What is Klippel-Trenaunay Syndrome (KTS)?

Klippel-Trenaunay syndrome (KTS) is a rare congenital (from birth) condition involving the blood vessels, lymphatic system, and surrounding tissues. This syndrome typically affects one or more limbs and the affected limb will be larger. What appears to be a port wine stain may be visible. Over time, the affected limbs may grow larger than the unaffected limbs.

What are the symptoms?

KTS is usually diagnosed via ultrasound during pregnancy. It causes a variety of problems:

  • Pain, cramping, and heaviness in the affected limb
  • Port wine stain over the affected limb
  • Swollen veins
  • Cysts
  • Blood clots and/or cellulitis (a bacterial infection in the skin)

How is KTS diagnosed?

A full physical exam and blood tests will be conducted. Imaging like traditional X-rays; angiography, which uses an injectable dye and X-rays; computed tomography (CT) scan, which produces a three dimensional view of the scanned area; and magnetic resonance imaging (MRI) which uses powerful magnets and radio waves to create detailed organ and tissue mapping. In some cases, a biopsy of the tumor may be needed.

How is it treated?

Your child may require a variety of treatments, depending on how KTS affects him or her. Treatment may involve a multidisciplinary team which could include orthopaedics, plastic surgery, dermatology, vascular anomalies, and other specialties. Treatments may include:

  • Surgery to correct uneven limb growth
  • Sclerotherapy to minimize swollen veins and/or surgery to remove veins
  • Surgery to remove excess tissue

About treatment for Klippel-Trenaunay syndrome at Children’s

Klippel-Trenaunay syndrome is one of the most common conditions treated through Children’s Vascular Anomalies Center, which is located on our Minneapolis campus. By coordinating experts from several pediatric disciplines into a single visit, families experience a new level of convenience in an environment focused exclusively on pediatrics. Every child is assigned a pediatric nurse case manager who coordinates all treatment and communication between care providers and the family.

Contact Us

For consultation, referral, or an appointment, call the Vascular Anomalies Center at 612-813-7100.

For information on a KTS family support group in Minnesota, visit the KT Support Group.