KLIPPEL-TRENAUNAY SYNDROME: CASE REPORT.

Introduction: Klippel- Trenaunay Syndrome (KTS) is characterized by a series of malformation concemig lymphatic vessels, capillaries and veins, without evidence of arterial-venousos shunt, that mainly involve one side. Typical pathological aspects are: cutaneous capillary malformations; regarding soft tissues or limbs bones hypertrophy; abnormal development of deep and superfici al veins and persistence of embryonal veins. Clinical case: We refer 11 months old patient's experience with a diagnosis of KTS, both for clinical picture, and for age was performed a conservative therapy with pressure bandage. Discussion: KTS is a rare congenital pathology. Its etiology is still unknown, but there are several embryo-genetic theories trying to explain altered angiogenesis, as VEGF (Vascular Endothelian Grow Factor) is considering the main responsible factor. The sintomathology is related to extension and site of the hypertrophy and vascular malformations. Presence of ulcer and edema at the limb, with or without pain is typical. Diagnostic protocol foresees: dermatological, orthopaedic and vascular evaluation; Rx of injuried limb; Color-Doppler; RMN; preoperative and intra-operative flebology. Regarding KTS treatment, it was proposed in literature a marginai veins "stripping", a binding of varicosity, abscission of lymphatic malformations, photocoagulation or sclerotherapy. However, the therapeutic approach remains related to the age of the patient and to the symptomatology and complications that eventually occurred. Surgical treatment, during paediatric age, is not properly opportune because of the high percentage offailure. For this reason children affected by KTS, we consider more useful a conservative treatment with pressure bandage and annual revaluation of clinical picture