Abstract

P 099

Schnyder’s crystalline corneal dystrophy: a clinico-pathological correlation

Fromberg C.¹, Gatzioufas Z.¹, Löw U.¹, Schirra F.¹, Moritz R.², Seitz B.^1
1Department of Ophthalmology, University of Saarland, Homburg/Saar, ²Department of Pathology, Universtity of Saarland, Homburg/Saar

Objective: Schnyder’s crystalline corneal dystrophy (SCCD) is a rare autosomal dominantly inherited corneal disorder affecting especially the central but also the peripheral corneal stroma and is characterized by cholesterol deposition in the stroma due to abnormal lipid metabolism. Only few sporadic cases are reported in the literature and therefore the pathophysiology of this entity is not thoroughly investigated up to date.
Methods: We report on a 71-year-old female patient with SCCD and coexistant systemic hyperlipidemia who underwent penetrating keratoplasty (PKP) on the right eye, whereas the left eye was already operated 20 years ago with signs of incipient “recurrence” on the graft. The corneal button obtained from the patient was subjected to histopathological examination, qualitative biochemical analysis of the corneal lipid composition and finally to electron microscopy.
Results: The cornel lipid accumulated in the stroma was predominantly composed of cholesterol, cholesterol ester and phospholipid. Histopathological analysis revealed the presence of cholesterol crystals mostly in anterior stroma and to some extent in the deeper stromal layers along with eminent endothelial damage. Moreover, electron microscopy revealed ultrastructural changes throughout the stroma as well as in the corneal endothelium. The most striking findings were numerous stromal electron-lucent spots representing areas of lipid deposition inside corneal keratocytes located in the superficial stroma and focal endothelial discontinuities as a result of endothelial cell degeneration.
Conclusions: In patients with SCCD systemic hyperlipidemias should be ruled out. Moreover, it seems that penetrating keratoplasty still represents the most appropriate surgical approach in SCCD, since our electron-microscopy results suggest that SCCD affects not only the anterior stroma but also the posterior corneal layers and the endothelium. Recurrence of SCCD on the graft seem to appear very late.

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