Pediatric Terrien Marginal Degeneration

Abstract

Purpose: To describe 2 cases of Terrien marginal degeneration (TMD) in young children. Methods: A total of 2 pediatric patients aged 5 and 10 years with unremarkable familial history underwent routine ophthalmological examination because of the presence of superior corneal opacities with no other associated symptoms. The younger patient showed lipid deposition adjacent to the superior limbus and peripheral thinning with vessels coursing across the left eye. The visual acuity was 20/20 in both eyes. The older patient had similar clinical features. The visual acuity improved from 20/25 and 20/60 in the right and left eye, respectively, to 20/20 with subjective refraction in both eyes. The left eye revealed a superior crescent-shaped corneal opacity and superotemporal peripheral thinning associated with lipid deposition and superficial vascularization. Serological markers for autoimmune diseases were negative. The patients were diagnosed with TMD. Results: Corneal tomography (Pentacam, Oculus, Wetzlar, Germany) revealed steepening of the superior cornea of the affected eye in both patients. Anterior segment optical coherence tomography of the younger patient confirmed marked furrow indentation and peripheral thinning. The older patient showed peripheral stromal thinning on Scheimpflug imaging. These findings supported the diagnosis of TMD. Conclusions: Ophthalmologists should acknowledge that TMD can present with or without ocular inflammation, especially at a young age. Given the variable progression rate of TMD, ongoing and thorough monitoring is crucial for effective management and prognosis in affected patients.