Klippel trenaunay weber

Clinical Syndrome

• Port-wine stain
• Varicose veins
• Bony and soft tissue extremity hypertrophy

Arteriovenous fistulas, the feature that distinguishes Klippel-Trenaunay syndrome from Parkes-Weber syndrome, are rarely found in the affected extremity.

Possible causes include:

1. Intrauterine damage to the sympathetic ganglia or intermediolateral tract leading to dilated microscopic arteriovenous anastomoses.
2. Deep vein abnormalities, with resultant obstruction of venous flow, lead to venous hypertension, the development of varices, and limb hypertrophy.
3. Underlying mixed mesodermal and ectodermal dysplasia.