The three main features are nevus flammeus (port-wine stain), venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb.

[citation needed] Those with large AVMs are at risk of formation of blood clots in the vascular lesion, which may migrate to the lungs (pulmonary embolism).

[14] Debulking has been the most common treatment for KTS for several decades and while improvements have been made, the procedure is still considered invasive and has several risks associated with it.

Mayo clinic studies demonstrate that primary surgical management of KTS has limitations and non-surgical approaches need to be developed in order to offer a better quality of life for these patients.

Ultrasound guided foam sclerotherapy is the state of the art new treatment which could potentially close many large vascular malformations.

Among older children and adults, 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.

For early treatment of infants and toddlers with KTS, custom compression garments are impractical because of the rate of growth.

Possible side-effects include a slight risk that the fluids may simply be displaced to an undesirable location (e.g., the groin), or that the compression therapy itself further impedes circulation to the affected extremities.

[citation needed] The condition was first described by French physicians Maurice Klippel and Paul Trénaunay in 1900; they referred to it as naevus vasculosus osteohypertrophicus.