port-wine nevus

port-wine nevus

Nevus flammeus, flat hemangioma, port-wine mark or stain  
A common congenital neurovascular malformation, appearing as deep red-purple macular lesions, corresponding to cutaneous angioma(s), often located in the ophthalmic branch of the trigeminal nerve; when located on the meninges, PWN may be confined to the occipitoparietal pial vessels, where sluggish blood flow predisposes to hypoxia of underlying cortex Clinical 5% of Pts with PWNs have convulsions, mental retardation, hemiparesis or hemianopsia contralateral to lesions; PWNs may occur in the normal population–eg, Mikhail Gorbachev, or be part of various syndromes–eg, Klippel-Trenaunay, Beckwith-Wiedemann, Cobb, Rubenstein-Taybi, trisomy 13 syndromes Management Flashlamp-pulsed tunable argon dye laser, most effective if administered < age 7; may require more therapy for facial lesions