Peripheral Hypertrophic Subepithelial Corneal Degeneration

The purpose of this study was to report a large series of patients with peripheral hypertrophic subepithelial corneal degeneration (PHSCD) and differentiate the condition from Salzmann nodular degeneration (SND).

We retrospectively reviewed the charts of 49 patients diagnosed with PHSCD and reported their clinical, refractive, and topographic/tomographic findings.

Most of the eyes were white and quiet. Minimal variable injection was present in a few eyes usually in the presence of pseudopterygium. Typical corneal involvement consists of peripheral circumferential-elevated whitish subepithelial opacities with fine superficial vessels along the limbus and linear deposits of iron in the epithelium along the central edge of the opacification. The typical topographic/tomographic findings consist of flattening directly over the corneal opacification with central flattening aligning with the axis of the opacification. In all subjects, the mean refractive astigmatism was significantly less than the mean topographic/tomographic Sim K astigmatism. Thirty-five eyes underwent surgical excision. The surgical eyes demonstrated significantly less astigmatism and better best-spectacle corrected visual acuity than pre-op. Moreover, all the eyes that underwent surgery for discomfort experienced significant improvement in their symptoms. Histopathology of the keratectomy specimens demonstrated paucicellular subepithelial fibrosis with overlying epithelium that was variable in caliber.

PHSCD is distinct from SND, primarily occurring in middle-aged women, bilateral and fairly symmetric with larger more peripheral opacities than SND, and absence of inflammatory signs and symptoms.