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Chapter-058 Stromal Corneal Dystrophies

BOOK TITLE: Copeland and Afshari's Principles and Practice of Cornea (2 Volumes)

Author
1. Weiss Jayne S
2. Mequio Michael
3. Coleman Michael
ISBN
9789350901724
DOI
10.5005/jp/books/11777_58
Edition
1/e
Publishing Year
2013
Pages
23
Author Affiliations
1. LSU; LSU Eye Center, LSUHSC, New Orleans, Louisiana, USA, LSU Eye Center, LSUHSC, New Orleans, Louisiana, USA
2. Ophthalmology United States Air Force, MacDill AFB, Tampa, Florida, USA
3. Kresge Eye Institute/Detroit Medical Center, Detroit, Michigan, USA
Chapter keywords
Stromal corneal dystrophies, TGFBI gene, keratoepithelin, lattice corneal dystrophy, granular corneal dystrophy, Schnyder corneal dystrophy, congenital stromal corneal dystrophy, fleck corneal dystrophy, macular corneal dystrophy, loss of visual acuity, loss of photopic vision

Abstract

In this chapter the stromal corneal dystrophies have been discussed with epidemiology, inheritance, clinical course, histopathology and treatment. Many corneal dystrophies map to the TGFBI gene which encodes for keratoepithelin, a 683-amino acid protein that is highly conserved among species. The International Committee for the Classification of Corneal Dystrophies (IC3D), classified the stromal corneal dystrophies based on the similar genetic origin (TGFBI gene) as lattice corneal dystrophy (LCD1 and LCD2), granular corneal dystrophy (GCD1 and GCD2), macular corneal dystrophy (MCD), Schnyder corneal dystrophy (SCD), central cloudy corneal dystrophy of François (CCDF), fleck corneal dystrophy (FCD), pre-descemet corneal dystrophy (PDCD) and congenital stromal corneal dystrophy (CSCD). The symptoms of the stromal dystrophies are often predictable-based on the depth of the abnormal deposits. The most prominent symptom in dystrophies with more diffuse stromal opacification is significant loss of visual acuity or loss of photopic vision.

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