KLIPPEL-TRENAUNAY-SYNDROME - SPECTRUM AND MANAGEMENT

Objective: To describe a series of 252 patients with Klippel-Trenaunay syndrome (KTS), a rare congenital malformation characterized by the t riad of capillary malformations, atypical varicosities or venous malfo rmations, and bony or soft tissue hypertrophy usually affecting one ex tremity. Material and Methods: We reviewed the clinical characteristic s and findings in 136 female and 116 male patients with KTS who underw ent assessment at Mayo Clinic Rochester between January 1956 and Janua ry 1995. In addition, management options are discussed. Results: Capil lary malformations (port-wine stains) were found in 246 patients (98%) , varicosities or venous malformations in 182 (72%), and limb hypertro phy in 170 (67%). All three features of KTS were present in 159 patien ts (63%), and 93 (37%) had two of the three features. Atypical veins, including lateral veins and persistent sciatic vein, occurred in 182 p atients (72%). Operations performed in 145 patients with KTS included epiphysiodesis, stripping of varicose veins or venous malformations, e xcision of vascular malformations, amputations, and debulking procedur es. Conclusion: Most patients with KTS should be managed conservativel y. The dearest indication for operation is a leg length discrepancy pr ojected to exceed 2.0 cm at skeletal maturity, which can be treated wi th epiphysiodesis in the growing child. If a functioning deep vein sys tem is present, removal of symptomatic varicosities or localized super ficial venous malformations in selected patients can yield good result s.