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Images in Neurology |

Neurologic and Vascular Abnormalities in Klippel-Trenaunay-Weber Syndrome

Dimitri Renard, MD; Chantal Campello, MD; Guillaume Taieb, MD; Jessica Orgeval, MD; Luc Jeanjean, MD; Laurent Collombier, MD; Liliane Metge, MD; Caroline Briere, MD; Giovanni Castelnovo, MD; Alain Bonafe, MD, PhD
JAMA Neurol. 2013;70(1):127-128. doi:10.1001/jamaneurol.2013.587.
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A 56-year-old woman woman with congenital Klippel-Trenaunay-Weber syndrome presented with progressive, fluctuating, right frontal headache and left brachiofacial paresthesias. Clinical examination showed right-sided predominant bone and soft tissue hypertrophy, varicose veins, capillary hemangiomae, and conjunctival varicosities in both eyes, in the absence of neurologic abnormalities (Figure 1).

Figure 1. Photographs and magnetic resonance and positron-emission tomographic images of 56-year-old patient. A, Right-sided leg hypertrophy, with varicose veins and capillary hemagiomae. B, Inferonasal conjunctival varicosities. C, Eye fundus examination showing retinal vascular tortuosities. D, Gadolinium-enhanced magnetic resonance angiography revealing tortuous extracranial vertebral arteries. E, Hemispheric brain asymmetry (right bigger than left) is seen on T2-weighted magnetic resonance imaging (MRI), especially in the posterior cortical-subcortical regions (arrows) and the thalamus (arrowhead). F, Gadolinium-enhanced T1-weighted MRI showing increased vascularization (arrows) in the cortical, subcortical (especially in the watershed areas; arrowheads), and deep gray matter regions of the right hemisphere. The right hemisphere is hypometabolic (arrows) on positron emission tomography with 18fluorodeoxyglucose (G, 3-dimensional reconstruction image, superior view; H, axial view, arrowheads showing deep gray matter regions) and hyperperfused on perfusion-weighted MRI (I, arrows).

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Figure 1. Photographs and magnetic resonance and positron-emission tomographic images of 56-year-old patient. A, Right-sided leg hypertrophy, with varicose veins and capillary hemagiomae. B, Inferonasal conjunctival varicosities. C, Eye fundus examination showing retinal vascular tortuosities. D, Gadolinium-enhanced magnetic resonance angiography revealing tortuous extracranial vertebral arteries. E, Hemispheric brain asymmetry (right bigger than left) is seen on T2-weighted magnetic resonance imaging (MRI), especially in the posterior cortical-subcortical regions (arrows) and the thalamus (arrowhead). F, Gadolinium-enhanced T1-weighted MRI showing increased vascularization (arrows) in the cortical, subcortical (especially in the watershed areas; arrowheads), and deep gray matter regions of the right hemisphere. The right hemisphere is hypometabolic (arrows) on positron emission tomography with 18fluorodeoxyglucose (G, 3-dimensional reconstruction image, superior view; H, axial view, arrowheads showing deep gray matter regions) and hyperperfused on perfusion-weighted MRI (I, arrows).

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Figure 2. Angiography showing extensive capillary-venous angiomatosis involving the cortical and deep gray matter regions of the right cerebral hemisphere (A-C, arrows) and the right cerebellar hemisphere (E, arrow), an intracavernous aneurysm of the right-sided carotid artery (A, arrowhead), tortuous extracranial vertebral arteries (D), segmental fibromuscular dysplasia in the extracranial internal carotid (B, arrowhead) and vertebral (E, arrowhead) arteries, and tortuous branches of the right external carotid artery (F).

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