Analysis of axial Length of Marfan Syndrome with Bilateral Ectopia Lentis by Z-scores for Chinese Patients under 20 years Old

Purpose: To analyze the axial length (AL) in young patients with Marfan syndrome (MFS) and bilateral ectopia lentis (EL) before the lens surgery. Methods: : This study reviewed MFS patients under 20 with bilateral EL from January 2015 to October 2020. The Z-scores were introduced in terms of the number of standard deviations (SD) from the mean of age-matched normative data. Using Z-scores, the distribution of AL and influence factors were evaluated. The correlations between AL and other biometrics were analyzed. Results: : We reviewed 183 patients, and enrolled both eyes. The mean age was 8.44 ± 4.69 years old. The mean AL increased from 23.41 ± 1.73 mm under 5 years old to 26.40 ± 2.28 mm in the 16-20 age group, and when plotted, the trend presented a logarithmic curvature. The mean Z-AL score was 1.74 ± 2.68. One-third of eyes had Z-score < 0. The right eye and the eye complicated with megalocornea had larger Z-AL scores. A positive correlation was found between Z-AL and Z-Corneal Curvature Radius (r = 0.265, P < 0.001). Conclusion: Young patents with bilateral EL but small AL should not be excluded from MFS without systematic examination. The age-adjusted Z-score will facilitate further study of the individual variations in AL across different ages.