Dilated bulbar vessels in<unk> <unk>  is usually manifested at birth by a port-wine stain on the forehead and upper eyelid of one side of the face, or the whole face. The birthmark can vary in color from light pink to deep purple and is caused by an overabundance of capillaries around the ophthalmic branch of the trigeminal nerve, just under the surface of the face. There is also malformation of blood vessels in the pia mater overlying the brain on the same side of the head as the birthmark. This causes calcification of tissue and loss of nerve cells in the cerebral cortex. Neurological signs include seizures that begin in infancy and may worsen with age. Convulsions usually happen on the side of the body opposite the birthmark, and vary in severity. There may also be muscle weakness on the side of the body opposite the birthmark. Some children will have developmental delays and cognitive delays; about 50% will have glaucoma (optic neuropathy often associated with increased intraocular pressure), which can be present at birth or develop later. Glaucoma can be expressed as leukocoria, which should suggest further evaluation for retinoblastoma. Increased pressure within the eye can cause the eyeball to enlarge and bulge out of its socket (buphthalmos).<unk>  rarely affects other body organs. Presentation Seizures 75–90% Vascular headache 40–60% Developmental (cognitive) delay 50–70% Glaucoma 30–70% Hemianopsia 40–45% Hemiparesis 25–60%
Sturge–Weber syndrome
TORCH syndrome
Pelger–Huët anomaly
Schinzel–Giedion syndrome
Cohen-Gibson syndrome
Letterer–Siwe disease
Cornelia de Lange syndrome
Pelizaeus–Merzbacher disease