EyeWorld Today is the official daily of the ASCRS Symposium & Congress. Each issue provides comprehensive coverage editorial coverage of meeting presentations, events, and breaking news
Issue link: https://daily.eyeworld.org/i/684900
10 EW SHOW DAILY Wednesday, May 11, 2016 friendly, easily accessible, and easily updated. It was last updated in 2015. The 2008 IC3D, she said, was adopted by the AAO Basic and Clin- ical Science Course Series and was referenced by the National Eye Insti- tute. It was made easily available at www.corneasociety.org in English, German, and Spanish. A category system, suggested by the late Gordon Klintworth, MD, was also introduced into the nomen- clature: Category 1 for well-defined clinical entities with known genes and loci; 2 for well-defined entities with unknown genes but known loci; 3 for well-defined entities with neither genes nor loci known; 4 for poorly defined clinical entities, with neither genes nor loci known. As more is learned about a dys- trophy, it moves up the category list, Dr. Weiss said. On the other hand, if a clinical entity never progresses from category 4 status, it may not actually exist and can be elimi- nated. Recently, she said, Grayson Wilbrandt corneal dystrophy was removed, having been based on a single drawing (no photos) and description published in the Ameri- can Journal of Ophthalmology in 1966, with no subsequent reports. The 2015 IC3D can be accessed through PubMed. On the PubMed article page, click through to Wolters Kluwer, and access the full paper through the Lippincott, Williams and Wilkins (LWW) link. EW Editors' note: Dr. Weiss has no finan- cial interests related to her comments. The name of the rose by Chiles Aedam R. Samaniego EyeWorld Asia-Pacific Senior Staff Writer W illiam Shakespeare was a genius, but if he'd met Jayne Weiss, MD, New Orleans, she might well have taken him down a peg. "I beg to differ with William Shakespeare who said a rose by any other name would still smell as sweet," she said. "When it comes to nomenclature it's partic- ularly important in medicine and ophthalmology. It allows us to de- cide prognosis as well as treatment." Dr. Weiss is the first author of the International Committee for Classification of Corneal Dystro- phies (IC3D), a living document. Published in 2008 and updated in 2015, Dr. Weiss first conceived of IC3D in 2005 to update the 19th century nomenclature of Attendees listen as Dr. Weiss presents "Update on the Classification of Corneal Dystrophies." Download the Exhibitor Directory with the ASCRS•ASOA 365 APP ascrs.org/gettheapp corneal dystrophies using 21st century information, integrating up- to-date information on phenotypic description, pathologic examina- tion, and genetic analysis. When a rose is not a rose The original definition of corneal dystrophies was essentially a list of characteristics, described on pheno- type. Phenotype, at the center of the definition, stood like a solitary can- dle, just going with the empirical, somewhat subjective observations, made without even the benefit of a slit lamp, as the sole basis for defin- ing an ostensibly complex family of disorders. Corneal dystrophies, the orig- inal definition went, are bilateral, hereditary, progressive conditions with no systemic associations. Each element of this set of characteristics, Dr. Weiss said, was a deeply flawed description of so-called dystrophies, with each characteristic having a number of exceptions: posterior polymorphous corneal dystrophy, for instance, could be unilateral; epithelial base- ment membrane corneal dystrophy is not hereditary, but most probably a degenerative condition; central cloudy dystrophy of François, punctiform and polychromatic pre-Descemet corneal dystrophy, fleck corneal dystrophy, and others are non-progressive; Schnyder (crys- talline) corneal dystrophy is associ- ated with hypercholesterolemia and genu valgum. The poor definition, she said, was compounded by inaccurate nomenclature—Schnyder crystalline corneal dystrophy, for instance, was not consistently characterized by crystals. In fact, there might not even be such a thing as a corneal dystrophy, Dr. Weiss thought, but rather a het- erogeneous set of corneal diseases. (Re)naming the rose In 2005, Dr. Weiss recruited interna- tional dystrophy specialists—clini- cians, geneticists, and pathologists— to begin work on IC3D. IC3D was intended to reflect current clinical, histopathological, and genetic in- formation, removing inaccuracies in the descriptions and nomenclature. In addition, IC3D, published online, was intended to be user