We present a case report of a patient with MAS. Multiple system atrophy (MAS) is a rare disease: prevalence is estimated at 4.6 cases per 100,000 people. This neurodegenerative disease is characterized by combinations of dysautonomia, cerebellar dysfunction, and parkinsonism. MAS poses a challenge to anesthesiologists because of the potential complications of impaired cardiovascular reflexes, life threatening changes in blood pressure, as well as vocal cord paralysis. Cases of MAS are underreported in anesthesia literature; this report would help in deciding future perioperative management of these patients. Our patient was 58-year-old male with MAS, autonomic failure supported by pacemaker implantation, midodrine, fludricortisone, and pyridostigmine. Patient was to undergo laparoscopic cholecystectomy for acute cholecystitis. He was wheelchair bound, and suffered from bilateral posterior neck spasms with limited neck range of motion. We managed our patient in light of the presenting symptoms. There were no conclusive studies on preference of type of anesthesia for MAS patients; we chose general anesthesia based on the nature of laparoscopic surgery and patient cooperation. To ensure perioperative hemodynamic stability, we adequately hydrated the patient, and optimized pharmacologic treatment: he continued his home medications and received a dose of pyridostigmine pre-operatively. We induced with propofol and remifentanil; as MAS is a neurodegenerative disease, patient was wheelchair bound, and taking pyridostigmine—which makes neuromuscular blockers unreliable—we avoided standard relaxants for induction. Due to our patients’ neck rigidity, we intubated using asleep fiberoptic technique. After discussion with the surgeons on use of cautery and surgery site, we kept pacemaker function intact. Intraoperatively, our patient experienced fluctuations in blood pressure, the first after induction. These were managed with fluid boluses and small doses of phenylephrine, as MAS patients typically have exaggerated responses to vasopressor drugs. Patient was extubated successfully in the operating room, with no reported problems post-operatively, particularly those that afflict this population, such as vocal cord paralysis, stridor, or apnea. Based on this case management, it seems that MAS patients can undergo general anesthesia for laparoscopic surgery with no significant perioperative complications. In the future we suggest routine use of a fiberoptic bronchoscope to intubate this population for better view of vocal cord anatomy. We also would consider etomidate as an alternative induction agent to avoid potential blood pressure lability post induction.
