Acute keratoconus with perforation in a patient with Down’s syndrome
- J Stoiber1,
- W Muss2,
- J Ruckhofer3,
- G Grabner3
- 1Department of Ophthalmology and Optometry, St Johanns-Spital, Landeskliniken Salzburg, Muellner Hauptstrasse 48, A 5020 Salzburg, Austria
- 2Department of Pathology
- 3Department of Ophthalmology and Optometry
- Correspondence to: Dr Josef Stoiber; j.stoiber{at}lks.at
- Accepted 16 July 2002
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.
Case report
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 …









