Characterised by
1.LEPTO-MENINGIAL ANGIOMAS
2.INTRACRANIAL CALCIUM ACCUMULATION
3.SEIZURES
4.FACIAL BITH MARKS - NAEVUS FLAMMEUS- PORT WINE STAIN ON FACE
5.INTRAOCULAR ANGIOMAS LEADING TO SECONDARY GLAUCOMA
The Port Wine stain on the face occurs on the same side as that of the leptomeningeal angiomas and along the distribution of trigeminal nerve.In some cases , it does not occur at all and in some cases it is B/L.
Eye problems occur in approximately 70 percent of patients on the same side of the head as the portwine birthmark and clumps of blood vessels (leptomeningeal angiomatosis) accompanied by intracranial calcifications. These eye problems do not tend to occur in Sturge-Weber patients who have no portwine birthmarks. In many cases, the problem is glaucoma. Typically, it is present at birth, accompanied by enlargement of the eyeball (buphthalmos), but it may begin anytime before (and/or) after the age of two years. Other eye anomalies include clumps of blood vessels (angiomas) in the membranes that line the inner surface of the eyelids (conjunctiva), choroid and cornea; loss of vision in half the visual field in one or both eyes (hemaniopsia); eyes of two different colors (i.e., one blue eye and one brown eye); an abnormal accumulation of fluid inside the eyeball causing enlargement (hydrophthalmos); optic atrophy; clouding (opacification) or displacement of the lens; retinal detachment; streaks resembling blood vessels (angioid streaks); or loss of sight due to an organic lesion in the visual cortex (cortical blindness).
1.LEPTO-MENINGIAL ANGIOMAS
2.INTRACRANIAL CALCIUM ACCUMULATION
3.SEIZURES
4.FACIAL BITH MARKS - NAEVUS FLAMMEUS- PORT WINE STAIN ON FACE
5.INTRAOCULAR ANGIOMAS LEADING TO SECONDARY GLAUCOMA
The Port Wine stain on the face occurs on the same side as that of the leptomeningeal angiomas and along the distribution of trigeminal nerve.In some cases , it does not occur at all and in some cases it is B/L.
Eye problems occur in approximately 70 percent of patients on the same side of the head as the portwine birthmark and clumps of blood vessels (leptomeningeal angiomatosis) accompanied by intracranial calcifications. These eye problems do not tend to occur in Sturge-Weber patients who have no portwine birthmarks. In many cases, the problem is glaucoma. Typically, it is present at birth, accompanied by enlargement of the eyeball (buphthalmos), but it may begin anytime before (and/or) after the age of two years. Other eye anomalies include clumps of blood vessels (angiomas) in the membranes that line the inner surface of the eyelids (conjunctiva), choroid and cornea; loss of vision in half the visual field in one or both eyes (hemaniopsia); eyes of two different colors (i.e., one blue eye and one brown eye); an abnormal accumulation of fluid inside the eyeball causing enlargement (hydrophthalmos); optic atrophy; clouding (opacification) or displacement of the lens; retinal detachment; streaks resembling blood vessels (angioid streaks); or loss of sight due to an organic lesion in the visual cortex (cortical blindness).
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