Abstract:
: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.
journal_name
Dev Med Child Neuroljournal_title
Developmental medicine and child neurologyauthors
Oyesiku NM,Gahm NH,Goldman RLdoi
10.1111/j.1469-8749.1988.tb04757.xsubject
Has Abstractpub_date
1988-04-01 00:00:00pages
245-8issue
2eissn
0012-1622issn
1469-8749journal_volume
30pub_type
杂志文章abstract::Gliomatosis cerebri is the unifying term used when diffuse glial infiltration occurs throughout the cerebral hemispheres. The very few cases reported in children have presented with intractable epilepsy, corticospinal tract deficits, unilateral tremor, headaches, and developmental delay. Antemortem diagnosis is diffic...
journal_title:Developmental medicine and child neurology
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