Year 2019 / Volume 111 / Number 12
Letter
Prehepatic portal hypertension of a vascular origin: Klippel-Trenaunay syndrome

979

DOI: 10.17235/reed.2019.6597/2019

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

Abstract
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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References
1. Frieden IJ, Chu DH. Klippel-Trenaunay syndrome: clinical manifestations, diagnosis, and management, ed. UpToDate. Waltham, MA: UpToDate Inc. http://www.uptodate.com (Accessed on August, 2019.)
2. Kihiczak GG, Meine JG, Schwartzc RA, Janniger CK. Klippel–Trenaunay syndrome: a multisystem disorder possibly resulting from a pathogenic gene for vascular and tissue overgrowth. Int J Dermatol 2006;45:883 –90.
3. Kaise M, Watanabe A, Kobayashi Y. A case of phacomatosis pigmentovascularis accompanied with esophageal varices due to hypoplasia of the portal veins. Gastroenterol Jpn 1992;27(4):546-9.
4. Kim JH, Kim CW, Son DK, Chung BW, Yang SE, Kim JI, et al. A case of Klippel-Trenaunay-Weber syndrome presenting with esophageal and gastric varices bleeding. Korean J Gastroenterol 2004;43:137-41.
5. Bataller R, Sans M, Escorsell A, Elizalde JL, Bosch J, Rodés J. Esophageal variceal bleeding caused by hypoplasia of the portal vein in a patient with Klippel-Trenaunay syndrome. Am J Gastroenterol 1998;93(2):275-6.
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Díaz Alcázar M, Ruiz Escolano E, García Robles A. Prehepatic portal hypertension of a vascular origin: Klippel-Trenaunay syndrome. 6597/2019


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Publication history

Received: 29/08/2019

Accepted: 03/09/2019

Online First: 22/11/2019

Published: 05/12/2019

Article Online First time: 85 days

Article editing time: 98 days


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