ABSTRACT
PURPOSE: The aim of this study was to analyze corneal topography relative to astigmatism, higher order aberrations, and corneal curvatures in Terrien marginal degeneration using 3-dimensional anterior-segment optical coherence tomography. METHODS: Twenty-nine eyes of 15 Finnish patients from a tertiary referral center had topographic axial power maps classified into 4 patterns by visual grading: crab claw (CC), mixed (M), arcuate (A), and normal. Regular astigmatism, keratometry, higher order aberrations, maximal corneal thinning, apex thickness, and curvature changes relative to best fit sphere toward maximal peripheral thinning were compared. RESULTS: Four, 9, and 12 eyes were classified as CC, M, and A, respectively; 1 as normal with clinical disease; and 3 as normal with unilateral disease. Median follow-up was 2.3 (range, 0-7.2) years. Three eyes changed pattern. Patients with the CC pattern were the youngest when diagnosed, progressed more rapidly, exhibited cavities in superior quadrants with anterior bulging, and had greater higher order posterior aberrations. Patients with the M pattern were older, progressed slower, and showed superonasal asymmetric corneal steepening extending centrally, often with asymmetric bow tie. Patients with pattern A showed little progression and were the oldest when diagnosed, with maximal corneal thinning equally in all quadrants. According to the Wang classification, the median stage was 4, 2, and 2 in CC, M, and A patterns, respectively, whereas it was always 2 by the Süveges classification. CONCLUSIONS: Terrien marginal degeneration is characterized by distinct corneal topographic patterns that differ in tomographic features, suggesting existence of subtypes in addition to different stages of disease. Patients representing CC and M patterns might benefit from more frequent monitoring.