Early surgical management of Klippel-Trenaunay syndrome in childhood can prevent long-term haemodynamic effects of distal venous hypertension

Abstract Purpose: The aim of this study was to evaluate the authors' preliminary experience in early surgical treatment of distal venous hypertension (DVH) in children affected by Klippel-Trenaunay syndrome (KTS). Methods: Clinical assessment, surgical management, and outcome of 29 children (18 girls, 11 boys) affected by KTS observed from October 1998 to October 2000 were reviewed retrospectively. Results: Patients ranged in age from 8 months to 17 years (median age at surgery, 10.3 years). The clinical findings are presented. Surgical treatments included stripping of persistent marginal vein (n = 16), multiple legation of bulky varicosities (n = 10), complementary sclerotherapy (n = 14) and laser photocoagulation (n = 13), and excision of associated lymphatic malformations (n = 5). No mortality or major postoperative morbidity occurred. Follow-up period ranged from 6 months to 2 years. Conclusion: These preliminary results suggest that early surgical management of DVH in KTS is safe and could be effective in preventing or minimising the long-term haemodynamic effects of DVH in absence of associated deep venous system anomalies. J Pediatr Surg 37:232-235. Copyright © 2002 by W.B. Saunders Company.