Klippel-Trenaunay Syndrome (KTS)

 

Klippel-Trenaunay syndrome is a slow-flow combined vascular anomaly (capillary-lymphatic-venous malformation) that is typically associated with marked overgrowth of the leg and geographic capillary stains.  The condition may rarely be associated with hypotrophy. Anomalous lateral veins, which are typically on the lateral aspect of the thigh, become prominent because of incompetent valves and deep venous anomalies. 

 
                            

Photo#1 shows typical features of Klippel-Trenaunay syndrome, enlarged leg with a capillary malformation or port-wine stain and varicoid veins. Figure#2 is a magnetic resonance venography showing varicoid venoud drainage on the outer side of the leg with absence of the deep venous system. This appearance is common in patients with Klippel-Treanaunay syndrome.  

Potential Complications: Thrombophlebitis and pulmonary embolism are the main complications in these patients. Pain is very severe in some patients. There may be symptoms related venous insufficiency in the diseased extremity.

 
     

Photos Provided by Dr. Marc A. Brenner, Podiatric Medicine and Surgery, Glendale, NY

 
 
 

ImagingImaging, typically MRI and/or MRA, is mainly used to evaluate the extent and patency of the deep veins. MRI findings are variable because of the wide range in the severity of this disorder. The lymphatic component of the disease may be macrocystic or microcystic (see the MRI in coronal section). Increased fat is often identified in the areas of soft-tissue overgrowth. In severe cases, the deep venous system is anomalous (hypoplastic or interrupted), and drainage occurs mainly via markedly dilated, anomalous superficial veins. The pathognomonic marginal vein of Servelle is frequently identified in the lateral calf and thigh. MR venography (MRV) is the imaging modality of choice in the evaluation of the patency of deep veins before attempts at surgery. Contrast-enhanced angiography may reveal discrete microarteriovenous fistulas.  

   
   First image is a conventional venogram showing a marginal vein on the lateral side of the thigh with a large communicating venous channel draining into the deep veins. 2nd image is a MR venogram (MRV) demonstrating multiple veins draining the leg via a marginal vein with no visualization of the deep veins. Normal deep veins in the right thigh. 
 
 

 

Recent Literature

  • The importance of "geographic stains" in identifying lymphatic disease and risk of complications. This retrospective review study included 40 patients with KTS. This study concluded that the presence of a geographic vascular stain is a predictor of the risk of both associated lymphatic malformation and complications in patients with KTS >>> more
  • Wilms tumor screening is unnecessary in Klippel-Trenaunay syndrome: 115 patients with Klippel-Treanaunay syndrome were evaluated in this study. The study concluded that patients with KTS are not at increased risk for developing Wilms tumor and thus should not undergo routine ultrasonographic screening  >>> more

 

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