Sturge-Weber syndrome is
a rare congential vascular condition characterized by a
facial portwine stain, with or without leptomeningeal
angiomatosis. The
nevus flammeus of the face and/or eyelids corresponds to
the distribution of the trigerminal nerve. The
conjunctival and choroidall vessels are usually engorged,
and the raised episcleral venous pressure can produce
glaucoma in one-third of these cases. Glaucoma associated
with this syndrome can occur at any age, representing a
significant visual threat often requiring surgery.
A choroidal hemangioma is
the most common intraocular abnormality associated with
Sturge-Weber syndrome. The angioma of the pia and
arachnoid often alters the vascular supply of the
underlying cerebral cortex, leading to secondary
degeneration and calcification.
Treatment varies according
to the location of the abnormality and the extent of
ocular involvement.
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