2017 FSA Posters

P007: RAPID INTUBATION USING ALFENTANIL IN A PEDIATRIC PATIENT WITH A MITOCHONDRIAL DISORDER
Wendy L Leavitt, MD, Sonia Mehta; University of Florida

Introduction: Mitochondrial disorders are becoming more frequently encountered in the pediatric operating room. Many of the commonly administered medications used for the induction of anesthesia have been shown to interfere with the process of oxidative phosphorylation. In addition, neuromuscular blockers are often contraindicated secondary to the myopathies present in these patients. Pediatric patients with mitochondrial disorders mandate a thorough anesthetic plan to prevent metabolic decompensation leading to potential acidosis and encephalopathy. This case report outlines the induction of anesthesia in a 4-year-old female with a complex 1 mitochondrial disorder with severe gastroparesis and esophageal dysmotility using alfentanil and volatile anesthetic.

Case Presentation: 4-year-old female with a diagnosis of a complex 1 deficiency mitochondrial disorder presented for placement of a temporary gastric stimulator. Her presenting symptoms included muscle weakness, limited endurance and myalgias with prolonged activity, gastroparesis, esophageal dysmotility, staring spells, apnea, dysmetria, and a broad based gait. She had a gastrostomy tube in place and was unable to tolerate tube feeds secondary to gastroparesis and vomiting.  

For her first placement, the patient received 2mg IV midazolam. General anesthesia was induced with inhaled sevoflurane, dexmedetomidine 0.5mg/kg IV loading dose followed by a 0.2-1mcg/kg/hr infusion, ketamine 1mg/kg IV, and fentanyl 0.5mg/kg IV. The vocal cords were sprayed with 40mg lidocaine prior to endotracheal intubation. 10% dextrose was infused throughout the case. Her intraoperative course was uneventful with successful placement of the temporary stimulator. Total case time from induction to extubation was 48 minutes. Post-operatively, her mother noted the patient was excessively sleepy. The patient was discharged from the PACU 120 minutes following extubation in the OR.

The patient presented about a month later for re-implantation of the temporary stimulator. One of the leads had come loose.  She received 2mg IV midazolam. General anesthesia was induced with inhaled sevoflurane and 25mcg/kg IV alfentanil. The vocal cords were sprayed with 30mg lidocaine prior to endotracheal intubation. 10% dextrose was infused throughout the case. Again, her intraoperative course was uneventful. Total case time from induction to extubation was 30 minutes. Post-operatively, the mother noted the patient quickly returned to her baseline mental status and she was discharged from the PACU 55 minutes following extubation.

Discussion: This case presented a unique set of requirements. First, the choice of anesthetic induction drugs was limited by the patient’s underlying mitochondrial disorder, eliminating the use of propofol as a sole agent to achieve intubating conditions, as well as succinylcholine secondary to her myopathy. Second, she required endotracheal intubation to facilitate the procedure and in the setting of severe gastroparesis and esophageal dysmotility. Third, she was presenting for a short procedure that would have been prolonged given the use of non-depolarizing neuromuscular blockers. Finally, while she had tolerated the procedure previously without lasting anesthetic complications, she did require an extended PACU stay secondary to prolonged sedation. Given this, an anesthetic with a shorter recovery time would facilitate resumption of oral intake, decreasing her risk of developing a metabolic acidosis, as well as timely patient discharge to home.