Klippel Trenaunay syndrome

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**Klippel Trenaunay syndrome**
*Classification & external resources*
ICD-10	Q 87.2 (EUROCAT Q87.21)
ICD-9	759.89
OMIM	149000
DiseasesDB	29324
eMedicine	derm/213

Klippel Trenaunay syndrome or KTS is a medical condition in which blood vessels and/or lymph vessels fail to form properly.

The condition was first described by French physicians Klippel and Trenaunay in 1900 and termed naevus vasculosus osteohypertrophicus.

This syndrome is commonly confused with Parkes Weber syndrome, and also is sometimes incorrectly termed "Klippel Trenaunay Weber syndrome". The widely accepted name is as the title reads.

1 Symptoms and Diagnosis
2 Treatment
3 See also
4 References
5 External links

[edit] Symptoms and Diagnosis

Although the cause and processes surrounding Klippel Trenaunay syndrome are poorly understood, the disease is diagnosed by the presence of a combination of these symptoms on approximately ¼th of the body:

A distinctive port-wine stain with sharp borders
Varicose veins
Hypertrophy of bony and soft tissues, that may lead to local gigantism or shrinking.
An overdeveloped lymph system

Note that KTS can either affect blood vessels, lymph vessels, or both. Most commonly is a mixture of the two. Those with veinous involvements are subject to an overall harder lifestyle due to the increased pain and complications.

The disease affects men and women equally, and is not limited to any racial group. It is not believed to be genetic in nature, although testing is ongoing. [1]

[edit] Treatment

KTS is a complex syndrome, and no single treatment is applicable for everyone. The best method for deciding on a treatment is to talk to your doctor

Debulking has been the most widely used treatment for the syndrome, and has been used for decades. Progress has been made in this method over the course of the past couple decades, but it is still an invasive procedure, and has many complications. As other choices now exist for KTS patients, this method is generally reserved as a last resort.

Mayo Clinic has reported the largest experience in managing KTS with major surgery. In 39 years at Mayo clinic the surgery teem evaluated 252 consecutive cases of KTS, of which only 145 (57.5%) could be treated by primary surgery. The immediate success rate for treating varicose veins was only 40%, excision of vascular malformation was possible in (60%) , debulking operation (65%) and correction of bone deformity / limb length correction ( epiphysiodesis 90 % success). All the procedures demonstrated high recurrence rate in the follow up. Mayo clinic studies demonstrate that primary surgical management of KTS has limitations and non-surgical approaches needs to be developed for offering a better quality of life in these patients. Major surgery including amputation and debulking surgery does not seem to offer any benefit on a long-term basis.

Sclerotherapy is a a treatment for specific veins and vascular malformations in the affected area. Such treatment may allow normal blood flow to resume. While considered a surgical procedure, it is not nearly as invasive as Debulking. Ultrasound guided foam sclerotherapy is the state of the art new treatment which could potentially close many large vascular malformations . Debulking operation could result in major deformities and has a high potential for recurrence and nerve injuries.

Compression Garments are finding more use as of the last ten years. The greatest issue with KTS syndrome is that the blood flow and/or lymph flow may be impeded, and will pool in the affected area. This can cause pain, swelling, inflammations, and in some cases, even infection. Compression garments can be used to alleviate almost all of these, and when combined with elevation of the affected area and proper management, can result in a comfortable lifestyle for the patient without any surgery. Compression garments are also used lately after a Debulking procedure to maintain the results of the procedure. While compression garments are not applicable for everyone, they are relatively cheap (compared to surgery), and have little side-effects. Though side-effects are generally minor or non-existent, there is a slight risk that the pooling may be moved to a more undesirable location, such as the groin.

Other treatments are also available, including Massage Therapy and other surgical procedures. There is not, however, any sort of cure for KTS, at this time.

[edit] See also

Casey Martin

[edit] References

Jacob AG, Driscoll DJ, Shaughnessy WJ, Stanson AW, Clay RP, Gloviczki P.Klippel-Trenaunay syndrome: spectrum and management[2]
Gloviczki P, Stanson AW, Stickler GB, Johnson CM, Toomey BJ, Meland NB, RookeTW, Cherry KJ Jr.Klippel-Trenaunay syndrome: the risks and benefits of vascular interventions[3]
Cabrera J, Cabrera J Jr, Garcia-Olmedo MA, Redondo P.Treatment of venous malformations with sclerosant in microfoam form.Arch Dermatol. 2003 Nov;139(11):1494-6 [4]
Lisko, Jane H. and Frederick Fish.Klippel-Trenaunay-Weber Syndrome (May 27, 2003). Accessed July 25, 2004.
McDonagh, B.1; Sorenson, S.1; Cohen, A.1; Eaton, T.1; Huntley, D.E.1; La Baer, S1; Campbell, K.1; Guptan, R.C.Phlebology, Volume 20, Number 2, June 2005, pp. 63-81(19)Management of Venous Malformations in Klippel-Trenaunay(KT) Syndrome With Ultrasound Guided foam Sclerotherapy Klippel Trenaunay Syndrome
Treatment of KTS with foam sclerotherapy. Venous Research Foundation [5]