1. A naevus (port wine stain) of one side of the face, which may involve one or more divisions of the trigeminal nerve. It is often associated with progressive mental retardation, which is present in 60%, and severe in 32%.
2. Skin haemangiomas can occur at other sites. Glaucoma and buphthalmos may occur. Massive involvement of the paranasal sinuses and facial distortion has been reported (Ku et al 1999).
3. Epilepsy, and a hemiparesis involving the contralateral side may occur. Uncontrolled status epilepticus may result in permanent cerebral damage. Fits may be refractory to drug control. Gum hypertrophy may be secondary to phenytoin therapy.
4. Stroke-like episodes. Decreased blood flow may affect neurological function by interfering with glucose metabolism.
5. There are variations in the full clinical picture. One side of the vault and the hemiparetic half of the body may be smaller than the other.There may be unilateral glaucoma, increased scalp vascularity, and unilateral hypertrophy of the carotid artery.
6. A venous haemangioma usually involves the meninges of the occipitoparietal surface of the brain.The adjacent cortex is gradually destroyed, possibly as a result of pressure. Deeper arteriovenous malformations, which occur only rarely, may be fed by large arteries and increasingly large veins. If this happens, a considerable arteriovenous shunt may result in cardiac hypertrophy and failure.
7. Skull X-ray shows linear calcification of the underlying brain tissue.
8. Arteriography or DVI will demonstrate the extent of the lesion.
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