Syndrome de Klippel-Trenaunay et grossesse : difficile choix du mode d'accouchement à partir d'un cas et revue de la littérature

Klippel-Trenaunay syndrome (SKT) is a rare disease characterized by cutaneous haemangiomas, capillary malformations venous (venous varicosities), bone and soft tissue hypertrophy affecting one or more limbs. It is often associated with hemorrhagic and thrombotic complications, especially during pregnancy. Vulvovaginal anomalies at risk of bleeding may occur in late trimester of pregnancy, affecting delivery. The terms of delivery depends on the severity of vascular malformations and the experience of the obstetrician. We report the case of a woman in labor at 38 weeks gestation with a SKT with involvement of the left leg up to the corresponding large lip. She presented after vaginal delivery a severe post-partum hemorrhage (2000 mL) secondary to vaginal lacerations requiring sutures and supplemented by arterial embolization. A multidisciplinary approach is required at delivery.