December 2002
Volume 43, Issue 13
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ARVO Annual Meeting Abstract  |   December 2002
A Clinical And In Vivo Confocal Microscopical Analysis Of Anterior Membrane Dystrophy Over Three Generations In A Single Family
Author Affiliations & Notes
  • CN Grupcheva
    Discipline of Ophthalmology University of Auckland Auckland New Zealand
  • Footnotes
    Commercial Relationships   C.N. Grupcheva, None. Grant Identification: Unrestreicted grant from theMaurice and Phyllis Paykel Trust
Investigative Ophthalmology & Visual Science December 2002, Vol.43, 1725. doi:
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      CN Grupcheva; A Clinical And In Vivo Confocal Microscopical Analysis Of Anterior Membrane Dystrophy Over Three Generations In A Single Family . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1725.

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Abstract

Abstract: : Purpose: To describe and analyse, clinical and in vivo confocal corneal characteristics, of multiple members of a family, diagnosed with an anterior corneal dystrophy involving Bowman's layer. Methods: Full ophthalmic examination was performed on 8 members of a family with 3 symptomatic subjects. All cooperative subjects (n=5) were examined by in vivo confocal microscopy. Molecular genetic analysis, to identify the gene is in progress. Results: The history of a father, one son and one grand daughter revealed intermittent eye irritation and painful episodes, consistent with recurrent erosion syndrome. The best spectacle corrected visual acuity, was 6/6 in both eyes for all subjects tested. All of the adults, excluding one son, exhibited normal corneal sensitivity. Slit lamp examination, for all 8 subjects, highlighted increased reflectivity of Bowman's membrane, with well circumscribed, circular scars, 0.2 to 0.9 mm in diameter, distributed in the para-central, anterior stroma. In vivo confocal microscopy of all subjects older than 8 years (n=5), demonstrated similar features including hyper-reflective Bowman's layer(a), very densely populated subbasal stroma(b), unusual keratocytes with round dark centres(c), and very prominent nerves with unusual branching and prominent beading(d). Conclusion: We describe a family with a rare, dominant, corneal dystrophy, involving Bowman's membrane and the anterior stroma, which clinically could be one of several subtypes of anterior membrane dystrophy. However, on the basis of the in vivo confocal microstructural observations, the described dystrophy is most consistent with Grayson-Wilbrandt dystrophy.  

Keywords: 369 cornea: clinical science • 385 degenerations/dystrophies • 420 genetics 
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