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Arch Pediatr Adolesc Med. 2009;163(7):665.

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Denouement and Discussion: Klippel-Trénaunay Syndrome

Klippel-Trénaunay syndrome (KTS) is a rare disorder characterized by the triad of port-wine stain, hypertrophy of bone and soft tissue, and varicose veins or venous malformations. The syndrome was described in 1900 by French physicians Klippel and Trénaunay.¹ In 1907, Weber² noted similar findings in association with arteriovenous malformations.

The diagnosis of KTS can be made when any 2 of the 3 features are present. Jacob et al,³ in a study of 253 patients with KTS, observed that 63% of the patients had all 3 features and that 37% had 2 of the 3 features. Port-wine stain has been observed in 98% of patients, varicosities or venous malformation in 72%, and limb hypertrophy in 67%.

The exact cause of KTS is unknown, although several theories exist. Most cases are sporadic, and a few cases could be of autosomal dominant inheritance. A new theory of paradominant inheritance was suggested in a . . . [Full Text of this Article]

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