Terrien marginal degeneration presenting with spontaneous corneal perforation

To report spontaneous corneal perforation as a presenting sign of unilateral Terrien marginal degeneration in a young adult.

A 19-year-old woman presented with sudden loss of vision in her left eye of 1-day duration. Examination revealed superior corneal thinning with superficial vascularization and secondary lipid keratopathy. There was an area of corneal perforation at the 11 o'clock position, leading to a shallow anterior chamber, ocular hypotony, and a hypotonic maculopathy. The right eye was normal on biomicroscopy.

The area of perforation was confirmed by a positive Seidel test. A 22-mm-diameter bandage contact lens was fitted, which led to closure of the perforation and reformation of the anterior chamber. Orbscan corneal topography confirmed superior corneal thinning, 2.3 D of against-the-rule astigmatism, and 3.4 D of irregular astigmatism in the central 3-mm zone. Topography of the right showed no apparent abnormality.

Terrien marginal degeneration is an unusual cause for a spontaneous corneal perforation. Patients with this disorder should be warned about this possibility.