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What is A 6-month old child presents with po wine stain, mental retardation, and recurrent focal seizures. All are true about the condition except? and explain in detail?
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