Klippel-Trenaunay-Weber syndrome with atypical presentation of hypersplenism and nephrotic syndrome: A case report

Linda Kundzina (Coresponding Author), Sandra Lejniece

Research output: Contribution to journalArticlepeer-review

5 Citations (Scopus)
4 Downloads (Pure)


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.

Original languageEnglish
Article number243
JournalJournal of Medical Case Reports
Issue number1
Publication statusPublished - 21 Aug 2017


  • Hypersplenism
  • Klippel-Trenaunay-Weber syndrome
  • Nephrotic syndrome

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database


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