Recommended Citation
Dziemianko J, Kim M, Griffith J. Trapped under anesthesia: Pseudocholinesterase deficiency in liver cirrhosis. Presented at: Midwest Anesthesia Residents Conference (MARC); April 26, 2025; Indianapolis, IN.
Presentation Notes
Presented at: Midwest Anesthesia Residents Conference (MARC); April 26, 2025; Indianapolis, IN.
Abstract
Introduction: Pseudocholinesterase (PChE), also known as butyrylcholinesterase, plasma cholinesterase or serum cholinesterase, is an enzyme produced in the liver and is responsible for the hydrolysis of choline-based esters, namely the neuromuscular blockade (NMB) drugs succinylcholine and mivacurium. In the presence of normal PChE levels, 90-95% of succinylcholine is inactivated before it reaches the neuromuscular junction (NMJ), resulting in paralysis expected to last about 10-15 minutes [1,2]. PChE deficiencies are exceedingly rare, affecting 0.02-0.03% of the population, and can prolong paralysis by minutes to hours [1]. While we often talk about the genetic etiology, there are many acquired causes to be aware of, such as malignancy, major burns, pregnancy, malnutrition, many different medications, and renal and liver disease. Case Presentation: We present the case of a 55-year-old male with PMHx of liver cirrhosis, undergoing microlaryngoscopy and bronchoscopy for the biopsy of a vocal cord mass. He was not taking any medications and denied any personal or familial complications with anesthesia. The patient was given midazolam pre-operatively, and then general anesthesia was induced with lidocaine, fentanyl, propofol, and succinylcholine. The procedure finished within 20 minutes of induction, and as the sevoflurane was weaned off, our patient failed to breathe spontaneously. On exam, his pupils were constricted, and naloxone was given, but he did not demonstrate any further emergence. Neuromuscular monitoring was employed, and a train-of-four (TOF) count was attempted in different areas and with different devices; however, his TOF count was 0/4, and he had no post-tetanic twitch response. Flumazenil was administered to rule out midazolam as a contributor to the patient’s status; however, the patient remained motionless. He was brought to the post-anesthesia care unit (PACU) intubated and was placed on a ventilator to allow recovery. After a few hours in PACU, he was extubated successfully and went home that day. Conclusion: When planning anesthesia in patients, it’s widely accepted that succinylcholine and mivacurium should be avoided in patients with known genetic variants of PChE deficiency. However, anesthesiologists should also be familiar with the acquired etiologies of PChE deficiency, such as liver cirrhosis, and consider either avoiding or reducing the doses of succinylcholine and mivacurium. Neuromuscular monitoring is warranted in all patients regardless of NMB choice, and an absence of twitches after administration of succinylcholine should alert the anesthesiologist to a possible PChE deficiency. Approximately 50% of patients with pseudocholinesterase (PChE) deficiency perceive their emergence from anesthesia with residual neuromuscular blockade as awareness under general anesthesia, often describing it as a traumatic experience with potential long-term psychological effects [1]. Management of PChE deficiency is primarily supportive, emphasizing measures to keep the patient calm and comfortable while awaiting the passive diffusion of the NMB from the neuromuscular junction. Finally, patients should receive education about their condition and undergo quantitative enzyme testing to confirm the diagnosis. 1. Robles, A. et al. (2019) The American Journal of the Medical Sciences 357(3):263-267 2. Andersson, M. L. et al. (2019) Anaesthesia 74(4):518-528
Type
Oral/Podium Presentation
Affiliations
Advocate Illinois Masonic Medical Center