Progressive Schnyder's corneal dystrophy.

Abstract

Schnyder's crystalline dystrophy is a central anterior bilateral corneal dystrophy characterized by the deposition of crystals composed of cholesterol and other lipids. This condition classically is described as nonprogressive after childhood, but a series of recent studies following patients over time has challenged this teaching. The authors present a family pedigree including a patient first reported in 1954 and followed for 25 years with dramatic progression of the corneal condition in a dense, discoid pattern of crystals. Best-corrected visual acuity decreased from 20/30 to 20/80 in both eyes. This patient now has three children, all with typical corneal involvement. Ocular pathologic examination performed on corneal biopsies submitted for light and electron microscopy demonstrated cholesterol deposition consistent with a diagnosis of Schnyder's. Schnyder's corneal dystrophy may progress significantly over time. The characterization of this condition as stationary may need to be reconsidered.