The Clinical Spectrum and a New Theory of Pathogenesis of True Exfoliation Syndrome

Abstract

© 2016 American Academy of Ophthalmology Purpose To describe the clinical spectrum and a new theory of pathogenesis of true exfoliation syndrome. Design Cross-sectional and prospective, observational case series. Participants Consecutive patients with characteristic peeling of the anterior lens capsule. Methods After maximal mydriasis, slit-lamp biomicroscopy, and photography, imaging of the anterior capsule and zonules was performed. The condition was classified into 4 clinical stages: annular anterior capsule thickening with a distinct splitting margin (stage 1), an inward detached crescentic flap lying on the anterior lens (stage 2), a floating and folding translucent membrane behind the iris (stage 3), and a broad membrane within the pupil (stage 4). Serial photography was performed at each 3-month follow-up visit. Ultrastructural examination of dislocated lenses and excised anterior capsules was performed. Main Outcome Measures Detached membrane morphologic features, zonular defects, pigment deposition, glaucoma, phacodonesis, and cataract. Results We enrolled 259 patients (424 eyes). Ages ranged from 52 to 97 years (mean age, 75.2±7.1 years). Eleven patients were associated with trauma (n = 1) or intense heat (n = 10), whereas 248 were idiopathic. Two hundred ten patients were followed up every 3 months, with a mean follow-up of 9.6±6.1 months (range, 3–50 months). The detachment started along the anterior zonular insertions in association with zonular disruption. It progressed centrally to higher stages, manifesting a spectrum of disease. Several stages coexisted in a single eye. At the final visit, including 49 patients who were examined once, there were 70, 87, 85, and 17 patients in stages 1, 2, 3, and 4, respectively. All stages shared common histologic findings consisting of diffuse capsular lamellar separation and anterior zonular disruption. All developed cataract. Pigment deposition on the membrane was present in 178 patients (68.7%). Twenty-six patients (10%) had spontaneous phacodonesis. Eighteen eyes (4.2%) demonstrated secondary delamination. Conclusions Capsular lamellar separation and anterior zonular disruption are characteristic findings. Aging, heat exposure, and trauma are risk factors. Initial capsular splits occur along the insertions of disrupted anterior zonules. The peeling progresses centrally in association with iris movement and aqueous flow. A second detachment can occur.