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

https://doi.org/10.1136/bjo.87.1.120

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    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 …

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