A 6-month old child presents with po wine stain, mental retardation, and recurrent focal seizures. All are true about the condition except?
Diagnosis is Sturge Weber syndrome. Strawberry Hemangioma is not a/w Sturge Weber syndrome. Choice A is correct since due to the presence of glaucoma, cupping of optic disc is seen. Choice B is correct due to presence of intracranial calcification, which on CT SCAN skull produces a tram-track appearance Choice C is correct as refractory focal seizures in patient will require vagal nerve stimulation to control recurrent seizures. D/D of Sturge Weber syndrome: 1. Klippel-Trenaunay-Weber syndrome Po-wine stain on the extremities and face Hemi hyperophy of soft and bony tissues. 2. Beckwith-Wiedemann syndrome Facial po-wine stain (PWS), Macroglossia, Omphalocele, Visceral hyperplasia. Severe hypoglycemia resulting from pancreatic islet-cell hyperplasia
Optic nerve cupping
Tram track appearance on CT scan on skull
vagal nerve stimulation to control recurrent seizures
Strawberry Hemangioma
CNS