A 56-year-old male patient (weight, 82 kg; height, 168 cm) with glutaric acidemia type 2 was scheduled for laparoscopic cholecystectomy for cholecystolithiasis. He had a history of rhabdomyolysis with lumbago and brown urine as initial symptoms at the age of 44 years. At the age of 52 years, he was diagnosed with glutaric acidemia. He did not receive nutrition therapy, including fat restriction; however, he developed rhabdomyolysis approximately once a year and needed fluid therapy. His previous medical history included diabetes mellitus requiring medical treatment, cerebral infarction, and bronchial asthma. Preoperative examination for laparoscopic cholecystectomy led to a diagnosis of angina pectoris with three-vessel disease. Consequently, an off-pump coronary artery bypass grafting (CABG) was planned preceding laparoscopic cholecystectomy.
Off-pump CABG
The preoperative serum creatine kinase (CK) and creatinine levels were within the normal range. Metformin hydrochloride, an oral hypoglycemic agent, was discontinued 2 days before the surgery. General anesthesia was induced using remifentanil (0.3 μg/kg/min) and midazolam (6 mg), with rocuronium (70 mg) administered as a muscle relaxant. Anesthesia was maintained using midazolam (0.03 mg/kg/h), dexmedetomidine (0.4 μg/kg/h), and remifentanil (0.1–0.5 μg/kg/min) with O2-air mixture. In addition to the standard intraoperative monitoring, direct radial arterial pressure, pulmonary artery pressure, transesophageal echocardiography, and electroencephalogram were monitored. Moreover, the arterial blood pH, glucose, serum CK, serum and urine myoglobin, and lactate levels were measured at 1-h intervals. Administration of 2 mg/kg/min glucose using 10% glucose solution was started from midnight of the day before surgery. It was continued throughout the operation and postoperatively in the intensive care unit (ICU) using 50% glucose solution. Continuous insulin administration was started when intraoperative blood glucose exceeded 200 mg/dL. The operation and anesthesia times were 8 h 2 min and 9 h 49 min, respectively. The myoglobin level started to increase just after surgery. In the ICU, the body temperature was 36.7°C, and postoperative shivering was not observed. Moreover, the serum potassium level was 4.0 mEq/L. The total in-out balance of volume was 2294 ml: intravenously infused fluid (crystalloid and colloids), 4260 mL (2260 mL and 2000 mL, respectively); cell saver autotransfusion, 1600 mL; estimated blood loss, 592 g; cell saver output, 2786 mL; and urine output, 178 mL. The patient was sedated using midazolam and dexmedetomidine; subsequently, postoperative analgesia was maintained through continuous fentanyl infusion. There was an increase in the Mm-CK levels to a maximum value of 2328 U/L, while the serum potassium level increased to 5 mEq/L on postoperative day (POD) 2. Further, the serum myoglobin level increased to a maximum value of 635.8 ng/mL on POD 4. Urine volume decreased to approximately 0.2–0.5 mL/kg/h, and serum creatinine increased to >4.0 mg/dL, which indicated acute renal failure. Consequently, continuous hemodiafiltration was started and continued up to POD 25 (Fig. 1a).
Laparoscopic cholecystectomy
The patient underwent laparoscopic cholecystectomy 316 days after undergoing CABG. General anesthesia was induced using midazolam (3 mg), remifentanil (0.2 μg/kg/min), and rocuronium (60 mg). Transversus abdominis plane block was performed with an ultrasound linear transducer using 40 mL of 0.25% levobupivacaine after anesthesia induction. Anesthesia was maintained using sevoflurane (1.5%) and remifentanil (0.03–0.15 μg/kg/min). The operation and anesthesia times were 1 h 51 min and 3 h 30 min, respectively. The total infused fluid and urine volumes were 1170 mL and 100 mL (0.36 mL/kg/h), respectively. The glucose infusion rate was 2 mg/kg/min using 10% glucose solution, was started from midnight of the day before surgery, and increased to 4 mg/kg/min using 50% glucose solution when introducing anesthesia. There was a slight increase in the postoperative serum CK and myoglobin levels at a maximum value of 534 U/L and 213 ng/mL on POD 1, respectively. The increased CK and myoglobin levels were promptly normalized. The serum creatinine level was normal, and renal function was not impaired (Fig. 1b).