STURGE WEBER SYNDROME

STURGE WEBER SYNDROME-

• Encephalotrigeminal angiomatosis[ETA]
• Sporadic
• Trisystem- Face

                    - Leptomeninges

                    - Eyes

*  Bisystem- Face& Eyes

                           Or         

                 - Face & Leptomeninges

• Presents at birth.
• SIGNS-

A ) FACE-  Port wine stain-over the V N distribution

B) LEPTOMENINGES-

• Ipsilateral parietal / occipital leptomeningeal haemangioma→

                                                                 - Seizures

                                                                 - Hemiparesis

                                                                 -Hemianopia

- MR

C) EYES-

• Glaucoma [IL]
• Diffuse choroidal haemangioma
• IL Episcleral haemangioma
• Heterochromia iridis

   INV-

1. X-ray – “Tramline” cerebral calcification
2. CT scan & MRI- for haemangioma

   T/T-

1. Intractable seizures→ Subtotal Hemispherectomy
2. Portwine stain→ dermatological laser
3. Choroidal haemangioma-

• Photo- argon, xenon, krypton or dye laser
• TTT
• PDT
• Radiation therapy
• Enucleation

4. Glaucoma- Latanoprost 0.005% HS

                    - Goniotomy- For angle anomalies

                    - Trabeculotomy + Trabeculectomy→

                      Removes barrier to aq outflow