Anesthesia for cesarean delivery in a patient with Klippel-Trenaunay syndrome

Abstract Introduction: Klippel-Trenaunay syndrome is a rare congenital vascular disease characterized by cutaneous hemangiomas, varicosities, and limb asymmetry, which may evolve with coagulation disorders and hemorrhage as those more frequent complications in pregnant patients. Pregnancy is not advised in women with this syndrome due to increased obstetrical risk. Case report: Female patient, 29 years old, 99 kg, 167 cm, BMI 35.4 kg.m−2, physical status ASA III, with 27 weeks of gestational age and diagnosis of Klippel-Trenaunay syndrome. She was admitted to attempt inhibition of preterm labor. As manifestations of Klippel-Trenaunay syndrome, the patient presented with cerebral and cutaneous hemangioma mainly in the trunk and lumbar region, paresis in the left upper and lower limbs, and limb asymmetry requiring the use of a walking stick. Physical examination revealed absence of airway vascular malformations and Mallampati class 3. Laboratory tests were normal and abdominal angiotomography showed irregular uterus, with multiple varices and vessels of arterial origin and bilateral periadnexal varices. She evolved with failure in preterm labor inhibition, and cesarean section under total intravenous anesthesia was indicated. Monitoring, central and peripheral venous access, radial artery catheterization, and diuresis were secured. Cesarean section was performed with median incision and longitudinal uterine body section for fetal extraction. Two episodes of arterial hypotension were seen intraoperatively. The postoperative evolution was uneventful. The choice of anesthesia was dependent on the clinical manifestations and the lack of imaging tests proving the absence of neuraxial hemangiomas.