• Dev Med Child Neurol · Apr 1988

    Case Reports

    Cerebral arteriovenous fistula in the Klippel-Trenaunay-Weber syndrome.

    • N M Oyesiku, N H Gahm, and R L Goldman.
    • Department of Surgery, University of Connecticut.
    • Dev Med Child Neurol. 1988 Apr 1;30(2):245-8.

    AbstractA 12-week-old girl presented with cutaneous haemangiomata and hemihypertrophy of the chest and right upper extremity--stigmata of the Klippel-Trenaunay-Weber syndrome. Screening cranial CT-scan followed by cerebral angiogram revealed what is believed to be the first reported occurrence of a cerebral arteriovenous fistula in a patient with this syndrome. Craniotomy and clip application successfully obliterated the fistula. Although rare at present, neurovascular involvement in the Klippel-Trenaunay-Weber syndrome may be found more frequently with the newer non-invasive diagnostic methods for screening these children. Such findings may strengthen the putative classification of this syndrome within the group of primary mesodermal (vascular) phakomatoses.

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