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Confocal Microscopy in Meretoja Syndrome

Rothstein, Anna B.A.; Auran, James D. M.D.; Wittpenn, John R. M.D.; Koester, Charles J. Ph.D.; Florakis, George J. M.D.

Clinical Sciences

Purpose. To study the correlation between severity of clinical systemic symptoms and the degree of stromal haze and visibility of epithelial and stromal nerves on scanning slit confocal microscopy examination in Meretoja syndrome.

Methods. Three patients with Meretoja syndrome were examined by slit-lamp microscopy and scanning slit confocal microscopy.

Results. Increased symptoms and abnormal slit-lamp findings correlated positively with confocal microscopic stromal haze intensity and inversely with visibility of epithelial and stromal nerves on confocal examination. A prominent deposit, presumably amyloid, was observed contiguous to a stromal nerve of an affected cornea. In a severely affected cornea, no stromal or epithelial nerves were seen.

Conclusion. The results of this study suggest that Meretoja syndrome causes corneal nerve damage and eventual degeneration that correlates with the degree of clinical involvement.

From Robert Wood Johnson Medical School (A.R.), University of Medicine & Dentistry of New Jersey, Piscataway, New Jersey; Department of Ophthalmology (J.D.A., C.J.K., G.J.F), Edward S. Harkness Eye Institute, Columbia University, New York, New York; and Department of Ophthalmology (J.R.W.), State University of New York at Stony Brook, Stony Brook, New York.

This study was performed at the Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University, New York, New York and the Department of Ophthalmology, State University of New York at Stony Brook, Stony Brook, New York. Confocal microscopy was carried out with approval from the Institutional Review Board of Columbia-Presbyterian Medical Center and informed consent was obtained from the three patients who participated in the study. Presented in part at the annual meeting of the American Academy of Ophthalmology, San Francisco, California, November 1994. Dr. Charles Koester is a consultant in the development of the scanning slit confocal microscope.

Submitted September 24, 2001.

Revision received February 1, 2002.

Accepted February 4, 2002.

Supported in part by a grant by the Vidda Foundation.

Address correspondence and reprint requests to Dr. G.J. Florakis Columbia-Presbyterian Medical Center, 635 West 165th Street, Suite 303, New York, NY 10032, U.S.A. E-mail: Gjf3@yahoo.com

© 2002 Lippincott Williams & Wilkins, Inc.