Corneal Dystrophies //
Corneal dystrophies are rare, hereditary disorders associated with microscopic deposits or changes in specific layers of the cornea. By convention, corneal dystrophies are primary corneal diseases unassociated with prior inflammation or with underlying metabolic or systemic diseases.
Most dystrophies are inherited as autosomal dominant traits. This means that one would expect to find half the family members of a patient to be similarly affected. Variations in the severity of the dystrophy and the age of its presentation often influence when a patient or family member starts to develop visual problems. As a result, it is not unusual for a patient’s family ocular history to be unremarkable. In many such cases, silent, subclinical manifestations of the dystrophy will be found among other family members when a thorough ophthalmologic examination is performed.
Many dystrophies are subtle, having minimal or no effect on vision. These dystrophies require no specific treatment. In other cases, irregularity of the surface or repeated surface breakdown may lead to blurred or distorted vision and pain. While drops and ointments may reduce these symptoms, often the surface changes need to be scraped or peeled away. Some of the more dramatic dystrophies cause diffuse clouding of the cornea and considerable loss of vision and require corneal transplantation to restore vision to a normal level.
The following is a partial list of corneal dystrophies that we see as corneal subspecialists.
Anterior basement membrane dystrophy
Schnyder crystalline corneal dystrophy
Posterior polymorphous dystrophy
Endothelial or Fuchs’ dystrophy