BACKGROUND: 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. PATIENTS: 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. RESULTS: Ocular pathologic examination performed on corneal biopsies submitted for light and electron microscopy demonstrated cholesterol deposition consistent with a diagnosis of Schnyder's. CONCLUSION: Schnyder's corneal dystrophy may progress significantly over time. The characterization of this condition as stationary may need to be reconsidered.
BACKGROUND: 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. PATIENTS: 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. RESULTS: Ocular pathologic examination performed on corneal biopsies submitted for light and electron microscopy demonstrated cholesterol deposition consistent with a diagnosis of Schnyder's. CONCLUSION:Schnyder's corneal dystrophy may progress significantly over time. The characterization of this condition as stationary may need to be reconsidered.