An atypical case of Klippel-Trénaunay syndrome presenting with crossed-bilateral limb hypertrophy and postaxial polydactyly: A case report

Rawan M. Al-Najjar, Rafael Fonseca

Research output: Contribution to journalArticle

Abstract

Background: Klippel-Trénaunay syndrome (KTS) is a rare congenital condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft tissue hypertrophy. Case presentation: Here we report the first case of a one-day-old male with KTS presenting with crossed-bilateral limb hypertrophy and post-axial polydactyly. Conclusion: This case serves to highlight the variable presentation and multiple problems faced by patients with KTS and why multidisciplinary management is mandatory.

Original languageEnglish (US)
Article number95
JournalBMC Pediatrics
Volume19
Issue number1
DOIs
StatePublished - Apr 6 2019

Fingerprint

Hypertrophy
Extremities
Port-Wine Stain
Polydactyly
Varicose Veins
Postaxial Polydactyly
Capillary Malformations, Congenital, 1

Keywords

  • Cross-bilateral limb involvement
  • Hypertrophy
  • Klippel-Trénaunay syndrome
  • Polydactyly
  • Port wine stain
  • Vascular malformation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

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abstract = "Background: Klippel-Tr{\'e}naunay syndrome (KTS) is a rare congenital condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft tissue hypertrophy. Case presentation: Here we report the first case of a one-day-old male with KTS presenting with crossed-bilateral limb hypertrophy and post-axial polydactyly. Conclusion: This case serves to highlight the variable presentation and multiple problems faced by patients with KTS and why multidisciplinary management is mandatory.",
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AU - Fonseca, Rafael

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AB - Background: Klippel-Trénaunay syndrome (KTS) is a rare congenital condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft tissue hypertrophy. Case presentation: Here we report the first case of a one-day-old male with KTS presenting with crossed-bilateral limb hypertrophy and post-axial polydactyly. Conclusion: This case serves to highlight the variable presentation and multiple problems faced by patients with KTS and why multidisciplinary management is mandatory.

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