亨廷顿舞蹈病患者全麻1例

2019-03-06 麻醉安全与质控 覃兆军

患者,女,33岁,身高160 cm,体质量56kg,因摔倒致左锁骨骨折入院,拟择期全麻下施行左锁骨骨折切开复位内固定术。患者5年前诊断Huntington病,其母亲和姐姐均患有此病,现合并轻度抑郁症。

患者,女,33岁,身高160 cm,体质量56kg,因摔倒致左锁骨骨折入院,拟择期全麻下施行左锁骨骨折切开复位内固定术。患者5年前诊断Huntington病,其母亲和姐姐均患有此病,现合并轻度抑郁症。
 
查体:T36.8T:,P72次/min,RR15次/min,BP117/72mmHg(1mmHg=0.133kPa),心肺无异常,头部及四肢均有轻度不自主运动。实验室检查:WBC8.7x109/L,RBC3.61x1012/L,Hb116g/L,HCT38%,PLT150x109/L,白蛋白34g/L,球蛋白21g/L,谷丙转氨酶31IU/L,Na+138mmol/L,K+3.83mmol/L,Ca2+2.01mmol/L,其他检查未见异常。平日服用丁苯喹嗪治疗。患者入室前未使用术前药物。
 
入室后开放静脉通道,静脉注入咪达唑仑2mg以缓解焦虑。面罩吸氧,静脉注入芬太尼100ug、丙泊酚150mg、罗库溴铵50mg,并对环状软骨向后施压,行气管内插管及机械通气。术中吸入0.8~1.0MAC七氟醚维持麻醉,监测BP、ECG、HR、SpO2、PEtCO2、BIS、肌松及鼻咽温,给予温毯仪保温,维持T>36℃。手术历时55min,术中生命体征平稳,未追加肌肉松她药。术毕前10min静脉注入氢吗啡酮0.5mg,术毕给予格隆溴铵0.4mg、新斯的明3mg拮抗肌松作用。停止七氟醚后约10min,患者清醒,自主呼吸恢复良好,拔除气管导管,送入PACU观察。在PACU,患者诉疼痛并出现轻度寒战,继续给予温毯仪保温,静脉追加氢吗啡酮0.5mg,约10min后好转。术后第1天随访,除存在轻度头部及四肢不自主运动外,未见其他异常。
 
讨论
 
Huntington病,中文译名为亨廷顿舞蹈病,是一种全外显性常染色体显性遗传病,其临床表现复杂多变,常累及运动、认知和精神3个方面。一般在40岁左右发病,病情呈进行性恶化,通常发病15~20年后死于肺部感染。本病罕见,最近研究显示北美、欧洲、大洋洲患病率为5.7/10万入,中国、日本患病率为0.4/10万入。目前尚无有效治疗方法。国内外关于合并Huntington病患者麻醉处理的报道较少,因此,目前还没有针对该疾病的最佳麻醉处理方案。
 
为减轻症状,该类患者术前往往应用抗精神病药、抗抑郁药和苯二氮卓类等药物,因此,我们应注意麻醉药物与这些药物之间的相互作用,防止可能存在的精神症状加重、麻醉药物作用异常等情况。术前使用抗胆碱药阿托品和止吐药甲氧氯普胺会加重舞蹈病症状,应避免使用;如必须使用抗胆碱药,可应用不透血脑屏障的格隆溴铵。
 
该疾病后期由于面部非自主运动障碍、运动控制能力下降等原因可引起吞咽困难。一方面影响患者进食、影响营养状况,另一方面易引起麻醉过程中反流误吸的发生。这也是此类患者麻醉管理的重点。术前给予抑酸剂、止吐药有一定预防作用。本例患者诱导时采用环状软骨压迫、快速序贯诱导方式进行气管插管,等待患者肌松作用和气道反射恢复满意后拔除导管,整个麻醉期间未发生返流误吸。在1例急诊腹腔镜腹股沟疝修补术的麻醉中,为预防反流误吸,Kang等采用局麻下纤维支气管镜引导清醒气管插管,并采用全凭静脉麻醉维持,患者反应良好,认为这是此类患者一种安全有效的麻醉方法。
 
有报道发现此类患者对巴比妥类和苯二氮卓类敏感性增加,琥珀胆碱肌松作用异常延长,可能与患者血桨胆碱酯酶水平降低有关,但也有反应正常的病例。本例患者采用临床常用的芬太尼、丙泊酚和罗库溴铵进行诱导,未见异常反应。在麻醉恢复阶段给予格隆溴铵和新斯的明拮抗肌松作用,患者反应良好。
 
有报道认为七氟醚等吸入麻醉剂可增加该类患者苏醒期发生全身性强直痉挛、寒战等风险,建议采用全凭静脉麻醉。本例患者采用七氟醚维持麻醉,苏醒期间未发生全身强直痉挛,但出现轻度寒战,静脉注入氢吗啡酮0.5mg、加强保暖后迅速恢复。
 
近年也有报道硬膜外麻醉被成功应用于Huntington患者开腹胆囊切除术中;右美托咪定深度镇静联合小剂量氯胺酮镇痛辅助Huntington患者局麻下顺利完成白内障手术。这些方法为此类患者顺利完成手术提供了更多参考经验。
 
原始出处:

覃兆军.Huntington病患者全麻1例[J].麻醉安全与质控,2018,2(1):34-35.

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