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Pediatrics Central Nervous System 94fb54dc

A 7-year-old boy presents with a right-sided hemangioma and left-sided focal seizures. The most likely diagnosis is:

A
Neurofibromatosis
B
Incontinentia pigmenti
C
Hyper melanosis of Ito
D
Sturge-Weber syndrome
High-Yield Explanation
d. Sturge-Weber syndrome(Ref: Nelson's 20/e p 2879, Ghai 8/e p 586)Sturge-Weber Syndrome (SWS)Angiomas involving leptomeninges & skin of face, typically in ophthalmic & maxillary divisions distributions of trigeminal nerve.The hallmark of SWS is a facial cutaneous venous dilation, also referred to as a nevus flammeus or port-wine stain.Contralateral focal seizures, calcification of cerebral cortex and glaucoma on same side as skin lesions are seen.

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