Unilateral glaucoma in Sotos syndrome (cerebral gigantism)

Am J Ophthalmol. 2000 Dec;130(6):851-3. doi: 10.1016/s0002-9394(00)00640-1.


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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Abnormalities, Multiple / etiology*
  • Abnormalities, Multiple / pathology
  • Brain / abnormalities*
  • Cataract / etiology
  • Cornea / abnormalities
  • Cornea / pathology
  • Eye Abnormalities / etiology*
  • Eye Abnormalities / pathology
  • Gigantism / complications*
  • Glaucoma / etiology*
  • Humans
  • Intraocular Pressure
  • Iris / abnormalities
  • Iris / pathology
  • Male
  • Middle Aged
  • Syndrome
  • Visual Fields