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S.O. Semb, S. Rand–Hendriksen; Ocular Features in Adult With Verified Marfan Syndrome: Preliminary Data From the Norwegian Marfan Syndrome Study . Invest. Ophthalmol. Vis. Sci. 2006;47(13):1163.
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© ARVO (1962-2015); The Authors (2016-present)
Marfan syndrome is an autosomal dominant disorder of connective tissue, often affecting the eyes. In the present study we have investigated the prevalence of lens dislocation, flattened cornea, increased axial length and megalocornea in patients with verified Marfan syndrome based on the revised criteria for Marfan syndrome (Gent criteria).
The study was a prospective population based study recruited from the Norwegian population. 87 adults given the diagnosis of Marfan syndrome were investigated for all features of the Gent criteria. We report the ocular results from the patients (173 eyes) fulfilling the Gent criteria. Investigations: Slitlamp biomicroscopy, keratometry measurements (Humphrey), corneal topography by Orbscan II (Bausch & Lomb), and ultrasound pachymetry and axial length (Tomey).
Ectopia lentis was observed in 53 (61%) of the patients. 22 patients (25%) presented subluxated lenses (dislocation slightly backwards) and in 31 patients (36%) the lens was luxated (most commonly superotemporal). The luxation was bilateral and symmetrical in nearly all patients. The axial length was >23,5 mm (minor criterion) in 113 (68%) eyes (mean ± SD, 25,07 ± 2,83 mm). The mean keratometric power was 41,3 ± 4,9 D. There was a significant correlation between axial length and corneal radius (P < 0,0001). The distribution of axial length/mean corneal radius was peaked (kurtosis 5,1) with a mean of 3,09. The corneal horizontal diameter (white–to–white) measured with Orbscan was 11,70 ± 0,60 mm which is in accordance with the diameter found in healthy white persons. The central corneal pachymetry was 511 ± 53 (Orbscan) and 544 ± 45 (ultrasound) respectively. Only 3 patients showed signs of hypoplastic iris.
In this strictly defined Marfan syndrome group fulfilling the Gent criteria, the prevalence of ectopia lentis was 61%. We found flatter corneas with increased axial lengths.
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