Klippel Trenaunay Weber syndrome in pregnancy

Abstract

Klippel Trenaunay Weber syndrome (KTW) is a rare disorder, which occurs, in one in 27500 live births.1 It is a congenital neuroectodermal disorder1 and is characterized by the triad of symptoms such as port wine stain, bone and soft tissue hypertrophy and varicose veins. It is also associated with arteriovenous malformation.2 Due to the complexity of this disease, it is a real challenge to the anaesthetist and the obstetrician. We report a case of a KTW syndrome patient in her first pregnancy who was transferred to us from a district general hospital for the delivery. She had all the features along with multiple vascular anomalies and thrombocytopaenia. She underwent an elective caesarean section under general anaesthesia. Her intraoperative period was uneventful. At her post-operative day one she had a sudden cardiac arrest due to massive pulmonary embolism. This case highlights the high risk of thromboembolic events in patients with KTW syndrome.