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PubMed This is a summary of 17 peer-reviewed journal articles Updated
Anesthesiology

Anesthesia Management and Safe Alternatives

At a Glance

Patients with hereditary butyrylcholinesterase deficiency can safely undergo surgery by strictly avoiding the muscle relaxants succinylcholine and mivacurium. Anesthesiologists should use safe alternatives like rocuronium and monitor muscle function with quantitative Train-of-Four monitoring.

Knowing you have hereditary butyrylcholinesterase deficiency does not mean you cannot have surgery. It simply means your anesthesia team must use a different “recipe” of medications. When the right drugs and monitoring are used, your risk during surgery is the same as anyone else’s [1][2].

Drugs to Avoid

The most critical step in your future care is ensuring you are never given the two specific “trigger” drugs that your body cannot efficiently break down. These are:

  1. Succinylcholine: A very fast-acting muscle relaxant often used in emergencies or to place breathing tubes [3][4].
  2. Mivacurium: A short-acting muscle relaxant used for certain types of procedures [5][6].

In a person with your condition, a dose of succinylcholine that normally lasts 5–10 minutes could cause paralysis for several hours [7][8].

Safe Alternatives

Modern medicine provides several alternative muscle relaxants that do not rely on the butyrylcholinesterase enzyme for breakdown. These are considered safe for you:

  • Rocuronium: Currently the preferred alternative [2]. It is widely used because it can be immediately “undone” by a reversal drug called sugammadex, which works regardless of your enzyme levels [9][10].
  • Vecuronium: Another safe option that can also be reversed with sugammadex [11].
  • Atracurium and Cisatracurium: These drugs break down on their own through a chemical process in the blood that does not require your specific enzyme [2][12].

The Gold Standard: Quantitative Monitoring

To keep you safe, your anesthesiologist should use quantitative neuromuscular monitoring (often called Train-of-Four monitoring) [9][13].

  • How it works: A small device is placed over a nerve (usually on your wrist) that sends tiny electrical pulses to see how your muscles respond [9].
  • Why it matters: This provides the doctor with an objective “number” showing exactly how paralyzed you are and—more importantly—exactly when the drugs have worn off [13][14]. This prevents the risk of waking up while still unable to move.

Protocol for Accidental Exposure

If you were to receive a trigger drug accidentally (for example, in an emergency where your history wasn’t known), the medical team must follow a strict safety protocol:

  1. Continued Ventilation: You will stay on the breathing machine until the drug wears off naturally (usually 1 to 8 hours depending on your exact genetics) [7][15].
  2. Maintaining Sedation: This is the most important step for your comfort. The team will keep you deeply sedated (asleep) so that you are not “aware” or frightened while your body clears the medication [15][16].
  3. DANGER - Do Not Use Reversal Agents: Standard reversal drugs (like neostigmine or pyridostigmine) are sometimes used to wake up paralyzed muscles in normal patients. However, for a patient with this deficiency, neostigmine actually worsens and prolongs the paralysis by further inhibiting what little enzyme activity you have. It must never be used to try and force the succinylcholine or mivacurium to wear off faster [4][8].
  4. Time: The drug will eventually be cleared by other pathways in your body. It is simply a matter of waiting for the muscles to wake up on their own schedule [17].

By carrying a medical alert card and discussing these safe alternatives with your surgical team, you can ensure your future procedures are routine and safe [4][5]. (See Protecting Yourself and Your Family for more information on alerts).

Common questions in this guide

Can I safely have surgery if I have butyrylcholinesterase deficiency?
Yes, you can safely have surgery with this condition. Your anesthesiologist will simply use a different combination of medications and monitor you closely to ensure you wake up and breathe safely after the procedure.
Which anesthesia drugs do I need to avoid?
You must absolutely avoid succinylcholine and mivacurium. Your body lacks the enzyme needed to quickly break down these specific muscle relaxants, meaning a standard dose could leave you paralyzed for several hours instead of a few minutes.
What are the safe alternative muscle relaxants?
Rocuronium and vecuronium are highly recommended because they can be instantly reversed using a medication called sugammadex. Atracurium and cisatracurium are also safe options because they break down in the bloodstream without needing the deficient enzyme.
What happens if I am accidentally given succinylcholine?
If accidentally given a trigger drug, you must be kept deeply asleep on a breathing machine until the medication wears off naturally, which can take 1 to 8 hours. Standard reversal medications like neostigmine must never be used, as they will actually make the paralysis worse.
How does the doctor know when the muscle relaxants have worn off?
Your anesthesiologist should use a quantitative neuromuscular monitor, often called a Train-of-Four monitor. This device sends tiny electrical pulses to your wrist to measure your muscle response, providing objective proof that the muscle relaxants have completely worn off.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Will you be using quantitative neuromuscular monitoring (such as a 'Train-of-Four' monitor) during my entire procedure?
  2. 2.Can we agree to avoid succinylcholine and mivacurium and use safe alternatives like rocuronium instead?
  3. 3.If you use rocuronium, will you have sugammadex available in the operating room to ensure a rapid reversal?
  4. 4.What is your specific protocol for maintaining sedation if it takes longer than expected for me to breathe on my own?
  5. 5.How do you ensure that my diagnosis is flagged in the surgical 'time-out' checklist?

Questions For You

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References

References (17)
  1. 1

    Early Neurophysiological Monitoring of Train of Four Assists in the Detection of Pseudocholinesterase Deficiency.

    Celis V, Gandhi S, Overzet K

    The Neurodiagnostic journal 2025; (65(1)):57-63 doi:10.1080/21646821.2024.2401641.

    PMID: 39331762
  2. 2

    Comparison between succinylcholine and rocuronium as neuromuscular blocking agents for electroconvulsive therapy in a patient with pseudocholinesterase deficiency.

    Takazawa T, Suto T, Aihara M, et al.

    JA clinical reports 2015; (1(1)):7 doi:10.1186/s40981-015-0009-2.

    PMID: 29497639
  3. 3

    Pseudocholinesterase Deficiency Uncovered During Electroconvulsive Therapy: Implications for Psychiatric Services.

    Naik MJ, Nirale A, Bhat I, et al.

    The journal of ECT 2025; doi:10.1097/YCT.0000000000001177.

    PMID: 40953291
  4. 4

    Suspected Pseudocholinesterase Deficiency During Left Thyroid Lobectomy and Isthmusectomy: A Case Report.

    Boleyn J, McLaury M, Wieman S

    South Dakota medicine : the journal of the South Dakota State Medical Association 2024; (77(6)):266-269.

    PMID: 39013099
  5. 5

    Prolonged Neuromuscular Blockade Following Succinylcholine Administration and the Clinical Importance of Family History: A Case Report.

    Hubbell G, Slomowitz S, Thornton I

    Cureus 2025; (17(2)):e78814 doi:10.7759/cureus.78814.

    PMID: 40078265
  6. 6

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

    Kurnutala LN, Rugnath N

    Cureus 2020; (12(9)):e10721 doi:10.7759/cureus.10721.

    PMID: 33150117
  7. 7

    Neuromuscular Blockade Monitoring: Having It but Knowing When Not to Trust It.

    Salvador S, Frada R, Campos M, Esteves S

    Cureus 2023; (15(9)):e45438 doi:10.7759/cureus.45438.

    PMID: 37859899
  8. 8

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

    Andersson ML, Møller AM, Wildgaard K

    Anaesthesia 2019; (74(4)):518-528 doi:10.1111/anae.14545.

    PMID: 30600548
  9. 9

    Pseudocholinesterase Deficiency in a Patient Undergoing Electroconvulsive Therapy: A Case Report.

    Ladeira AC, Laranjeira J, Guariento L, et al.

    Cureus 2025; (17(12)):e98333 doi:10.7759/cureus.98333.

    PMID: 41487739
  10. 10

    Anesthetic Management of Eosinophilic Granulomatosis with Polyangiitis: A Narrative Review with an Illustrative Case in Cardiac Surgery.

    Torre DE, Pirri C

    Journal of personalized medicine 2026; (16(5)) doi:10.3390/jpm16050241.

    PMID: 42188335
  11. 11

    Isolation, structural characterization and quality control strategy of an unknown process-related impurity in sugammadex sodium.

    Zhuang T, Chen Y, Xu J, et al.

    Journal of pharmaceutical and biomedical analysis 2021; (200()):114072 doi:10.1016/j.jpba.2021.114072.

    PMID: 33866296
  12. 12

    Genetic Pseudocholinesterase Deficiency Unmasked After Succinylcholine-Rivastigmine Interaction: A Case Report.

    Ho E, Simpson B, Smith NA, Harper S

    A&A practice 2025; (19(9)):e02062 doi:10.1213/XAA.0000000000002062.

    PMID: 40985564
  13. 13

    Premature awakening and underuse of neuromuscular monitoring in a registry of patients with butyrylcholinesterase deficiency.

    Thomsen JL, Nielsen CV, Palmqvist DF, Gätke MR

    British journal of anaesthesia 2015; (115 Suppl 1()):i89-i94 doi:10.1093/bja/aev103.

    PMID: 26174307
  14. 14

    Unanticipated Profound Paralysis and Sugammadex Dosing Implications After Videoscopic Thoracic Surgery.

    McKittrick ML, Lombard FW

    Seminars in cardiothoracic and vascular anesthesia 2022; (26(1)):86-89 doi:10.1177/10892532211059885.

    PMID: 34889149
  15. 15

    Delayed recovery from paralysis associated with plasma cholinesterase deficiency.

    Zhou W, Lv S

    SpringerPlus 2016; (5(1)):1887 doi:10.1186/s40064-016-3561-y.

    PMID: 27843744
  16. 16

    A Comparison of Midazolam and Propofol for Deep Sedation in Patients with Acute Respiratory Distress Syndrome Requiring Neuromuscular Blocking Agents.

    Addison JD, Daley MJ, Curran M, Hodge EK

    Journal of pharmacy practice 2024; (37(2)):271-278 doi:10.1177/08971900221131420.

    PMID: 36189765
  17. 17

    Prolonged neuromuscular block associated with cholinesterase deficiency.

    Zhang C, Cao H, Wan ZG, Wang J

    Medicine 2018; (97(52)):e13714 doi:10.1097/MD.0000000000013714.

    PMID: 30593143

This information on safe anesthesia practices is for educational purposes only. Always discuss your diagnosis and personalized anesthesia plan with your anesthesiologist and surgical team prior to any procedure.

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