Klippel-Trenaunay syndrome and pregnancy

General recommendations on how to deal with pregnancy in patients with Klippel-Trenaunay syndrome (KTS) are rare. We describe the case of a 32-year-old female with KTS, involving the head and the left arm and leg, delivering a healthy female child, and are reviewing the recent literature. The risk to deliver an ill child is low in women with KTS. At the end of the 1st trimester a sonographic investigation can exclude angiodysplastic alterations of the fetus. If the fetus shows changes compatible with KTS, a termination can be discussed because the risk of fatal complications after delivery is high. During pregnancy the careful monitoring of coagulopathic disorders is necessary. Prior to delivery an MR-scan may be useful to detect angiodysplastic vascular structures next to the spinal cord, pelvic structures or the lower abdominal wall, which might complicate peridural anesthesia or caesarean section.