The clinical syndrome of naevus flammeus associated with glaucoma has been recognized for many years. It is a relatively rare condition. Schirmer1 in 1860 was the first to describe this clinical entity, and since then about 75 cases have been reported. O'Brien and Porter2 in a complete survey of the literature showed that in the majority of the cases the glaucoma was of the infantile type and generally unilateral, the affected eye in many instances being partially or completely blind.
Salus3 was the first author to show that the glaucoma associated with naevus flammeus need not be congenital but may appear later in life. He cited 2 cases ; 1 was that of a woman aged 45 and the other that of a man aged 57.
Usually, the nevus and the glaucoma occur on the corresponding side of the face, but there are exceptions to this rule. Löwenstein
PINCUS MH. NAEVUS FLAMMEUS ASSOCIATED WITH GLAUCOMAREPORT OF A CASE. Arch Ophthalmol. 1939;21(5):741–745. doi:10.1001/archopht.1939.00860050025003
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