Klippel-Trenaunay syndrome associated with antithrombin III deficiency

Klippel-Trenaunay syndrome (KTS) is a rare phakomatosis characterized by cutaneous hemangiomata, venous varicosities and bone and soft tissue hypertrophy also of the affected limb. Central nervous system involvement is rare, arising from a malformation or from coagulation disorders. We report the ca...

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Bibliographic Details
Published in:Revue neurologique 2008-10, Vol.164 (10), p.855-858
Main Authors: Grira, M, Ben-Jemaa, H, Lammouchi, T, Benammou, S
Format: Article
Language:fre
Subjects:
Adult
Antithrombin III Deficiency - blood
Antithrombin III Deficiency - complications
Antithrombin III Deficiency - diagnostic imaging
Brain - pathology
Collateral Circulation - physiology
Humans
Klippel-Trenaunay-Weber Syndrome - blood
Klippel-Trenaunay-Weber Syndrome - complications
Klippel-Trenaunay-Weber Syndrome - diagnostic imaging
Leg - diagnostic imaging
Leg - pathology
Magnetic Resonance Imaging
Male
Radiography
Stroke - etiology
Vena Cava, Inferior - pathology
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Description
Summary:Klippel-Trenaunay syndrome (KTS) is a rare phakomatosis characterized by cutaneous hemangiomata, venous varicosities and bone and soft tissue hypertrophy also of the affected limb. Central nervous system involvement is rare, arising from a malformation or from coagulation disorders. We report the case of a patient presenting a KTS with stroke. The biological assessment revealed antithrombin III deficiency. Although rare, antithrombin III deficiency should be kept in mind in KTS patients with neurological involvement.
ISSN:0035-3787
DOI:10.1016/j.neurol.2007.09.009