High-order ocular aberrations and Pupillometry profile in patients with Marfan disease

Abstract

Purpose: The aim of our study was to assess whether lens dislocation is associated with specific high-order ocular optical aberrations and to investigate the pupillary light reflex (PLR) in patients with Marfan disease (MD).

Methods: Thirty-two non-operated eyes of 16 patients with MD (Sex ratio 5/11, mean age 38) and 32 eyes of 16 age-matched healthy subjects were included in this study. High-order ocular optical aberrations were measured in MD patients with the topograph-aberrometer KR-1W (Topcon). Coma, spherical aberrations, trefoil, tetrafoil and Root Mean Square (RMS) were reported on 4 mm and 6 mm pupils. PLR was measured by the “Neurolight” pupillometer in mesopic condition and the data analyzed were: Baseline Pupil Diameter (BPD) in mm, Maximal Pupillary Constriction (MPC) after light stimulation in mm, Speed of pupillary constriction (SPC) in mm/s and latency (L) in ms.

Results: In the MD group, mean best-corrected visual acuity was 0.98±0.06 (decimal scale). Mean keratometry K1 and K2 were 41.7±1.7D and 42.4±1.7D respectively. Mean sphere was -1.1±5.8D and mean cylinder was 0.9±0.7D. 18 eyes had a clinically suspected lens dislocation. Internal aberrations (total minus corneal) were higher in patients with lens dislocation compared to those without lens dislocation. The difference was found to be statistically significant for the following aberrations: coma-4mm (mean 0.27vs.0.07, p=0.03), spherical-4mm (mean 0.08vs.0.01, p=0.02), trefoil-4mm (mean 0.20vs.0.06, p=0.02), and RMS-4mm (mean 0.37vs.0.12, p=0.02). Total aberrations were also higher in patients with lens dislocation. However, a statistically significant threshold was only reached for spherical total aberrations-4 mm (p=0.042). There was no significant difference in PLR between MD patients and the controls (BPD: 4.73±0.97 vs 4.99±1.04; MPC: -2.09±0.65 vs -2.25±0.67; SPC: 5.09±2.13 vs 5.66±2.02; L: 216± 55 vs 213± 48 respectively).

Conclusions: Hypoplastic iris is a minor criterion of MD, but automated PLR analysis failed to found a significant difference between MD patients and controls in our study. Despite comparable and high visual acuity in both sub-groups of MD patients, we found significantly higher optical aberrations in the group of patients with lens dislocation. Aberrometry might help clinicians diagnose mild ectopia lentis wich is a major criterion in MD.

Keywords: 626 aberrations • 667 pupil • 550 imaging/image analysis: clinical