“ Spontaneous disorder due to d isrupted development of the cortical veins. The face is affected ( port wine stain in the distribution of ...
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Spontaneous disorder due to disrupted development of the cortical veins. The face is affected (port wine stain in the distribution of the trigeminal nerve) and there are ipsilateral leptomeningeal venous angiomas.
In total, 90% have seizures; these are contralateral to the facial naevus.
CT
• Gyral/subcortical white matter calcification is pathognomic—these are like phleboliths and arise from venous stasis (apparent from 1 year of age).
• Choroid plexus hypertrophy, engorgement and enhancement, ipsilateral to the stain on the face.
• Long term—cortical atrophy on the affected side.
• Check for a choroidal angioma (eye)—leads to glaucoma.
• Angiomas are more common over the parieto-occipital region ![]()
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Axial T1 post-contrast magnetic resonance image of the brain showing a large venous angioma (short arrow) and ipsilateral hypertrophy and enhancement of the choroid plexus (long arrow). The appearance is in keeping with Sturge-Weber syndrome.
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