Monday, December 26, 2011

Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome is a congenital disorder of limb asymmetry associated with vascular abnormalities. It is also known as nevus vasculosus osteohypertrophicus and Parkes Weber syndrome (after Frederick Parkes Weber).

Diagnosis requires 2 or more of the following three classic characteristics:
  • Cutaneous vascular lesions: Port-wine stain (nevus flammeus). These are mostly capillary malformations and usually involve the affected limb. Most common manifestation (seen in 98% of patients). Unlike hemangiomas, the cutaneous vascular lesions don't evolve with time.
  • Abnormal venous and lymphatic structures : Varicosities and venous malformations can be superficial, deep, or perforating.
  • Enlargement of an extremity: Least common of the three abnormalities. Usually unilateral involvement of a lower extremity. Enlargement of the extremity can be caused by circumferential soft-tissue hypertrophy, bone elongation, or both. The bony and soft-tissue enlargement may be due to local hyperemia and venous stasis from the aforementioned venous abnormalities.
The images above are from a patient with left leg hypertrophy, nevi predominantly on the left foot, and varicose veins throughout the left lower extremity.


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