Cerebral arteriovenous fistula in the Klippel-Trenaunay-Weber syndrome.
A 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.
Oyesiku
N M
NM
Department of Surgery, University of Connecticut.
Gahm
N H
NH
Goldman
R L
RL
D000798:Angiomatosis / Q000150:complications*; D002533:Cerebral Angiography; D005260:Female; D006801:Humans; D007223:Infant; D002538:Intracranial Arteriovenous Malformations / Q000150:complications* / Q000000981:diagnostic imaging; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications* / Q000000981:diagnostic imaging; D014057:Tomography, X-Ray Computed