Klippel–Trénaunay syndrome

Klippel–Trénaunay syndrome, formerly Klippel–Trénaunay–Weber syndrome and sometimes angioosteohypertrophy syndrome and hemangiectatic hypertrophy, is a rare congenital medical condition in which blood vessels and/or lymph vessels fail to form properly. The three main features are nevus flammeus (port-wine stain), venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb. It is similar to, though distinctly separate from, the less common Parkes Weber syndrome.

Klippel–Trénaunay Syndrome
Other namesKTS or KT
MRI
SpecialtyMedical genetics 

The classical triad of Klippel–Trenaunay syndrome consists of:

  1. vascular malformations of the capillary, venous and lymphatic vessels;
  2. varicosities of unusual distribution, particularly the lateral venous anomaly; and
  3. unilateral soft and skeletal tissue hypertrophy, usually the lower extremity.
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