The patient was a 19-year-old female with a height of 163.5 cm and a weight of 21.5 kg. She was born after 38 weeks of gestation and weighed 2624 g without asphyxia, but was diagnosed as having severe MMA at 3 days of age due to deterioration of general status, and the presence of metabolic acidosis and hyperammonemia. Complicating her cerebral palsy, she was repeatedly hospitalized due to acidosis and seizures. Since 2009, her renal function gradually worsened, and in 2017, plasma creatinine (Cr) was found to be above 4.0 mg/dl. In 2018, the patient was admitted to the emergency room due to a deterioration of general status from infection, and an operation for a continuous ambulatory peritoneal dialysis catheter placement was scheduled.
Preoperative venous blood gas analysis did not indicate metabolic acidosis: pH, 7.432; PCO2, 42.1 mmHg; PO2, 38.2 mmHg; and base excess (BE), 2.9 mEq/l. Blood results showed anemia and renal failure: hemoglobin (Hb), 6.1 g/dl; blood urea nitrogen (BUN), 56.0 mg/dl; Cr, 4.04 mg/dl; and estimated glomerular filtration rate (eGFR), 13 ml/min/L.
General anesthesia was combined with a peripheral nerve block. No premedication was administered. After the patient entered the operating room, electrocardiography, saturation of percutaneous oxygen, and noninvasive arterial pressure were monitored. Anesthesia was induced using thiamylal (4.6 mg/kg), remifentanil (0.4 mcg/kg/min), and rocuronium (0.9 mg/kg). After tracheal intubation, the patient underwent an ultrasound-guided bilateral rectus sheath block with 0.2% ropivacaine (30 ml). Anesthesia was maintained with a gas mixture of oxygen and air (FiO2 0.4), 1.0 minimum alveolar anesthetic concentration sevoflurane (1.2–2%), and remifentanil (0.2–0.4 mcg/kg/min).
Intraoperative arterial blood gas values were as follows: pH, 7.369; pCO2, 32.9 mmHg; pO2, 182 mmHg; BE, − 5.9 mEq/l; Na, 140 mmol/l; K, 3.8 mmol/l; and Cl, 104 mmol/l. Glucose preparation was used to prevent hypoglycemia while maintaining a concentration of 100–200 mg/dl to ensure optimal dosing. The preoperative blood sugar level was 113 mg/dl. Hypotonic fluid (2.5% glucose) was used in the perioperative period. Blood sugar level declined to 82 mg/dl during surgery, and 5% glucose was administered. The level was 120 mg/dl at the end of the surgery. To avoid perioperative accumulation of methylmalonic acid, the temperature was maintained above 36.0 °C with a heating device, and clindamycin was administered intravenously. Throughout the surgery, hemodynamic parameters were stable, and the operation was over without using vasopressor agents. Extubation was performed after intravenous administration of sugammadex (4 mg/kg). The duration of surgery was 2 h and 10 min, and that of anesthesia was 3 h and 30 min. Postoperatively, the patient did not experience pain at rest; however, she experienced mild pain during movement. The patient did not use any rescue analgesic agents in the postoperative period. Additionally, no evidence of metabolic acidosis or hyperammonemia (NH3, 29 μg/dl) was observed. Continuous ambulatory peritoneal dialysis was started 10 days after the surgery, and the patient was discharged after 36 days.