Characteristics of the anterior eye segment in patients affected by ehlers‐danlos‐syndrome

Abstract

AbstractPurpose Ehlers‐Danlos Syndrome (EDS) characterizes an inherited connective tissue disorder caused by a molecular defect in the synthesis of collagen. The refractive, biometrical and densitometrical characteristics of the anterior eye segment in patients affected by various forms of EDS were analysed.Methods 46 EDS patients in an age‐range of 6 to 62 years (mean age 33 years) affected by various types of EDS, as diagnosed by genetic screening or skin biopsies, were recruited on a voluntary basis for this study. They were subjected to a complete orthoptical and clinical examination including documentation of the anterior eye segment with the EAS 1000 (Eye Analysis System, Nidek, Gamagory, Japan). Apart from clinical parameters like visual acuity, ocular motility and intraocular pressure, biometric measurements have been performed for corneal thickness and curvature, anterior chamber geometry, lens biometry and densitometric analysis of corneal and lens light scattering.Results The EDS patient population comprised 37% classical type, 28% hypermobile type and 13 % vascular type. Among the clinical parameters, prevalence of myopia combined with astigmatism was 33% that of hyperopia combined with astigmatism 28%. Strabismus was found in 11% and about 59% of the EDS patients complained about dry eyes problems. Densitometric aanalyses of the lens revealed a normal age‐related scattering profile in all patients. Similarly the biometric data for lens thickness and anterior chamber depth were within the normal range. Patients with the classical type EDS showed thinner corneas compared to the vascular or hypermobility type of EDS.Conclusion Most of the clinical features described in association with EDS like thin and translucent sclera, keratoconus, lens luxation, retinal detachment or angoid streaks have not been observed in this study. Thinner cornea and perhaps more frequent anterior vitreal detachment were the only consistent pathological findings associated with the classical type of EDS which could be demonstrated in this study.