Butyrylcholinesterase deficiency and its clinical importance in anaesthesia: a systematic review

Abstract

Summary Butyrylcholinesterase deficiency prolongs the effects of the drugs it degrades; succinylcholine and mivacurium. Existing literature on butyrylcholinesterase deficiency is dominated by genetic and biochemical studies. We searched MEDLINE, Embase, Web of Science and Biosis to systematically review the causes and clinical consequences of butyrylcholinesterase deficiency. We considered outcomes clinically relevant if neuromuscular blockade, induced by succinylcholine or mivacurium, was assessed using clinical criteria or neuromuscular monitoring. We included 66 studies: 25 randomised controlled trials; 13 clinically controlled trials; 26 prospective observational studies; 1 retrospective study; and 1 qualitative study. Data heterogeneity precluded quantitative synthesis. Studies described genetic, physiological, acquired or pharmacologically induced causes of butyrylcholinesterase deficiency. The prolongation of neuromuscular blockade by butyrylcholinesterase deficiency was most pronounced with homozygosity of a genetic variant, but other more common factors included increasing age, pregnancy, severe liver disease, burn injuries and drug interactions.

Pseudocholinesterase Deficiency – Is Succinylcholine Still Needed to Facilitate Endotracheal Intubation?

“…Pseudocholinesterase deficiency is an uncommon condition that is present in about 1 in 3,200 to 5,000 people [ 1 - 2 ]. Although it is an autosomal recessive genetic condition, this condition is more prevalent in certain populations, including the Persian Jewish community and Alaskan natives.…”

“…Pseudocholinesterase (butyrylcholinesterase) is an enzyme produced in the liver and is responsible for the metabolism of several common anesthetic drugs, including mivacurium and succinylcholine. In individuals with normal serum enzyme levels, it metabolizes succinylcholine and the duration of action of the medication is less than 10 minutes [ 1 - 2 ]. In an individual with a pseudocholinesterase deficiency, there is a reduced ability to efficiently metabolize these anesthetic medications which results in the prolonged effect of neuromuscular paralysis [ 3 - 4 ].…”

Break the Spasm with Succinylcholine, but Risk Intraoperative Awareness with Undiagnosed Pseudocholinesterase Deficiency

“…Succinylcholine is normally metabolized by enzymes synthesized by the liver known as pseudocholinesterase. As discussed by Andersson [ 1 ], pseudocholinesterase deficiency can result from congenital defects or acquired events such as liver failure and drug interactions. The use of succinylcholine in the setting of genetic pseudocholinesterase deficiency leads to prolonged paralysis that can last for several hours.…”

“… Dibucaine inhibition is the percent of the pseudocholinesterase enzyme inhibited by the local anesthetic dibucaine. A dibucaine number less than 30 in addition to decreased plasma cholinesterase activity indicates a patient at high risk for prolonged paralysis after succinylcholine use [ 1 ]. …”

“…We present such a case where succinylcholine was emergently and successfully used to treat acute, refractory laryngospasm following extubation, but this was followed by prolonged neuromuscular blockade. As discussed elsewhere [ 1 , 2 ], pseudocholinesterase deficiency is a well-known condition that can significantly prolong succinylcholine activity, but this condition can be undiagnosed at the time of presentation. When succinylcholine is needed emergently at the time of emergence, it can place patients with undiagnosed pseudocholinesterase deficiency at increased risk of airway complications and anesthesia awareness with recall.…”