Background and Aims Lymphangioleiomyomatosis (LAM) is a rare, progressive, idiopathic disease, affecting almost exclusively women of reproductive age. Mainly involves the pulmonary, renal, and lymphatic systems, with an increased risk of complications during pregnancy with significant anaesthetic implications. Information regarding obstetric anaesthesia management of patients affected by this condition is limited.

Methods The authors describe the successful use of neuraxial anaesthesia in a 39-year-old female patient (72kg, 1.60m) at 39 weeks pregnancy with pulmonary and abdominal LAM proposed for elective caesarean section. LAM was incidentally diagnosed two years earlier during in vitro fertilization treatments and was responsible for ICU admission at 20 weeks of pregnancy due to massive bleeding from a 22x18cm renal angiomyolipoma. After multidisciplinary team discussion, and considering the patient´s hematological recovery and currently asymptomatic status, a combined spinal-epidural anaesthesia was performed according to our protocol with an initial subarachnoid injection of 1.6 ml of 0,75% ropivacaine and 2.5 µg of sufentanil. The epidural catheter was left in place for potentially prolonged surgery and postoperative multimodal analgesia.

Results General anaesthesia with positive pressure ventilation was successfully avoided. The procedure was uneventful with no need for vasopressors and postoperative recovery was unremarkable, with the patient being discharged two days after the procedure.

Conclusions Combined spinal-epidural anaesthesia may be a safe approach for elective caesarean section in patients with LAM under similar circumstances.