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Chapter 59 – Stromal Corneal Dystrophies and Ectasias

Chapter 59 – Stromal Corneal Dystrophies and Ectasias

 

JOEL SUGAR

 

 

 

 

 

DEFINITION

• A disorder in which an abnormal substance accumulates in the cornea.

 

KEY FEATURES

• Characteristically bilateral

• Progressive

• Isolated to the cornea

 

ASSOCIATED FEATURES

• Usually autosomal dominant

• Not associated with systemic disease

 

 

 

INTRODUCTION

A corneal dystrophy is a disorder that is typically bilateral, progressive, and isolated to the cornea; usually it presents in the central cornea. Most disorders are inherited, characteristically in a dominant fashion, and often appear clinically to involve only one layer of the cornea. With the present and ongoing identification of the genes involved in many of these entities, much hope exists that a better pathophysiological understanding. Ultimately this should lead to better treatment for these diseases. The epithelial and endothelial dystrophies are discussed in the chapters on the corneal epithelium ( Chapter 56 ) and corneal endothelium ( Chapter 57 ), respectively.

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