KLIPPEL-TRENAUNAY-SYNDROME - CLINICAL-FEATURES, COMPLICATIONS, AND MANAGEMENT

Klippel-Trenaunay Syndrome (KTS) is rare and not well recognized among most physicians, which has lead to inappropriate management and delay ed referral in many cases, Presented herein is the largest series ever reported from a single center in Saudi Arabia, comprised of 18 patien ts seen between 1990 and 1996, whose clinical features, complications, and management are discussed, All 18 patients had large angiomatous n avel, hypertrophy of the soft tissue with bone overgrowth in the lower limbs, and extensive lower limb varicosities, which extended to the b uttocks and lower abdomen in 2 patients. There were 4 patients who had undergone at least one operation for lower limb varicose veins prior to referral, The diagnostic workup and treatment for KTS is discussed in detail, It is essential that physicians understand how this complex syndrome presents, and the most appropriate means of investigating an d managing it, to avoid unnecessary surgery being performed prior to r eferral to a specialist center.