Anesthetic management of a patient with Huntington's chorea -A case report-
A 51-year-old male, 50 kg in weight who had been diagnosed with Huntington's chorea at the 41 years-of-age was admitted for an emergency laparoscopic hernia repair. On physical examination, the patient displayed choreic movements in all limbs, swallowing dysfunction and excessive oral secretions. Laboratory values were all within normal limits, except for leukocytosis. A chest radiograph did not show any abnormal finding and the electrocardiogram (ECG) revealed left ventricular hypertrophy. The patient was transferred to the operating room without being pre-medicated. Routine monitoring included pulse oximetry, ECG, noninvasive blood pressure and end-tidal carbon dioxide and gas monitoring. To check the depth of anesthesia during the operation, A BIS sensor (BISquatro™, Aspect® Medical Systems, Inc., USA) was attached and measurements were taken when the signal quality index (SQI) was above 95. Muscle relaxation was monitored using an electrical stimulator (Relaxograph®, Datex Relaxograph, Finland) by applying the train of four (TOF) on the wrist's ulnar nerve and measuring the contraction of the adductor pollicis muscle. To avoid possible pulmonary aspiration, awake fiberoptic intubation was planned by an expert. The procedure and need to be intubated while awake was explained to the patient. The base of the tongue and pharyngeal walls were anesthetized with 10% xylocaine spray and 4% lidocaine was injected trans-tracheally to anesthetise the trachea. The fiberoptic bronchoscope (LF-TP, Olympus, Japan) was checked and loaded with a cuffed endotracheal tube with an inner diameter (ID) of 7.5 mm. The vocal cords were visualized and the tube was passed over the fiberscope into the trachea without difficulty. After confirmation of tracheal intubation by viewing the tube tip inside the trachea using fiberoptics, tube fogging, the inability of the patient to vocalize and end-tidal carbon dioxide, anesthesia was induced with an intravenous computer-controlled infusion pump (Orchestra® Base Primea, Fresenius Vial, France) of 2% propofol with a target of 4 µg/ml and remifentanil with a target of 4 ng/ml, and an intravenous bolus of 0.5 mg/kg rocuronium. The radial artery cannula was inserted for invasive blood pressure monitoring. The TOF ratio was zero after induction.\nDuring the surgery, the concentrations of propofol and remifentanil were regulated according to the BIS and mean arterial pressure. Anesthesia was maintained with a TCI of propofol and adequate concentrations to maintain the BIS between 40 and 60. The mean arterial pressure and heart rate remained within normal limits during the procedure. Four twitches were detected on TOF at 60 and 90 minutes after induction and rocuronium 5 mg was administered each time. The surgery lasted 105 minutes. Ondansetron 4 mg was administered to reduce postoperative nausea and vomiting.\nThe infusions of remifentanil and propofol were discontinued at the end of the procedure and a neuromuscular blockade was reversed with pyridostigmine 15 mg and glycopyrrolate 0.4 mg. Spontaneous respiration and consciousness returned after 9 minutes, at which time the endotracheal tube was removed. The postoperative course was uneventful and there were no complications such as nausea, vomiting or shivering. The patient was transferred to a general ward and discharged from the hospital after 3 uneventful days.