A 15-year-old female (height, 140 cm; weight, 34 kg) was scheduled for endoscopic treatment of vesicoureteral reflux using dextranomer. The patient had been diagnosed with PHS as a neonate. She had undergone hypothalamic tumor resection as a neonate, repair of imperforate anus at 8 months of age, and gamma knife treatment for hypothalamic tumor recurrence at 11 years of age. Prolonged intubation (2–4 weeks) had occurred after the first and second surgeries due to difficult extubation. Respiratory failure after tracheal extubation occurred several times, without clear reasons. The reason why the difficult extubation had happened was not clear. The anesthetic record showed that tracheal intubation was difficult due to micrognathia and bifid epiglottis. The largest uncuffed endotracheal tube that could be inserted into the trachea when the patient was 11 years old was a 4.0-mm ID. The patient had undergone a gastrostomy due to chronic aspiration. She was also taking medications for hypopituitarism and seizures. She had multiple allergies including egg, latex, and aminophylline. Pre-anesthetic examination of the patient showed that she had micrognathia. Stridor was heard over the trachea. We judged that it would be difficult to obtain the patient’s cooperation with regional anesthesia, as she had mild mental retardation and hearing difficulty. Hence, we selected general anesthesia for the surgery. The patient was planning to receive resection of residual hypothalamic tumor in a few years after the endoscopic treatment of vesicoureteral reflux.
The major anesthetic concern was airway management. Pre-anesthetic problems included (1) difficult intubation due to micrognathia and bifid epiglottis, (2) predisposition to aspiration, and (3) airway stenosis due to prolonged intubation during infancy. We had three options to secure the patient’s airway during the operation: tracheal intubation using video laryngoscopy or fiberoptic bronchoscopy, SAD, or surgical tracheotomy. We elected to use a SAD. Because we wanted to investigate the patient’s airway closely, we decided to observe the vocal cords using a muscle relaxant to minimize the risk of laryngospasm. The neurosurgeons treating the patient were planning to perform resection of the residual brain tumor in a few years. Hence, it seemed important for us to check the patient’s airway before the residual brain tumor resection.
Preoperatively, the patient was fasted for 8 h with intravenous hydration. Metoclopramide (10 mg) and an H2-blocking agent (ranitidine 50 mg) were administered intravenously to minimize the risk of aspiration. The equipment for difficult airway management was prepared, and two anesthesiologists were present in the operation room. On arrival at the operating room, pulse oximetry, electrocardiography, and noninvasive blood pressure monitoring were established. General anesthesia was induced with intravenous propofol (50 mg) and fentanyl (25 μg). Mask ventilation was successful. Rocuronium (20 mg) was administered to facilitate positive pressure ventilation. Because we needed to investigate the patient’s airway for future surgery, we observed the epiglottis and vocal cords using a McGRATH MAC video laryngoscope with an X-blade (Medtronic, Minneapolis, MN) and confirmed the bifid epiglottis (Fig. 1). A laryngeal mask airway (size 2.5) was inserted, and the cuff was inflated with air (10 ml). We evaluated the vocal cords and trachea using fiberoptic bronchoscopy and visualized the subglottic stenosis (Fig. 2, video). The subglottic stenosis was the reason that only a small-sized endotracheal tube could be inserted when the patient was 11 years old. Anesthesia was maintained by sevoflurane 2.5% and remifentanil (0.2–0.5 μg/kg/min) and intermittent doses of rocuronium. The operation time was 27 min. For postoperative analgesia, acetaminophen (500 mg) was administered intravenously. After confirming spontaneous recovery of a train-of-four ratio to 100%, we decided not to antagonize the rocuronium, as the patient had multiple allergies and was considered high risk for sugammadex allergy. The laryngeal mask airway was removed after recovery of spontaneous breathing. The postoperative course was uneventful, and the patient was discharged the day after the procedure.
Additional file 1: Video. Subglottic stenosis was observed using fiberoptic bronchoscopy. (MP4 88,406 kb)