Port-wine stain in V1 distribution, suspected Sturge-Weber syndrome
Recommended: MRI Brain with and without contrast (look for pial enhancement) is the preferred imaging study for port-wine stain in v1 distribution, suspected sturge-weber syndrome. Leptomeningeal angiomatosis usually ipsilateral to facial port-wine stain.
Recommended study
MRI Brain with and without contrast (look for pial enhancement)
Leptomeningeal angiomatosis usually ipsilateral to facial port-wine stain. Glaucoma in 50%.
If the default doesn't apply
Calcification assessment (older child)
CT
CT Head without contrast
No contrast
Watch-outs
Seizure onset typically < 1 year
Early seizure control critical; refractory cases may require hemispherectomy.
Glaucoma + choroidal hemangioma
Ophthalmology referral essential — vision-threatening.
Pearls
- Tram-track gyriform calcifications on CT — classic but late finding.
- Atrophy + enlarged choroid plexus + deep venous prominence on MRI.
- Pial enhancement on contrast MRI is the earliest finding; consider in infants before classic findings.