Chen Chao-Qin, Hu Zhen-Ping, Ma Xu-Jie, Tang Xiao-Dong, Zheng Xia, Yao Yong-Xing
Department of Anesthesia, First Affiliated Hospital, Zhejiang University School of Medicine, 79 Qingchun Road, Hangzhou, 310003, P. R. China.
Department of Intensive Care Unit, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, P. R. China.
BMC Anesthesiol. 2025 Jul 1;25(1):326. doi: 10.1186/s12871-025-03202-y.
Acute hyperammonemic encephalopathy (AHE) is a life-threatening condition. Hyperammonemia-induced mental disorders that appear after surgery and general anesthesia can be easily confused with postoperative delirium, especially in patients without signs of liver dysfunction. Currently, no reports of AHE precipitated by operative stress or general anesthesia exists. Here, we describe a patient without obvious liver dysfunction who developed AHE after general anesthesia and was diagnosed with postoperative delirium. Gene sequencing revealed that the patient had ornithine transcarbamylase deficiency (OTCD), which led to AHE and hepatic coma.
A 41-year-old man with a history of mild hypertension and depression was scheduled to undergo lung wedge resection under general anesthesia. Laboratory examination revealed low blood urea nitrogen levels. The anesthesia and surgery were uneventful. Four hours after returning to the surgical ward, the patient experienced agitation and developed mental disorders. His Confusion Assessment Method score was positive, and the Nursing Delirium Screening Scale score was 6; therefore, he was medically treated for postoperative delirium. However, his symptoms did not improve over the following days. On the 3rd postoperative day, the patient became unconscious and experienced limb twitching. Blood analysis revealed severe hyperammonemia (498 µmol/L) and respiratory alkalosis. Consequently, the patient underwent tracheal intubation and continuous venovenous hemodiafiltration, along with comprehensive supportive treatments, including intracranial pressure reduction and antiviral and gamma globulin therapy. However, the patient's serum ammonia level remained high. A computed tomography scan of the head revealed diffuse cerebral swelling. On the ninth postoperative day, the patient remained in a deep coma, with loss of brainstem reflex and brain electrical activity, at which point the treatment was terminated by family members. Postmortem genetic sequences revealed that the patient had OTCD.
AHE following general anesthesia with no evidence of liver failure can be easily misdiagnosed because of its non-characteristic clinical features. We recommend that in patients who experience refractory mental disorders after surgery, clinicians should remain vigilant for signs of hyperammonemia.
急性高氨血症性脑病(AHE)是一种危及生命的疾病。手术后和全身麻醉后出现的高氨血症诱发的精神障碍很容易与术后谵妄混淆,尤其是在没有肝功能障碍迹象的患者中。目前,尚无因手术应激或全身麻醉引发AHE的报道。在此,我们描述一名无明显肝功能障碍的患者,其在全身麻醉后发生AHE,并被诊断为术后谵妄。基因测序显示该患者患有鸟氨酸转氨甲酰酶缺乏症(OTCD),这导致了AHE和肝昏迷。
一名有轻度高血压和抑郁症病史的41岁男性计划在全身麻醉下进行肺楔形切除术。实验室检查显示血尿素氮水平较低。麻醉和手术过程顺利。返回外科病房4小时后,患者出现躁动并发展为精神障碍。其混乱评估方法评分呈阳性,护理谵妄筛查量表评分为6分;因此,他接受了术后谵妄的药物治疗。然而,在接下来的几天里他的症状并未改善。术后第3天,患者昏迷,出现肢体抽搐。血液分析显示严重高氨血症(498µmol/L)和呼吸性碱中毒。因此,患者接受了气管插管和持续静脉血液滤过,并接受了包括降低颅内压、抗病毒和γ球蛋白治疗在内的综合支持治疗。然而,患者的血清氨水平仍然很高。头部计算机断层扫描显示弥漫性脑肿胀。术后第9天,患者仍处于深度昏迷状态,脑干反射和脑电活动消失,并于此时被家属终止治疗。尸检基因序列显示该患者患有OTCD。
无肝功能衰竭证据的全身麻醉后AHE因其非典型临床特征很容易被误诊。我们建议,对于术后出现难治性精神障碍的患者,临床医生应警惕高氨血症的迹象。