Abstract
INTRODUCTION: Klippel-Trenaunay syndrome (KTS) is a rare congenital disease, characterized by the triad: capillary malformations that manifest as port wine stain, typically venous varicosities, bone and soft tissue hypertrophy. CASE REPORT: LFRR, term newborn weighing 3360g, male, cesarean delivery due to imminent eclampsia (G3P2C0). Apgar score 9/9 with physical examination showing left lower limb with port wine stain, increased volume and diffuse violaceous lesions extending from the toes to the buttocks, palpable and symmetrical peripheral pulses. DISCUSSION: KTS management does not have a well-established consensus, and conservative treatment can be performed using non-invasive measures (elastic or non-elastic compressive stockings) associated or not with psychological support, elevation of the affected limb and physiotherapy. Active conducts such as sclerotherapy can be used in small areas, as well as the use of laser therapy on port wine stains and ulcerations. Sirolimus may be a promising therapeutic option for the management of venous malformations, including KTS, leading to an improvement in symptoms such as pain and a decrease in the size of the lesions. CONCLUSION: KTS, as a rare syndrome, has scarce literature. The main management is based on the use of conservative therapies, others related to the complications presented and now begins the phase of using off-label drugs (Sirolimus) that slow down the progression of the disease.