What are the ocular changes 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

Increased conjunctival vascularity can be seen on slit lamp examination or can be viewed by the naked eye as a pinkish discoloration. The abnormal plexus of episcleral vessels may be hidden by the overlying tissue of the Tenon capsule in infancy and only appreciated clinically in later childhood.

Prominent, tortuous conjunctival and episcleral vascular plexuses affect as many as 70% of patients with SWS and often correlate with increased episcleral venous pressure, probably resulting from arteriovenous shunts within the episcleral hemangiomas. The overlying retinal vessels may be affected, demonstrating dilation and tortuosity, as well as peripheral arteriovenous communications.

Iris heterochromia occurs in approximately 10% of patients with SWS. The more deeply pigmented iris usually is ipsilateral to the PWS, signifying an increase in melanocyte number or activity.

The diagnosis of diffuse choroidal hemangioma is based on tumor appearance on indirect binocular ophthalmoscopy.

Several possible mechanisms may be responsible for decrease in visual function in patients with SWS. As soon as the syndrome is first suspected or documented, a complete ophthalmic evaluation is essential to rule out glaucoma, since the infant's eye is damaged quickly by increased intraocular pressure. The earlier glaucoma is documented and the more effectively it is controlled, the less likely secondary glaucomatous changes will occur.


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