Sturge Weber Syndrome

Charactersitic features:-

• Port wine stain in trigeminal distribution area.(upperface, eyelids)
• Periventricular calcification
• Hemiparesis contralateral to facial lesion side
• Mental retardation
• Seizures( may develop in first year)
• Developmmental delays
• Glaucoma (Later on)

Dx:-

Skulll Radipgraphs
Ct Head

Rx:-

Portwine stain:
it doesnt resolve on its own , so either need
cosmetic coverage or
Flashlamp pulsed laser therapy.
Seizures: Need apt medication; uncontrolled seizures may be cured by hemispherectomy and hemilobectomy and may play a role in preserving mental functions .
Treatment is conservative in absence of mental retardation.

Important:- Uncontrolled seizures appearing in infancy may be associated with hemiparesis.