Clinical Details:11yr old male patient presented to us with H/O seizures. CT was advised.

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Legend: image 1,2,3 –plain axial CT brain shows atrophy of left cerebral hemisphere with gyriform calcification . image 4,5 -Contrast scan shows increased enhancement of ependymal and medullary veins .

Discussion:
Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a sporadically occurring phakomatosis characterized by “port-wine stain” vascular nevus flammeus in the trigeminal nerve distribution, leptomeningeal venous angiomatosis, seizures , dementia, hemiplegia, hemianopsia, buphthalmos and glaucoma. Faulty development of cortical venous drainage is the cause of this syndrome.

Pathology:
Plexus of multiple small telengiectatic capillaries or venules that lies along the brain in sub-arachnoid space.There is venous stasis and vascular congestion with hypoxia of cortex.slowly there is atrophic changes of the brain and dystrophic calcification in middle layers of cortical gray matter.Angioma itself is not calcified.

Age:
Sturge Weber syndrome is unusual before 2 years of age.

Imaging:

1) Calcification
Located in cortex underlying angioma
Unusual before 2yrs of age
Often gyriform,curvilinear
Most common in parital,occipital lobes

2) Atrophy
Atrophic cortex with dystrophic calcification
Adjacent subarachnoid spaces enlarged
Secondary skull changes
Thickened diploic space
Ipsilateral frontal sinus enlarged
Enlarged mastoid with elevated petrous ridge

3) Enhancement may occur in ipsilateral choroid plexus, angioma, chronically ischaemic cortex, or all three areas.

4) Enlarged medullary , supenpendymal veins

5) Ocular lesions
Buphthalmos
Scleral/choroidal angiomata

MR angiography along with MR imaging can give detailed information regarding the vascular anomalies in Sturge Weber syndrome .

Associated anomalies:
One third of patients with Sturge Weber have ocular abnormalities like “Congenital Glaucoma with bupthalmos in 10-30% while scleral and choroidal angiomata is also common.”
Sturge Weber syndrome is also associated with the angio-osteo-hypertrophy of Klippel-trenaunay syndrome.

Management:
The condition is managed conservatively with the use of anti-epileptics.

Refrences:

1. Audren F, Abitbol O, Dureau P. Non-penetrating deep sclerectomy for glaucoma associated with Sturge-Weber syndrome. Acta Ophthalmol Scand. Oct 2006;84(5):656-60. [Medline].
2. Board RJ, Shields MB. Combined trabeculotomy-trabeculectomy for the management of glaucoma associated with Sturge-Weber syndrome. Ophthalmic Surg. Nov 1981;12(11):813-7. [Medline].
3. Cheng KP. Ophthalmological manifestations of Sturge-Weber syndrome. In: Brodensteiner JB, Roach ES, eds. Sturge-Weber Syndrome. 1999.
4. Cibis GW, Tripathi RC, Tripathi BJ. Glaucoma in Sturge-Weber syndrome. Ophthalmology. Sep 1984;91(9):1061-71. [Medline].

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