A 10-yr old male child was presented to the pediatrician for evaluation of a seizure disorder. On examination a vascular plaque was found along the ophthalmic and maxillary divisions of the trigeminal nerve. The mother informed the pediatrician that the lesion was present since birth and there was no change in morphology. The most likely possibility ls- (AIIMS Nov 2014)
|A||Sturge Weber syndrome|
Sturge weber syndrome
o This child has :
i) Seizure disorder
ii) Vascular plaque along the ophthalmic and maxillary divisions of trigeminal nerve
iii) Lesion presenting since birth and not chonging in morphology
o The diagnosis is sturge weber syndrome.
o Sturge weber syndrome is a rate congenital vascular disorder characterized by facial capillary malformation (port wine stain) and associated capillary venous malformation affecting the brain and eye.
it consists of angiomas involving the leptomeninges and skin of face typically in the ophthalmic and maxillary distributions of trigeminal nerve