December 2002
Volume 43, Issue 13
Free
ARVO Annual Meeting Abstract  |   December 2002
Corneal Diameters and Ocular Biometric Characteristics of Patients with Aarskog Syndrome
Author Affiliations & Notes
  • CL McCloskey
    Ophthalmology
    Royal Liverpool Children's Hospital Liverpool United Kingdom
  • SB Kaye
    Ophthalmology
    Royal Liverpool Children's Hospital Liverpool United Kingdom
  • E Sweeney
    Cytogenetics
    Royal Liverpool Children's Hospital Liverpool United Kingdom
  • J McKenzie
    Ophthalmology
    Royal Liverpool Children's Hospital Liverpool United Kingdom
  • S Natha
    Ophthalmology
    Royal Liverpool Children's Hospital Liverpool United Kingdom
  • Footnotes
    Commercial Relationships   C.L. McCloskey, None; S.B. Kaye, None; E. Sweeney, None; J. McKenzie, None; S. Natha, None.
Investigative Ophthalmology & Visual Science December 2002, Vol.43, 1109. doi:
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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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Abstract

Abstract: : 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%).  

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.

Keywords: 369 cornea: clinical science • 363 comparative anatomy • 622 visual development 
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