Klippel Trenaunay Syndrome

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Klippel Trenaunay Syndrome (KTS) is a rare and sporadic vascular malformation.  It is characterized by the presence of

  • Capillary Malformation- often called a port wine stain
  • Soft tissue over-growth of the affected area
  • Bone over-growth of the affected area
  • Varicosities of the affected area.

 

Most often KTS occurs in one lower extremity, however it can affect the upper extremities or may occur in both arms and legs simultaneously

 

KTS is a mixed vascular malformation with large capillary, venous and lymphatic anomalies.  There is generally no arterio-venous shunting.

 

Management of this syndrome depends on the extent of the diagnosis.  Conservative management is preferred and designed to manage symptoms.

 

Imaging of the affected area helps identify the venous anatomy involved and the extent of the abnormalities.  Imaging may include MRI, Arteriogram and ultrasound.  Imaging should be used to confirm venous anatomy and drainage prior to any invasive interventions.

 

Compression therapy is often used to manage the swelling and pain associated with KTS.  Fitted compression garments are prescribed to reduce the swelling in the affected limb which may also alleviate some pain.

 

Laser can be used to treat the vascular components of port wine stain and other vascular anomalies seen in the skin.

 

Ablation of the affected veins and malformations may slow the progression of KTS.  Sclerotherapy with alcohol, foam or glues are often used.  These agents are injected into the vessels resulting in a collapse of the vessel and reduced blood flow through the malformation.  Thermal Ablation, Surgical Stripping and Phlebectomy are commonly used to ablate the affected veins and malformations.  Techniques are used to decrease bleeding and prevent pulmonary emboli are employed during these procedures.  They include the use of an intra-operative tourniquet and inferior vena cava filters.

 

Most Vascular Anomaly Treatment Centers or multi-disciplinary vascular anomaly teams advise a multi-disciplinary approach to the management of KTS.

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References:

Dogan R, Dogan OF, Akata D, Gumus B, Balkanci F, A reare vascular malformatin, Klippel Trenaunay Syndreom, Report of a case with deep vein agenesis and review, J Cardiovas Surg, 2003 Feb:44(1):95-100 ( Abstract)

MCGrory BJ, AmadioPC, Klippel Trenaunay Syndrome:  Orthapaedic Considerations, Ortho Rev, 1993, Jan:22(1):41-50.

Patel GK, BanerjeeD, Harding KG, Klippel Trenaunay Syndreom: the implications for wound are.  J Wound Care 2001, mar, 10(3):73-75

 

 

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Revised 08/08_2011

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