A descriptive study of ocular characteristics in Marfan syndrome

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

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.

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.

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.