What is the management for patients with plasma cholinesterase deficiency?

From the Guidelines

Patients with plasma cholinesterase deficiency should be managed by avoiding succinylcholine and other ester-based local anesthetics, and instead using non-depolarizing neuromuscular blocking agents like rocuronium, vecuronium, or cisatracurium, with quantitative neuromuscular monitoring used throughout anesthesia and recovery. This approach is supported by recent guidelines, such as those from the Association of Anaesthetists in 2021 1, which emphasize the importance of quantitative neuromuscular monitoring when administering neuromuscular blocking drugs, including succinylcholine and mivacurium. Key aspects of management include:

• Preoperative identification of patients with plasma cholinesterase deficiency through genetic testing or plasma cholinesterase activity measurement, especially if there is a personal or family history of prolonged neuromuscular blockade after anesthesia
• Use of alternative medications for anesthesia, such as non-depolarizing neuromuscular blocking agents
• Quantitative neuromuscular monitoring, which should be applied along with other essential monitoring, activated after induction of general anesthesia but before neuromuscular blockade, and used throughout all phases of anesthesia 1
• Ensuring adequacy of recovery, demonstrated by a train-of-four (ToF) ratio > 0.9, before patient awakening and extubation 1
• Consideration of fresh frozen plasma administration in severe cases, although this is rarely necessary
• Patient education and provision of medical alert identification to prevent future complications.

From the FDA Drug Label

Succinylcholine should be used carefully in patients with reduced plasma cholinesterase (pseudocholinesterase) activity. The likelihood of prolonged neuromuscular block following administration of succinylcholine must be considered in such patients (see DOSAGE AND ADMINISTRATION) Plasma cholinesterase activity may be diminished in the presence of genetic abnormalities of plasma cholinesterase (e.g., patients heterozygous or homozygous for atypical plasma cholinesterase gene), pregnancy, severe liver or kidney disease, malignant tumors, infections, burns, anemia, decompensated heart disease, peptic ulcer, or myxedema Patients homozygous for atypical plasma cholinesterase gene (1 in 2500 patients) are extremely sensitive to the neuromuscular blocking effect of succinylcholine In these patients, a 5 to 10 mg test dose of succinylcholine may be administered to evaluate sensitivity to succinylcholine, or neuromuscular blockade may be produced by the cautious administration of a 1 mg/mL solution of succinylcholine by slow intravenous infusion. Apnea or prolonged muscle paralysis should be treated with controlled respiration

The management for patients with plasma cholinesterase deficiency includes:

• Using succinylcholine with caution
• Considering the likelihood of prolonged neuromuscular block
• Evaluating sensitivity to succinylcholine with a 5 to 10 mg test dose
• Administering neuromuscular blockade with cautious slow intravenous infusion
• Treating apnea or prolonged muscle paralysis with controlled respiration 2

From the Research

Management of Plasma Cholinesterase Deficiency

The management of patients with plasma cholinesterase deficiency is primarily focused on supportive care, as there is no specific treatment for this condition 3, 4, 5. The key aspects of management include:

• Mechanical ventilation support to ensure adequate oxygenation and ventilation until the patient recovers from paralysis 3, 4, 5
• Sedation to keep the patient comfortable during the recovery period 4, 5
• Monitoring of neuromuscular function to assess the patient's recovery 5
• Avoidance of further administration of succinylcholine or mivacurium, which can exacerbate the condition 3, 4, 6, 7

Laboratory Testing

Laboratory testing, including plasma cholinesterase activity testing, can help diagnose plasma cholinesterase deficiency 3, 4. However, this testing may not always be able to identify patients at risk of prolonged neuromuscular blockade 4.

Treatment of Prolonged Neuromuscular Blockade

In cases of prolonged neuromuscular blockade, the best course of action is to provide ongoing ventilation and sedation until the patient recovers 4, 5. Transfusion of fresh frozen plasma may also be considered as a viable option 3.

Prevention

Prevention of prolonged neuromuscular blockade in patients with plasma cholinesterase deficiency involves avoiding the use of succinylcholine and mivacurium, which can exacerbate the condition 3, 4, 6, 7. In patients who have received high-dose cyclophosphamide or have malnutrition-induced pseudocholinesterase deficiency, caution should be exercised when using depolarizing muscular relaxants 6, 7.