The patient provided informed consent for the procedure and the publication of anonymized case details presented in this report.
A 52-year-old woman (height, 155 cm; weight, 64 kg) with HAE was scheduled for laparoscopic cholecystectomy. An immunology clinic confirmed the presence of HAE at the age of 39, and the patient was diagnosed with type-I HAE according to genetic testing. Tranexamic acid 1000 mg and danazol 100 mg had been administered daily for long-term symptom control. In addition, prednisolone 4 mg was orally administered due suspected systemic lupus erythematosus. Preoperative blood tests revealed mild liver dysfunction (total bilirubin, 1.9 mg/dL; aspartate aminotransferase, 41 IU/L; alanine aminotransferase, 75 IU/L). Chest X-ray imaging, electrocardiography, and respiratory function tests were normal.
Previously, the patient had repeatedly developed signs of angioedema at a frequency of once every 2–3 months. Nasal obstruction, edema around the cervical region, or a feeling of discomfort in the throat due to angioedema were reported, necessitating emergency infusion with C1-esterase inhibitor concentrate (Berinert® P, CSL Behring, Germany) to relieve the symptoms.
During preoperative planning of anesthesia for the current surgery, avoiding tracheal intubation to reduce the risk for tracheal angioedema was considered. Wall-lifting laparoscopic surgery was scheduled so that surgical stress could be managed using combined spinal-epidural anesthesia.
Danazol, tranexamic acid, and prednisolone were administered orally on the morning of the day of surgery; in addition, 1500 U of C1-esterase inhibitor was administered intravenously 2 h before the surgery.
An epidural catheter was inserted through the intervertebral space at T9/10, and spinal anesthesia was instilled using 0.5% hyperbaric bupivacaine 3 mL and fentanyl 15 μg via the L3/4 intervertebral space. In addition, 5 mL of 0.375% levobupivacaine solution and 3 mg of morphine were administered via the epidural catheter. Anesthesia was achieved below the T4 cutaneous level. In accordance with the patient’s request, she was sedated by intermittent administration of midazolam (7 mg total) and continuous infusion of propofol (0.8–1.6 mg/kg/h). Oxygen was administered at 4 l/min via a face mask, and peripheral oxygen saturation was maintained at 100% through the operation. Blood pressure was maintained by intermittent administration of phenylephrine. A single-hole, Nishii-type lifting laparoscopic surgery, without pneumoperitoneum (i.e., gasless) was completed uneventfully. The operation duration was 2 h and 45 min, and anesthesia duration was 3 h and 20 min. After confirming the absence of complications in respiratory status, she was returned to the ward and discharged uneventfully on postoperative day 3.