4.5 Article

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

Journal

BMC PEDIATRICS
Volume 19, Issue -, Pages -

Publisher

BMC
DOI: 10.1186/s12887-019-1480-0

Keywords

Port wine stain; Hypertrophy; Vascular malformation; Klippel-Trenaunay syndrome; Cross-bilateral limb involvement; Polydactyly

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BackgroundKlippel-Trenaunay 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 presentationHere we report the first case of a one-day-old male with KTS presenting with crossed-bilateral limb hypertrophy and post-axial polydactyly.ConclusionThis 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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