Peripartum management of a parturient with Klippel-Trénaunay syndrome

Klippel-Trénaunay syndrome is a rare congenital disorder affecting the vascular and lymphatic systems. The clinical presentation can vary widely, but the syndrome is broadly characterised by capillary, venous and lymphatic malformations as well as limb hypertrophy. We present the case of a 35-year-old parturient who underwent an emergency caesarean section for suspected fetal distress, and describe the anaesthetic management during the peripartum period. Only a small number of similar cases have been described, and the multisystem nature of the condition presents several challenges to both the obstetric and anaesthetic management. The major points of concern to the anaesthetist are haematological, with a tendency to both abnormal bleeding and clotting disorders, compounded by vascular malformations which may present anywhere in the body including the epidural space and airway. Other considerations relate to limb hypertrophy and spinal abnormalities, as well as pulmonary and ocular sequelae and chronic pain. Strategies for safe patient management include early multidisciplinary involvement, and assessment of the presence and extent of any vascular anomalies with advanced imaging techniques. The risk of significant blood loss can be mitigated with antifibrinolytic and uterotonic medication as well as cell salvage, with treatment carefully balanced against the concurrent risk of thrombosis.