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Case Reports and Small Case Series
September 1998

Hemifacial Atrophy and Primary Corneal Endothelial Failure

Arch Ophthalmol. 1998;116(9):1246-1248. doi:

Hemifacial atrophy (Parry-Romberg syndrome) is characterized by a slowly progressive atrophy of the skin, subcutaneous tissue, and muscle and bone of the face, usually beginning in the first 2 decades of life.1 This disease progresses through an active phase of decompensation, generally lasting 2 to 10 years, followed by a quiescent phase without continued atrophy.2 Ocular findings most commonly described include progressive enophthalmos, restrictive strabismus, pupillary disturbances, heterochromic iridocyclitis, and blepharoptosis.1,3 To our knowledge, this case is the first report of primary corneal endothelial failure and penetrating keratoplasty in association with Parry-Romberg syndrome.

A 59-year-old woman with a history of left hemifacial atrophy since age 15 years had continuous pain in the left eye that was unresponsive to topical lubricants. She had a history of ocular hypertension treated with a topical β-blocker and a single episode of iritis in the left eye.