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CL McCloskey, SB Kaye, E Sweeney, J McKenzie, S Natha; Corneal Diameters and Ocular Biometric Characteristics of Patients with Aarskog Syndrome . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1109.
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Purpose: To report the ocular biometric characteristics of patients with Aarskog syndrome, and to determine whether enlargement of the cornea in Aarskog syndrome represents megalocornea or an enlarged globe. Methods: The ophthalmic and systemic features of a family with Aarskog syndrome were studied. The horizontal corneal diameters (HCD), corneal radii, anterior chamber depth (ACD), cupula, posterior limbal depth (PLD), axial length, vitreous length and vitreous index were measured from the ocular biometric data. Results: Three of the affected children (III: 1,2 and 3) and affected grandparent (I:1), had mild features of an anterior segment dysgenesis, predominantly in the form of extensive posterior embryotoxon. Two of the affected children (III:1 and III:3) aged 8yrs and 9yrs had enlarged corneas (12mm and 12.5mm) which were significantly larger than their parents (11mm) and affected grandfather (I:1) (10.5 mm). They showed increased corneal radii of curvature (8.00mm to 8.22mm), but average PLD (0.4mm), cupula (2.7mm to 2.9mm), vitreous length (16.2mm to 16.8mm) and vitreous indices (70% to 71%). View OriginalDownload SlideView OriginalDownload Slide Conclusion: In all affected individuals, the ACD, cupula, PLD, vitreous lengths and vitreous indices were similar to that expected in a normal population as was the vitreous lengths and vitreous index. In the 2 children with enlarged corneas, there was an expected increase in the anterior radius of curvature. All of these features differ from those found in association with megalocornea and are more consistent with an overall ocular enlargement.
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