Biomaterial Database

Cerebral arteriovenous fistula in the Klippel-Trenaunay-Weber syndrome. 1988

N M Oyesiku, and N H Gahm, and R L Goldman

Department of Surgery, University of Connecticut.

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.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007715	Klippel-Trenaunay-Weber Syndrome	A congenital disorder that is characterized by a triad of capillary malformations (HEMANGIOMA), venous malformations (ARTERIOVENOUS FISTULA), and soft tissue or bony hypertrophy of the limb. This syndrome is caused by mutations in the VG5Q gene which encodes a strong angiogenesis stimulator.	Klippel-Trenaunay Disease,Angio-Osteohypertrophy Syndrome,Angioosteohypertrophy Syndrome,Congenital Dysplastic Angiopathy,KTW Syndrome,Klippel Trenaunay Syndrome,Klippel-Trenaunay Syndrome,Klippel-Trénaunay-Weber Syndrome,Angio Osteohypertrophy Syndrome,Angio-Osteohypertrophy Syndromes,Angioosteohypertrophy Syndromes,Angiopathies, Congenital Dysplastic,Angiopathy, Congenital Dysplastic,Congenital Dysplastic Angiopathies,Disease, Klippel-Trenaunay,Dysplastic Angiopathies, Congenital,Dysplastic Angiopathy, Congenital,KTW Syndromes,Klippel Trenaunay Disease,Klippel Trenaunay Weber Syndrome,Klippel Trénaunay Weber Syndrome,Klippel-Trenaunay Syndromes,Syndrome, Angio-Osteohypertrophy,Syndrome, Angioosteohypertrophy,Syndrome, KTW,Syndrome, Klippel Trenaunay,Syndrome, Klippel-Trenaunay,Syndrome, Klippel-Trenaunay-Weber,Syndrome, Klippel-Trénaunay-Weber,Syndromes, Angio-Osteohypertrophy,Syndromes, Angioosteohypertrophy,Syndromes, KTW,Syndromes, Klippel-Trenaunay
D002533	Cerebral Angiography	Radiography of the vascular system of the brain after injection of a contrast medium.	Angiography, Cerebral,Angiographies, Cerebral,Cerebral Angiographies
D002538	Intracranial Arteriovenous Malformations	Congenital vascular anomalies in the brain characterized by direct communication between an artery and a vein without passing through the CAPILLARIES. The locations and size of the shunts determine the symptoms including HEADACHES; SEIZURES; STROKE; INTRACRANIAL HEMORRHAGES; mass effect; and vascular steal effect.	Arteriovenous Malformations, Cerebral,Intracranial Arteriovenous Malformations, Congenital,AVM (Arteriovenous Malformation) Intracranial,Cerebral Arteriovenous Malformations,Congenital Intracranial Arteriovenous Malformations,Intracranial Arteriovenous Malformation, Ruptured,Ruptured Intracranial Arteriovenous Malformation,Arteriovenous Malformation, Cerebral,Arteriovenous Malformation, Intracranial,Arteriovenous Malformations, Intracranial,Cerebral Arteriovenous Malformation,Intracranial Arteriovenous Malformation,Malformation, Cerebral Arteriovenous,Malformation, Intracranial Arteriovenous,Malformations, Cerebral Arteriovenous,Malformations, Intracranial Arteriovenous
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000798	Angiomatosis	A condition with multiple tumor-like lesions caused either by congenital or developmental malformations of BLOOD VESSELS, or reactive vascular proliferations, such as in bacillary angiomatosis. Angiomatosis is considered non-neoplastic.	Angiomatoses
D014057	Tomography, X-Ray Computed	Tomography using x-ray transmission and a computer algorithm to reconstruct the image.	CAT Scan, X-Ray,CT Scan, X-Ray,Cine-CT,Computerized Tomography, X-Ray,Electron Beam Computed Tomography,Tomodensitometry,Tomography, Transmission Computed,X-Ray Tomography, Computed,CAT Scan, X Ray,CT X Ray,Computed Tomography, X-Ray,Computed X Ray Tomography,Computerized Tomography, X Ray,Electron Beam Tomography,Tomography, X Ray Computed,Tomography, X-Ray Computer Assisted,Tomography, X-Ray Computerized,Tomography, X-Ray Computerized Axial,Tomography, Xray Computed,X Ray Computerized Tomography,X Ray Tomography, Computed,X-Ray Computer Assisted Tomography,X-Ray Computerized Axial Tomography,Beam Tomography, Electron,CAT Scans, X-Ray,CT Scan, X Ray,CT Scans, X-Ray,CT X Rays,Cine CT,Computed Tomography, Transmission,Computed Tomography, X Ray,Computed Tomography, Xray,Computed X-Ray Tomography,Scan, X-Ray CAT,Scan, X-Ray CT,Scans, X-Ray CAT,Scans, X-Ray CT,Tomographies, Computed X-Ray,Tomography, Computed X-Ray,Tomography, Electron Beam,Tomography, X Ray Computer Assisted,Tomography, X Ray Computerized,Tomography, X Ray Computerized Axial,Transmission Computed Tomography,X Ray Computer Assisted Tomography,X Ray Computerized Axial Tomography,X Ray, CT,X Rays, CT,X-Ray CAT Scan,X-Ray CAT Scans,X-Ray CT Scan,X-Ray CT Scans,X-Ray Computed Tomography,X-Ray Computerized Tomography,Xray Computed Tomography