Year 2019 / Volume 111 / Number 12
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Year 2019 / Volume 111 / Number 12

Research letter

Prehepatic portal hypertension of a vascular origin: Klippel-Trenaunay syndrome

979

María del Mar Díaz Alcázar, Elena Ruiz Escolano, Adelina García Robles,

Female of 17 years-old with Klippel-Trenaunay syndrome: port-wine stain, overgrowth of bone and soft tissue in limbs, dental malposition and intellectual disability. Moreover, severe portal hypertension due to portal vein malformation. The Klippel-Trenaunay syndrome includes venous and capillary malformations in the skin, hypertrophy of bones and soft tissue in a limb and, in some patients, lymphatic malformations. Structural abnormalities of the deep venous system can also occur in addition to the key clinical features, although there are few case reports with portal hypertension due to portal vein malformation published.

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The Spanish Journal of Gastroenterology is the official organ of the Sociedad Española de Patología Digestiva, the Sociedad Española de Endoscopia Digestiva and the Asociación Española de Ecografía Digestiva
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