- Page(s) : 625-34
- Published in: 1998
Various patterns of complex-combined vascular malformations in the lower limbs are important to recognise. We report eight cases of such vascular anomalies in order to define their specific current management. Eight cases are described and this allowed us to identify five different patterns: an extensive portwine stain with congenital limb hypertrophy and no subsequent worsening (case n°1 ); a pure extensive venous malformation involving skin as well as muscles and joints (case n° 2); case n° 3 had a very rare extensive micro and macro-kystic lymphatic malformation ; three patients had the so called Klippel and Trenaunay syndrome e.g. a complex-combined capillary, venous and lymphatic malformation with possible gigantism of the limb (cases n° 4, 5, 6); case n° 7 had capillary, lymphatic and venous anomalies with hypotrophy of the affected limb (Servelle-Martorell syndrome); case n° 8 had a severe high-flow vascular malformation with hypertrophy of the leg (Parkes Weber syndrome). Vascular anomalies in the lower limb are more or less invasive depending on their type, the most deeply invasive being the purely venous or lymphatic types: these can permeate the skin, the muscles, and even the joints. Complex-combined anomalies of the Klippel-Trenaunay or Servelle-Martorell types usually involve only the skin and subcutaneous tissues. Investigations include MRI, CT scans, Doppler evaluation. Angiographies are not systematically requested. They are decided in accordance with the clinical symptom (e.g. when arteriovenous shunting is evidenced clinically and by Doppler, arteriography may be indicated). Orthopedic management is always necessary particularly in patients with either hypo- or hypertrophy of the affected limb. From a cosmetic point of view, it is rarely possible to improve the situation. Elastic stockings are always necessary. Cardiac symptoms can develop in the high- flow vascular anomalies (Parkes Weber type) but they are much less common than usually said.