Corneal Perforation In Terrien Marginal Disease : Case Report

Our case describes a patient with bilateral peripheral corneal ulceration, complicated by an inferior corneal perforation in the right eye, which required surgical treatment with a peripheral crescentic corneal graft.

The patient is a 33-year-old man with no history of ocular disease, ocular surgery or trauma, presenting with eye pain, redness, and photophobia in the right eye. Ophthalmological examination revealed a best-corrected visual acuity of 20/200 in the right eye and 20/30 in the left eye. 

Slit-lamp examination showed 360° peripheral corneal thinning with corneal neovascularization and lipid deposition in the right eye,  and inferior corneal thinning in the left eye. The Seidel test was positive in the right eye, indicating an inferior corneal perforation. A complete workup was conducted for the patient, including systemic biological examinations, all of which yielded negative results. The final diagnosis was marginal Terrien disease, based on the clinical features, the patient’s background, and the absence of systemic pathology. Due to the inferior corneal perforation, the treatment was surgical, involving an urgent tectonic crescent-shaped lamellar corneal graft. 

Terrien disease is a rare ophthalmological condition without other associated systemic conditions. Corneal grafts can help preserve the eye's integrity in case of impending or established corneal perforation. According to some studies, standard techniques do not achieve optimal visual outcomes in peripheral corneal disorders. Therefore, small-diameter tectonic grafts or crescent-shaped patch grafts are more commonly used. We performed crescent lamellar keratoplasty for our patient and achieved a satisfactory anatomical and functional result, with the disappearance of the Seidel sign and a reduction in inflammatory signs. The visual outcome is satisfactory, with a slight improvement with glasses.

Perforation in advanced peripheral corneal thinning disorders is challenging to treat due to the location of the pathology and the high risk of graft failure. The primary goal of treatment is to reduce ocular morbidity through both topical and surgical approaches.

In severe cases, targeted tectonic corneal transplantation, particularly in the form of a peripheral patch graft, remains the surgical treatment of choice.