Pseudocholinesterase Deficiency: What the Proceduralist Needs to Know

Am J Med Sci. 2019 Mar;357(3):263-267. doi: 10.1016/j.amjms.2018.11.002. Epub 2018 Nov 10.

Abstract

Pseudocholinesterase deficiency is a rare genetic as well as an acquired disorder that affects the body's ability to metabolize choline esters such as succinylcholine and mivacurium. It can be inherited as an autosomal recessive trait, occurring in approximately 1 in 3,200 to 1 in 5,000 people. In most cases of pseudocholinesterase deficiency, no signs or symptoms of the condition exist. It is first suspected after a prolonged recovery from paralysis following general anesthesia in which succinylcholine or mivacurium are administered. We emphasize the importance of obtaining a detailed history prior to any endoscopic intervention or surgery requiring monitored anesthesia care or general anesthesia. In addition, a family history of anesthesia complications may help identify patients at risk. This article examines a case of a patient who underwent a successful endoscopic pneumatic dilation under general anesthesia for the treatment of achalasia, but was subsequently admitted to the intensive care unit, requiring mechanical ventilator support, for 18 hours. The patient made a complete recovery and was discharged home with no further complications. This case stimulated a review of this entity and we provide recommendations relevant to all proceduralist and anesthesia staff, as well as all personnel involved in procedures.

Keywords: Pseudocholinesterase deficiency; Succinylcholine.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Anesthesia, General / adverse effects
  • Anesthesia, General / methods
  • Apnea* / complications
  • Apnea* / diagnosis
  • Apnea* / metabolism
  • Butyrylcholinesterase / deficiency*
  • Butyrylcholinesterase / metabolism
  • Dilatation / methods
  • Endoscopy / methods
  • Esophageal Achalasia* / physiopathology
  • Esophageal Achalasia* / surgery
  • Humans
  • Male
  • Metabolism, Inborn Errors* / complications
  • Metabolism, Inborn Errors* / diagnosis
  • Metabolism, Inborn Errors* / metabolism
  • Middle Aged
  • Neuromuscular Depolarizing Agents / administration & dosage
  • Neuromuscular Depolarizing Agents / adverse effects
  • Respiration, Artificial / methods*
  • Respiratory Paralysis* / etiology
  • Respiratory Paralysis* / therapy
  • Succinylcholine* / administration & dosage
  • Succinylcholine* / adverse effects
  • Treatment Outcome

Substances

  • Neuromuscular Depolarizing Agents
  • Butyrylcholinesterase
  • Succinylcholine

Supplementary concepts

  • Butyrylcholinesterase deficiency