Abstract

MORA-DE LOA, Brenda Janette de la  and  TELLEZ-MARTINEZ, Susana Elizabeth. Primary intestinal lymphagiectasia in Klippel-Trénaunay syndrome. Anesthetic management. Case report. Rev. mex. anestesiol. [online]. 2023, vol.46, n.2, pp.144-148.  Epub Aug 28, 2023. ISSN 0484-7903.  https://doi.org/10.35366/110204.

Klippel-Trénaunay syndrome with associated primary intestinal lymphangiectasia is a rare entity in pediatrics. It presents with edema due to hypoproteinemia, and in some cases with ascites. The objective is to present a clinical case whose diagnosis was certified by intestinal esdoscopy and biopsy. For refractary ascities, the treatment was surgical, performing peritoneal venous bypass. The anesthetic management of this type of patients has been described in limited reports, so in the present case we emphasize supportive therapy with albumin infusion as the proposed symptomatic treatment for patients with massive drainage of ascities.

Keywords : Klippel-Trénaunay; intestinal lymphagiectasia; albumin; pediatric anesthesiology.

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