Which ocular findings are characteristic of Sturge-Weber syndrome (SWS)?

Updated: Dec 26, 2018
  • Author: Masanori Takeoka, MD; Chief Editor: George I Jallo, MD  more...
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Answer

Ocular involvement in SWS may include the following signs:

  • Hemangiomalike, superficial changes (which on histology demonstrate only venous dilation) in the eyelid

  • Buphthalmos

  • Glaucoma

  • Tomato-catsup color of the fundus (ipsilateral to the nevus flammeus) with glaucoma

  • Conjunctival and episcleral hemangiomas

  • Diffuse choroidal hemangiomas

  • Heterochromia of the irides

  • Tortuous retinal vessels with occasional arteriovenous communications

  • Ocular signs that may indicate the presence of infantile glaucoma include the following:

  • Corneal diameter of more than 12 mm during the first year of life

  • Corneal edema

  • Tears in the Descemet membrane (Haab striae)

  • Unilateral or bilateral myopic shift

  • Optic nerve cupping greater than 0.3

  • Any cup asymmetry associated with intraocular pressure above the high teens

  • Optic nerve damage resulting in myopia, anisometropia, amblyopia, strabismus, and visual field defects


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