Klippel Trenaunay Weber (KTW) Syndrome with solid organ involvement: Case Report of a rare case
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-yearold 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
Download Options
Introduction
KTS syndrome was first described in 1900 by french physician Klippel and Trenaunay in two patients having port wine stain, varicosity in lower limb, bony and soft tissue hypertrophy of affected limbs1 . In 1907, Parkes Weber described a patient with above mentioned features as well as an AVM of the affected limb. This was thus called Klippel Trenuanay Weber syndrome2 It is a rare syndrome and rarely seen and reported by researches. When this KWT syndrome case observed in surgery outdoor, it was further evaluated and was found that it also have Splenic lymphangiomatosis which a further rare entity with KWT. So it was decided to report this rare case.