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Keratoconus Complicated by Acute Corneal Hydrops

Hiroko Suzuki, M.D., and John Robert Minarcik, M.D.

N Engl J Med 2009; 361:e32October 15, 2009

Article

A 21-year-old woman with a history of myopia presented with the acute onset of foggy vision in her right eye and associated photophobia and mild sensation of the presence of a foreign body. Visual acuity was 20/20 in the unaffected eye and hand-motion vision only in the affected eye. She had a bulging, cloudy cornea (Panel A) and protrusion of the lower eyelid on downward gaze, known as the Munson sign (Panel B). Slit-lamp examination showed edema and opacification of the central cornea (corneal hydrops) (Panel C).

Keratoconus is a disorder in which the cornea assumes an irregular conical shape. Acute hydrops is a well-known complication, occurring in approximately 3% of patients with keratoconus. Hydrops occurs after rupture of the posterior cornea leads to an influx of aqueous humor into the cornea, resulting in edema. Corneal edema typically resolves in 6 to 10 weeks; therefore, hydrops is usually not an indication for emergency corneal transplantation. Infectious causes of corneal opacification and visual loss, such as bacterial, viral, or fungal keratitis, must be ruled out as the cause of acute visual loss.

Hiroko Suzuki, M.D.
John Robert Minarcik, M.D.
U.S. Naval Hospital Okinawa, Okinawa, Japan