To report a case of a patient with a corneal band-shaped microcystic dystrophy.
A 70-year-old woman with unilateral painless loss of visual acuity presented an epithelial feathery corneal opacification involving visual axis that showed an intraepithelial dense microcystic pattern. Histopathologic examination of the epithelial scraping revealed scattered areas of vacuolization within almost the entire epithelium. Visual acuity improved after treatment, and no recurrence was observed.
Clinical and histopathologic features correspond with those described by Lisch in his original report of 1992.
[Show abstract][Hide abstract] ABSTRACT: Corneal dystrophies are a heterogenous group of rare, inherited corneal diseases that are typically bilateral, symmetric, non-inflammatory, slowly progressive, and usually bear no relationship to environmental or systemic factors. The word dystrophy is derived from Greek literature (dys = wrong, difficult; trophe = nourishment). Clinically, the corneal dystrophies are divided into three groups based on the principal anatomical location of the abnormalities. Some affect primarily the corneal epithelium and its basement membrane or Bowman layer and the superficial corneal stroma (anterior corneal dystrophies), the corneal stroma (stromal corneal dystrophies), or Descemet membrane and the corneal endothelium (posterior corneal dystrophies). Most corneal dystrophies have no systemic manifestations and present with variable shaped corneal opacities in a clear or cloudy cornea and they affect visual acuity to different degrees.
[Show abstract][Hide abstract] ABSTRACT: The identification of the genetic basis of approximately half of the corneal dystrophies in the past decade has resulted in significant advances in our understanding of the genetic control of corneal clarity and has provided clinicians with a definitive means to confirm or refute presumptive clinical diagnoses. This article serves as a guide to understanding the genetic basis of the corneal dystrophies and provides a revised anatomically based classification system that is intended for the clinician, who must possess a working knowledge of the molecular genetic basis of the corneal dystrophies to accurately diagnose, counsel, and manage the disease in affected patients.
Archives of Ophthalmology 03/2007; 125(2):177-86. DOI:10.1001/archopht.125.2.177 · 4.40 Impact Factor
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