Abstract—Klippel-Trenaunay syndrome (KTW) is a rare, sporadic syndrome characterized by a triad of port-wine stain, varicose veins with or without venous malformations, and bony and soft tissue hypertrophy. It usually affects only one extremity, organ involvement is uncommon. A case of 17-year-old boy with this syndrome having solid viscous organ involvement was observed at surgery Outdoor. This case is rare so it was reported. Splenic lymphangiomatosis is also observed in this KTW syndrome case as association of AVM and lymphangioma together in spleen is rarest which was observed in this case. So it is a very rare case.
Keywords: Arterio-Venous Malformations, Limb Hypertrophy, Port-Wine Stain, Splenic Lymphangiomatosis
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