Klippel-Trenaunay-Weber syndrome with atypical presentation of hypersplenism and nephrotic syndrome : A case report
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2017-08-21
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Abstract
Background: Klippel-Trenaunay-Weber syndrome is a rare syndrome; unfortunately, very few studies of the connection between hypersplenism, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome have been published. Case presentation: We report the case of a 40-year-old white man with a typical clinical presentation of Klippel-Trenaunay-Weber syndrome, including "port-wine stains," varicose veins, hypertrophy of lower extremities, and arteriovenous fistula, as well as an unfortunate development of hypersplenism and nephrotic syndrome. Conclusions: This case report described considerable atypical relevance of Klippel-Trenaunay-Weber syndrome and hypersplenism together with nephrotic syndrome. A multidisciplinary approach was made. Unfortunately, hypersplenism is characterized by pancytopenia that suggests splenectomy, whereas nephrotic syndrome is an indication for renal biopsy; the splenectomy and renal biopsy were delayed due to our patient's severe condition. Deeper analysis including study of other patients with Klippel-Trenaunay-Weber syndrome would help us to understand the connection between elevated spleen and liver sizes, nephrotic syndrome, and Klippel-Trenaunay-Weber syndrome.
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Publisher Copyright: © 2017 The Author(s).
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Hypersplenism, Klippel-Trenaunay-Weber syndrome, Nephrotic syndrome, 3.2 Clinical medicine, 1.1. Scientific article indexed in Web of Science and/or Scopus database, General Medicine
Citation
Kundzina, L & Lejniece, S 2017, 'Klippel-Trenaunay-Weber syndrome with atypical presentation of hypersplenism and nephrotic syndrome : A case report', Journal of Medical Case Reports, vol. 11, no. 1, 243. https://doi.org/10.1186/s13256-017-1413-1