Biometric and corneal characteristics in marfan syndrome with ectopia lentis

Abstract

Purpose: To describe the biometric and corneal characteristics of patients with Marfan Syndrome (MFS) and ectopia lentis.

Study design: Observational, descriptive, prospective study. Subjects Individuals with MFS with ectopia lentis (EL).

Methods: Fourty-four eyes of 23 patients underwent Scheimpflug analysis using the Pentacam (Oculus, Wetzlar, Germany), axial length (AL) using the IOL master 700 (Carl Zeiss AG, Oberkochen, Germany), endothelial cell count (ECC) using the CEM-350 (NIDEK, Maihama, Japan) and corneal biomechanics evaluation with the Ocular Response Analyzer: ORA (Reichert Ophthalmic Instruments, Buffalo, New York, USA) and Corvis (Oculus, Wetzlar, Germany). Statistical analysis was performed using IBM SPSS Statistics 25.0.

Results: The direction of lens subluxation was most frequently supero-nasal 40.9% (18/44). Mean keratometry (Km) was 40.22±1.76 Diopters (D); mean corneal astigmatism was 1.68±0.83 D; total corneal aberrometric root mean square (RMS) was 2.237±0.795μm; higher-order aberrations (HOAs) RMS were 0.576±0.272μm; mean AL was 25.63±3.65mm; mean ECC was 3315±459cell/mm²; mean CBI was 0.13±0.24, mean TBI was 0.31±0.25, mean posterior elevation was 4.3±4.5μm; mean total corneal densitometry was 16.0±2.14 grayscale units (GSU).

Conclusion: Increased axial length, flatter and thicker corneas with higher regular astigmatism, normal densitometry, normal corneal biomechanical indices and normal posterior elevation were observed in Marfan patients with EL.

Keywords: Axial length; Biomécanique cornéenne; Cornea; Corneal biomechanics; Cornée; Longueur axiale; Marfan syndrome; Syndrome de Marfan.