Klippel Trenaunay Weber Syndrome

It is possible that the major title of the report Klippel-Tr naunay Disorder is not the name you anticipated. Kindly inspect the synonyms detailing to locate the alternating name(s) and problem class(s) covered by this report.


  • KTS

Problem Neighborhoods

  • None

General Conversation
Klippel-Tr naunay disorder (KTS) is an unusual condition that is present at birth (genetic) as well as is defined by a set of three of cutaneous capillary malformation (“port-wine tarnish”), lymphatic abnormalities, and abnormal veins in organization with variable overgrowth of soft tissue as well as bone. KTS happens most often in the lower arm or leg and less typically in the top extremity and also trunk. KTS similarly impacts males and ladies.

The eponym KTS has actually produced controversy in the medical literary works considering that the first record of the condition in the early 20th century. The French medical professionals, Klippel as well as Tr naunay, described people with capillary spots (poorly called “hemangiomas” at that time), venous varicosities, and over growing. At concerning the exact same time, the English skin specialist Parkes Weber reported the mix of “hemangiomas” and also overgrowth of an arm or leg. For several years, the names of all three physicians were linked as a confusing (as well as wrong) term “Klippel-Weber-Tr naunay syndrome,” which still is (unfortunately) utilized to now.

Since the latter 20th century, it is well-recognized that Parkes Weber and also Klippel-Tr naunay disorders are totally different. Parkes Weber disorder includes fast-flow, several tiny arteriovenous links with variable capillary discoloration of a bigger arm or leg (generally the lower extremity). By genetic screening, many of these patients have a dominant, germline anomaly in the gene RASA1.

On the other hand, KTS is a slow-flow consolidated vascular problem involving unusual veins (C), lymphatics (L) and also veins (V). Therefore, lots of investigators make use of the abbreviation CLVM, instead of KTS, as well as limit the classification for patients that have all three vascular irregularities. Various other authors use the KTS term a lot more broadly and include clients with just capillary discolor (CM) or capillary as well as venous anomalies (CVM) in the arm or leg in the absence of lymphatic problems.

Once the genetic source for KTS is discovered, it will be possible to much more specifically designate clients with these various combinations of vascular irregularities.

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