• Steven Yeh and Janine A Smith.
• Division of Epidemiology and Clinical Research, National Eye Institute, National Institutes of Health, Bethesda, MD 20892, USA.
• Cornea. 2008 Oct 1; 27 (9): 1062-5.

PurposeTo describe a patient with cone dystrophy who presented with acute hydrops and perforation, leading to the diagnosis of keratoconus.MethodsCase report and literature review.ResultsA 21 -year-old male patient with a history of cone dystrophy presented with a flat anterior chamber, diffuse corneal stromal edema with an intrastromal cleft, and ruptured Descemet membrane, findings consistent with acute hydrops with corneal perforation. After bandage contact lens placement and instillation of a cycloplegic agent, the anterior chamber reformed within 24 hours. Over the next week, conservative management with a bandage lens, pressure patching, topical fluoroquinolone antibiotic, and topical cycloplegic led to the reformation and maintenance of anterior chamber stability. Corneal topography of the unaffected eye showed global corneal thinning and steep sim K readings suspicious for early keratoconus.ConclusionsAlthough the association between keratoconus and cone dystrophy is extremely rare, our patient's vision-threatening complication of acute hydrops with corneal perforation highlights the importance of corneal evaluation including topography in cone dystrophy. Conservative management was successful in the restoration of anatomic integrity in this situation.

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