The leptomeningeal angiomatosis, on the side of the facial nevus, usually in the occipital or occipito-parietal region, is composed of thin-walled small- and medium-sized arteries, veins, and capillaries often showing proliferation, degenerative changes and calcifications (see Fig. 13.31).

Cortical calcifications occur roughly beneath the leptomeningeal angiomatosis. The size of calcific deposits varies from small mulberry-like granules to large coalescent concretions (see Fig. 13.31).

Cortical cytoarchitectonic anomalies and atrophic and dystrophic changes are common.

Encephalofacial angiomatosis may occur in conjunction with angiomatosis osteohypertrophy or the Klippel Trenaunay-Weber syndrome. This consists of cutaneous and bony angiomatosis and hypertrophy of the limbs.

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