A descriptive study of ocular characteristics in Marfan syndrome

Abstract

Purpose: To study the ocular characteristics in patients with Marfan syndrome (MFS).

Methods: One hundred and two eyes of 51 participants with MFS were included in this descriptive study. Ocular characteristics, visual acuity (VA) and biometric values were studied.

Results: The mean patient age was 39.0 years (range, 12.7-71.6). Seventy-seven eyes were phakic, five aphakic and 20 pseudophakic. Thirty-one eyes had ectopia lentis (EL), 12 eyes cataract, nine eyes strabismus, five eyes glaucoma and four eyes had undergone surgery for a retinal detachment (RD). Ninety-four eyes (92%) had a best spectacle-corrected VA of 0.3 logarithm of the minimum angle of resolution (logMAR) or better. In the 77 phakic eyes, myopia exceeding -3 D was seen in 12/31 eyes (39%) with EL, and in 12/46 eyes (26%) without EL. The mean axial length (AL) was 24.73 mm (range, 21.59-32.75); 39 eyes (51%) had abnormally increased AL (≥24.5 mm). The mean Kmed (mean value of Kmin and Kmax ) was 42.2 D (38.7-46.9 D). No significant difference was found in refraction, AL or corneal curvature between eyes with and without EL. Lens-related astigmatism was seen in 15 (48%) eyes with EL versus four (12%) eyes without EL. Seventeen of the pseudophakic eyes underwent surgery for EL, with history of postoperative RD in one eye.

Conclusion: Despite increased AL, high myopia is not as common in MFS as expected because of a flat cornea. Biometry should be considered in suspected cases of MFS when EL is not present.

Keywords: Marfan syndrome; aphakia; axial length; cataract; ectopia lentis; flattened cornea; myopia; pseudophakia; retinal detachment.