: In this cross-sectional observational study, patients with EDS underwent a complete orthoptic examination. The following orthoptic tests were performed: corneal light reflex test, stereoscopic test, cover test, OM assessment, evaluation of eye pain in different gaze positions and red filter test for diplopia. Results: The corneal light reflex test at 33 cm showed an intermittent divergent deviation in 31.7% of patients and an intermittent horizontal deviation associated with a vertical deviation in 4.9% of patients. A manifest strabismus was observed in 2.4% of patients, whereas 2.4% of patients showed a microstrabismus. The corneal light reflex test at 5 m revealed microstrabismus in 9.8% and manifest strabismus in 2.4% of our patients. Moreover, intermittent exotropia was observed in 2.4% of cases. No significant alterations involving the inferior rectus and the superior oblique muscles were observed. Significant associations were observed between medial rectus muscle deficit of both eyes with pain (p = 0.020) and diplopia (p = 0.014). Furthermore, a significant association between lateral rectus muscle alteration of both eyes and pain was observed (p = 0.004). Conclusions: Our results show various OM alterations in patients with EDS, specifically superior and medial rectus muscle hypofunction. A full orthoptic evaluation in these patients is recommendable to detect OM involvement and possible ligamentous laxity changes over time through an accurate OM assessment.

Keywords

• Ehlers-Danlos Syndrome
• collagen alteration
• ocular motility
• strabismus