Pregnancy in Klippel-Trenaunay syndrome: review of literature

Abstract

Klippel-Trenaunay syndrome is a rare congenital anomaly of unknown etiology, characterized by capillary and venous malformations, and hypertrophy of bone and soft tissue. Cases of pregnancy in women with Klippel-Trenaunay syndrome are rare and usually associated with adverse perinatal outcomes. Objective: To resume knowledge about pregnancy in the Klippel-Trenaunay syndrome as well as the syndrome so these patients be readily recognized and receive proper care to have a successful pregnancy outcome. Method: It was performed a review of the English literature, in the MEDLINE and Cochrane Library bases until October 2009. Results: Only 17 case reports of pregnancy in women with Klippel-Trenaunay syndrome have been found. The evolutions of pregnant were variable and involve adverse events, with worsening of previous manifestations, bleeding, disseminated intravascular coagulation, thromboembolic events and pain. Conclusion: Pregnancy increases the risk of adverse events. Knowledge of the syndrome and multidisciplinary prenatal, intra and postpartum care are essential to successful outcome of pregnancy.