Cranial CT and MR in the Klippel-Trenaunay-Weber syndrome

Abstract

This report describes the intracranial CT and MR findings in two cases of Klippel-Trenaunay-Weber Syndrome. The findings are 1) markedly enhancing choroid plexuses, 2) severe cerebral atrophy, 3) cerebral calcifications, and 4) angiomatous leptomeningeal enhancement. The findings may resemble those seen in cases of bilateral Sturge-Weber syndrome. The two diseases should be distinguishable by the external stigmata. The authors raise the question of a spectrum of involvement in the angiodysplasias of Klippel-Trenaunay-Weber syndrome and Sturge-Weber syndrome with considerable overlap.