Obstetric anesthetic management of Klippel–Trénaunay syndrome: a case report

We report a 39-year-old gravida 2 para 1 woman with Klippel–Trénaunay syndrome (KTS) who presented at 39 weeks' gestation for scheduled repeat cesarean delivery. KTS was identified during her second pregnancy, prompting multidisciplinary evaluation involving anesthesiology, obstetrics, neurosurgery, and neonatology. Preoperative MRI of the entire spine excluded neuraxial vascular malformations, while brain imaging identified an incidental 2-mm basilar tip aneurysm that was deemed nonsurgical by neurosurgery. Coagulation studies were reassuring. After risk-benefit discussion, the patient elected spinal anesthesia, which was performed without complication. Intraoperative bleeding and uterine atony were managed medically without requiring blood transfusion. This case demonstrates that neuraxial anesthesia may be considered in carefully selected parturients with KTS when neuraxial vascular malformations have been excluded by imaging and coagulation status is reassuring. The comparison of this patient's two deliveries—one without and one with knowledge of the diagnosis—illustrates that the principal value of recognizing KTS lies not necessarily in altering the anesthetic technique, but in enabling comprehensive perioperative risk stratification, multidisciplinary planning, and targeted hemorrhage preparedness.