The association of glaucoma with facial nevus, accompanied in some instances by symptoms of intracranial disturbance of the opposite side, is a syndrome which was recognized many years ago but which has received little attention on account of its extreme rarity. In a rather complete survey of the available literature reports of fifty-five cases, over a period of seventy-two years, were found.
Schirmer,1 in 1860, was the first to record the association of telangiectasis of the face and body with infantile glaucoma and enlargement of the eyeball. In addition, varicosities of the retinal veins were present.
Allen Sturge,2 in 1879, presented a case of unilateral "port wine" mark of the face, complicated by enlargement of the corresponding eyeball ; there were also changes in the choroid and tortuous retinal vessels. Epileptic seizures, on the side opposite the nevus, were attributed to a similar nevoid condition of the brain.
O'BRIEN CS, PORTER WC. GLAUCOMA AND NAEVUS FLAMMEUS. Arch Ophthalmol. 1933;9(5):715–728. doi:10.1001/archopht.1933.00830010738002
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