Uzun Davut Deniz, Wildenberg Katharina, Ruping Fabian, Westhoff Jens H, Schmitt Felix C F, Weigand Markus A, Zivkovic Aleksandar R
Department of Anesthesiology, Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany.
Division of Pediatric Surgery, Department of General, Visceral and Transplantation Surgery, Medical Faculty Heidelberg, Heidelberg University, Heidelberg, Germany.
BMC Pediatr. 2025 Aug 11;25(1):619. doi: 10.1186/s12887-025-05996-9.
Pseudocholinesterase, an enzyme produced by the liver and abundantly present in plasma, plays a role in the metabolism of neuromuscular blocking agents such as mivacurium. The administration of mivacurium to patients with pseudocholinesterase deficiency during general anesthesia has been associated with the occurrence of prolonged postoperative paralysis, a rare but potentially severe side effect.
A 24-month-old girl underwent routine, elective minor surgery. The neuromuscular blocking agent mivacurium was used during general anesthesia. Following the procedure, there was no spontaneous recovery of breathing. The peripheral nerve stimulator used to measure neuromuscular relaxation did not elicit any responses. The patient was subsequently transferred to the pediatric intensive care unit, where she was successfully weaned from the ventilator and extubated four hours later. The following day, the child was deemed fit for discharge from the clinic, exhibiting no residual symptoms. A targeted laboratory analysis conducted subsequent to the event revealed a cholinesterase level of 4176 U/l (normal values 4260-12920 U/l), a cholinesterase dibucaine-number of 12 (normal values > 70) and cholinesterase fluoride-number of 29 (normal values 55-60).
This is a case report of pseudocholinesterase deficiency in a 24-month-old child undergoing minor surgery. Quantitative neuromuscular monitoring should be used routinely to recognize prolonged muscle relaxation. If the diagnosis of pseudocholinesterase deficiency is confirmed, the patient should be given information about the disorder, the associated risks, inheritance and the need for testing in other family members.
假性胆碱酯酶是一种由肝脏产生并大量存在于血浆中的酶，在米库氯铵等神经肌肉阻滞剂的代谢中起作用。全身麻醉期间，给假性胆碱酯酶缺乏的患者使用米库氯铵与术后长时间麻痹的发生有关，这是一种罕见但可能严重的副作用。
一名24个月大的女孩接受了常规择期小手术。全身麻醉期间使用了神经肌肉阻滞剂米库氯铵。手术后，呼吸未自主恢复。用于测量神经肌肉松弛的外周神经刺激器未引出任何反应。患者随后被转至儿科重症监护病房，4小时后成功脱机并拔管。第二天，该患儿被认为适合出院，无残留症状。事件发生后进行的针对性实验室分析显示，胆碱酯酶水平为4176 U/l（正常值4260 - 12920 U/l），胆碱酯酶二丁卡因值为12（正常值>70），胆碱酯酶氟化物值为29（正常值55 - 60）。
这是一例24个月大接受小手术患儿的假性胆碱酯酶缺乏病例报告。应常规使用定量神经肌肉监测来识别肌肉松弛时间延长的情况。如果假性胆碱酯酶缺乏的诊断得到证实，应向患者提供有关该疾病、相关风险、遗传情况以及其他家庭成员进行检测必要性的信息。
