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Acute keratoconus with perforation in a patient with Down’s syndrome

Down’s syndrome has been reported to be frequently associated with keratoconus, a chronic non-inflammatory corneal disorder leading to scarring and progressive stromal thinning. An incidence of up to 15% in patients with Down’s syndrome was reported in the literature.1 Acute keratoconus or “corneal hydrops” is a frequent feature in these patients leading to a further decrease in visual acuity and a mostly central corneal opacification.2 The spontaneous appearance of a fistula in the acute hydrops state in keratoconus has very rarely been observed. We describe both the clinical and histopathological findings in a patient with Down’s syndrome with acute keratoconus who underwent penetrating keratoplasty following a spontaneous corneal perforation.

We report on a 59 year old female patient with Down’s syndrome and late stage keratoconus who was referred to our clinic with a 1 day history of corneal hydrops on her left eye. On initial slit lamp examination a marked oedema of the central corneal stroma …