[Klippel-Trenaunay syndrome and pregnancy: difficult choice of delivery from a case and a review of the literature].
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 (2000mL) secondary to vaginal lacerations requiring sutures and supplemented by arterial embolization. A multidisciplinary approach is required at delivery.
Département de gynécologie-obstétrique, hôpitaux universitaires de Strasbourg, 19, rue Louis-Pasteur, BP 120, 67300 Schiltigheim cedex, France.
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Koch
Département de gynécologie-obstétrique, hôpitaux universitaires de Strasbourg, 19, rue Louis-Pasteur, BP 120, 67300 Schiltigheim cedex, France. Electronic address: germain.aissi@chru-strasbourg.fr.
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Aïssi
Département de gynécologie-obstétrique, hôpitaux universitaires de Strasbourg, 19, rue Louis-Pasteur, BP 120, 67300 Schiltigheim cedex, France.
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Gaudineau
Département de gynécologie-obstétrique, hôpitaux universitaires de Strasbourg, 19, rue Louis-Pasteur, BP 120, 67300 Schiltigheim cedex, France.
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Sananes
Département de gynécologie-obstétrique, hôpitaux universitaires de Strasbourg, 19, rue Louis-Pasteur, BP 120, 67300 Schiltigheim cedex, France.
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Murtada
Département de gynécologie-obstétrique, hôpitaux universitaires de Strasbourg, 19, rue Louis-Pasteur, BP 120, 67300 Schiltigheim cedex, France.
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Favre
Département de gynécologie-obstétrique, hôpitaux universitaires de Strasbourg, 19, rue Louis-Pasteur, BP 120, 67300 Schiltigheim cedex, France.
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Nisand
D000328:Adult; D036861:Delivery, Obstetric; D005260:Female; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000150:complications; D006473:Postpartum Hemorrhage / Q000209:etiology / Q000628:therapy; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular
Anomalies vulvo-vaginales; Delivery; Embolisation; Embolization; Hémorragie du post-partum; Klippel-Trenaunay syndrome; Post-partum haemorrhage; Syndrome de Klippel-Trenaunay; Voie d’accouchement; Vulvovaginal anomalies