May 2004
Volume 45, Issue 13
Free
ARVO Annual Meeting Abstract  |   May 2004
Clinical Characteristics of 22 Families With Schnyder's Crystalline Corneal Dystrophy
J.S. Weiss; S.B. Muenk; V. Theendakara; G. Tromp; T. Tervo; P. Riebling; M. Hoeltzenbein; W. Henn; H. Kuivaniemi
Author Affiliations & Notes
  • J.S. Weiss
    Ophthalmology, Kresge Eye Institute, Wayne St University, Detroit, MI
  • S.B. Muenk
    Ophthalmology, Kresge Eye Inst, Detroit, MI
  • V. Theendakara
    Center for Molecular Medicine and Genetics, Wayne State Univ, Detroit, MI
  • G. Tromp
    Center for Molecular Medicine and Genetics, Wayne State Univ, Detroit, MI
  • T. Tervo
    Helsinki University Eye Hospital, Helsinki, MI
  • P. Riebling
    Augenklinik der Universitat Greifswald, Griefswald, MI
  • M. Hoeltzenbein
    Max–Planck–Institut fur Molekulare Genetik, Berlin, MI
  • W. Henn
    Human Genetics and Ophthalmology, Saarland University, Homberg, MI
  • H. Kuivaniemi
    Center for Molecular Medicine and Genetics, Department of Surgery, Wayne St University, Detroit, MI
  • Footnotes
    Commercial Relationships  J.S. Weiss, Alcon R; S.B. Muenk, None; V. Theendakara, None; G. Tromp, None; T. Tervo, None; P. Riebling, None; M. Hoeltzenbein, None; W. Henn, None; H. Kuivaniemi, None.
  • Footnotes
    Support  NIH Grant EY12972–01
Investigative Ophthalmology & Visual Science May 2004, Vol.45, 1519. doi:
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      J.S. Weiss, S.B. Muenk, V. Theendakara, G. Tromp, T. Tervo, P. Riebling, M. Hoeltzenbein, W. Henn, H. Kuivaniemi; Clinical Characteristics of 22 Families With Schnyder's Crystalline Corneal Dystrophy . Invest. Ophthalmol. Vis. Sci. 2004;45(13):1519.

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Abstract

Abstract: : Purpose: Although Schnyder's Crystalline Corneal Dystrophy causes progressive corneal opacification from abnormal deposition of lipid, it has traditionally been thought to cause minimal visual impairment and usually not require penetrating keratoplasty (PKP). We wanted to objectively document the visual impairment and ophthalmologic findings in the largest number of patients with SCCD that has been reported to date. Methods: Retrospective chart review of 184 members of 22 families with SCCD was performed.. Results of history, genetic studies and ocular exams including best corrected visual acuity and slit–lamp examination.were recorded. Results: 72 patients had SCCD documented by genetic testing. Of the 65 affected patients who had ocular exams, all were documented to have the corneal findings of SCCD. All patients less than 23 years of age had central corneal haze and /or anterior stromal crystals.with average BCVA of 20/25., 75% of patients betweeen 24 to 38 years of age also had arcus lipoides with average BCVA of 20/40. 100% of patients greater than 39 years of age had mid peripheral corneal haze with average BCVA of 20/60. Only 55% of patients had evidence of anterior stromal crystalline deposition. 15 eyes of 10 patients previously had PKP with an average age of 59 at the time of surgery. Conclusions: This is the largest collection of patients with SCCD ever reported. As previously reported (Weiss (1992) Schnyder's dystrophy of the cornea. A Swede–Finn connection. Cornea 11:93–101) , the corneal findings of SCCD can be predicted based on patient age and causes progressive decrease in vision. In this study, 15% of patients examined required PKP. Despite the name Schnyder's crystalline corneal dystrophy, only 55% of patient with SCCD have crystalline deposits. The misconception that crystalline deposition is required to diagnose SCCD should be corrected.

Keywords: cornea: clinical science • cornea: storage • lipids 
© 2004, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. Permission to republish any abstract or part of an abstract in any form must be obtained in writing from the ARVO Office prior to publication.
Copyright © 2024 Association for Research in Vision and Ophthalmology.
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