Brief report
Unilateral glaucoma in Sotos syndrome (cerebral gigantism)

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Abstract

PURPOSE: To report a patient with unilateral glaucoma associated with Sotos syndrome. Sotos syndrome (cerebral gigantism) is a disorder of growth and development with characteristic facial changes and normal endocrine function. Ocular manifestations may also include megalocornea, iris hypoplasia, cataracts, megalophthalmos, strabismus, nystagmus, and retinal dystrophy.

METHODS: Case report. A 50 year-old man with the clinical features of Sotos syndrome presented with complaints of decreased vision in the left eye.

RESULTS: Ophthalmologic examination revealed bilateral megalocornea, megalophthalmos, iris hypoplasia and transillumination defects, cataracts, and unilateral glaucoma. Intraocular pressure was lowered, and visual field loss was stabilized with topical medications.

CONCLUSION: Sotos syndrome patients should be examined routinely to allow for early detection and treatment of potential ocular problems, including glaucoma.

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Supported in part by a grant from Research to Prevent Blindness, New York. Dr. Yen is currently with Oculoplastic Surgery, Inc., Salt Lake City, Utah. Dr. Flynn is currently with the Department of Ophthalmology, Harkness Eye Institute, Columbia University, New York, New York.