2018 FSA Posters

P044: TO INTUBATE OR NOT TO INTUBATE? THE CASE OF ANESTHESIA IN A PATIENT WITH STIFF PERSONS SYNDROME AND VAGAL MEDIATED SINUS PAUSES
Joseph LaGrew II, MD, Steve R Robicsek, MD, PhD; University of Florida Department of Anesthesiology

Introduction/Background: Stiff person syndrome (SPS) is a rare syndrome, first described in 1956,1 and  characterized by mild to severe muscle rigidity, stiffness and spasm. Symptoms can progress to cause severe disability. The pathophysiology of this disease has yet to fully elucidated2 and therapy is centered on symptomatic treatment with around GABAergic agonists, with IVIG therapy reserved for more severe cases. Previous case reports have described delayed emergence and weakness requiring prolonged ventilation on emergence after general anesthesia with volatile anesthetic agents and neuromuscular blocking agents in patients with SPS.2,3,4 Though subsequent cases have described uneventful general anesthetics,4,5,6 airway management and anesthetic agents to be used should be considered carefully in these patients. 

Case:  A 58 year-old female with a past medical history significant for SPS and multiple sinus pauses presented for mastectomy. The pauses occurred while the patient was intubated in the ICU for hypoventilation and required temporary pacemaker placement. Twenty four hour Holter monitor was negative for further events and cardiology evaluation diagnosed them as vagal-mediated. Recommendations prior to surgery included availability of atropine during the procedure but no permanent pacemaker placement or additional workup was pursued. For her SPS, the patient took scheduled diazepam, tylenol, gabepentin as well as IVIG treatments every 3 weeks.  

Given her history of sinus pauses while intubated and SPS, a total intravenous (TIVA) general anesthetic with a laryngeal mask airway (LMA) was planned. She was induced with a propofol bolus followed by an infusion after the return of spontaneous respirations. Fentanyl was used during the case for analgesia. The patient required pressure support but maintained tidal volumes greater than 4mL/kg for the entire case. She emerged from anesthesia and was observed for an extended period in the post operative recovery area prior to discharge to monitor her respiratory status.  She remained in sinus rhythm throughout the case and recovery period.

Discussion/Conclusion:  We describe here the successful use of an LMA and pressure support with TIVA for general anesthesia for this patient with stiff person syndrome. Given the rarity of stiff person’s syndrome and the paucity of published reports on anesthetics given to patients like the one described here, the risk of respiratory complications could not be clearly defined. The patient had no antibodies to glutamic acid decarboxylase; however, her symptoms and medication regimen corresponded to a more severe disease state. Although prior case reports have described using volatile agents at reduced doses from 0.2-0.4% of isoflourane with remifentanyl3 and 0.5%-1.7% sevoflourane with propofol and fentanyl4 with no significant weakness, delayed emergence or respiratory complications, volatile agents have been associated with adverse effects previously. No cases to our knowledge have described these reactions with Propofol based anesthetics. Consideration was given to general endotracheal anesthesia, but the patient’s recent history of sinus pauses while intubated made LMA placement preferable.

This patient with a past medical history of STS and vagal-mediated sinus pauses tolerated a TIVA general anesthetic without adverse effects.