Terrien Marginal Degeneration: Clinical Characteristics and Outcomes

To describe outcomes of patients with Terrien marginal degeneration.

Retrospective case series.

Database review of 25 patients (43 eyes) seen over 10 years (2004-2013) at Toronto Western Hospital cornea clinic. Outcome measures included demographics, location of disease, topographic astigmatism, visual acuity, coexisting ocular disease, and surgical management.

Mean age at presentation was 44 years (range, 20-82 years) and 54% were male. Eighteen patients (72%) had bilateral disease. Mean follow-up was 30.3 months. Mean topographic astigmatism was 4.02 diopters (D) at 5 degrees. Mean change in astigmatism 1 year from baseline was 0.75 D; at 2 years was 1.22 D; and at 3 years was 1.68 D. Mean best spectacle-corrected visual acuity (BSCVA) at presentation was 20/46 and 20/48 at last follow-up. Eyes requiring surgery (23.3%) had mean BSCVA of 20/81 at presentation and 20/106 after surgery. Five eyes perforated: 4 spontaneously, and 1 from trauma. Three eyes (6.9%) presented with pseudopterygium. Two eyes (4.7%) had intracorneal cysts. Fourteen patients (56%) presented with ocular surface inflammation.

Terrien marginal degeneration is a slow-progressing, bilateral but asymmetric degeneration of the peripheral cornea. Men over 40 are more commonly affected. Stromal thinning, vascularization, lipid deposition, and against-the-rule astigmatism are classic signs. Though typically noninflammatory, a variant form characterized by prominent inflammation exists. Surgery (lamellar graft) can preserve corneal integrity and is indicated when conventional options fail to maintain vision or if perforation is imminent. Perforations are rare but can result in significant vision loss.