port wine naevus
Gorbachev sporting a port wine naevus
A common congenital neurovascular malformation, which appears 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, port-wine nevi may be confined to the occipitoparietal pial vessels, where sluggish blood flow predisposes to hypoxia of underlying cortex; port-wine nevi may occur in the normal population–e.g., Mikhail Gorbachev, or be part of various syndromes–e.g., Klippel-Trenaunay syndrome, Beckwith-Wiedemann syndrome, Cobb syndrome, Rubenstein-Taybi syndrome, trisomy 13 syndrome.
Clinical findings 5% of patients with port-wine nevi have convulsions, mental retardation, hemiparesis or hemianopsia contralateral to lesions
Pathology Densely-packed, dilated capillaries in dermis and subdermis
Management Flashlamp-pulsed tunable argon dye laser, most effective if administered < age 7. More treatment may be required for facial lesions.
Synonyms Gorbachev naevus, naevus flammeus, flat haemangioma, port-wine mark, port-wine stain