Primary X-linked megalocornea presenting in adulthood

Abstract

A48-year-old visually asymptomatic male was referred for assessment of possible arrested congenital glaucoma. No relevant medical or family history were reported and the visual acuities and intraocular pressures were within normal limits bilaterally. Examination revealed prominent arcus lipoides (Figure 1A), crocodile shagreen (Figure 1B), mild cataract and an open, heavily pigmented iridocorneal angle in each eye. Fundoscopy was unremarkable with healthy optic discs. Further evaluation revealed horizontal corneal diameter of 14mm (Figure 1C), central corneal thickness of ∼410µm and anterior chamber depth of 6mm bilaterally.