Klippel Trenaunay Syndrome

It is possible that the primary title of the record Klippel-Tr naunay Disorder is not the name you anticipated. Kindly examine the basic synonyms listing to discover the alternating name(s) as well as disorder community(s) covered by this report.


  • KTS

Condition Class

  • None

General Discussion
Klippel-Tr naunay disorder (KTS) is an uncommon problem that is present at birth (congenital) and is characterized by a triad of cutaneous capillary malformation (“port-wine tarnish”), lymphatic irregularities, as well as uncommon capillaries in association with variable over growing of soft tissue and bone. KTS takes place most regularly in the lower limb and much less typically in the upper extremity and trunk. KTS similarly impacts men as well as women.

The eponym KTS has produced debate in the medical literature because the initial report of the condition in the early 20th century. The French physicians, Klippel and also Tr naunay, explained people with capillary stains (poorly called “hemangiomas” during that time), venous varicosities, and over growing. At concerning the same time, the English skin doctor Parkes Weber reported the mix of “hemangiomas” and over growing of an arm or leg. For years, the names of all three doctors were linked as a complicated (and also improper) term “Klippel-Weber-Tr naunay syndrome,” which still is (sadly) used to now.

Since the last 20th century, it is well-recognized that Parkes Weber and Klippel-Tr naunay disorders are totally different. Parkes Weber syndrome includes fast-flow, numerous tiny arteriovenous connections with variable capillary discoloration of a bigger limb (generally the reduced extremity). By hereditary testing, a lot of these clients have a dominant, germline mutation in the genetics RASA1.

In contrast, KTS is a slow-flow consolidated vascular condition involving unusual veins (C), lymphatics (L) as well as veins (V). Consequently, many private investigators utilize the acronym CLVM, as opposed to KTS, as well as restrict the designation for individuals that have all 3 vascular abnormalities. Various other writers use the KTS term much more broadly as well as consist of patients with just capillary discolor (CENTIMETERS) or capillary and also venous irregularities (CVM) in the limb in the absence of lymphatic irregularities.

Once the genetic reason for KTS is uncovered, it will certainly be possible to a lot more exactly mark patients with these various mixes of vascular anomalies.

Leave a reply

Your email address will not be published. Required fields are marked *