遗传性血管性水肿患者眼科手术麻醉一例

2019-11-12 邓伟 曹草 郭丽伟 临床麻醉学杂志

患者,女,55岁,56kg,160 cm,因车祸伤致“右侧眼球破裂伤、右眼眶骨折”入院,拟在全麻下行“右眼内容物剜出伴义眼置入术+眼窝成形术+眼睑裂伤缝合术”。10年前在北京协和医院诊断为“遗传性血管性水肿(hereditary angioedema,HAE)”。

患者,女,55岁,56kg,160 cm,因车祸伤致“右侧眼球破裂伤、右眼眶骨折”入院,拟在全麻下行“右眼内容物剜出伴义眼置入术+眼窝成形术+眼睑裂伤缝合术”。10年前在北京协和医院诊断为“遗传性血管性水肿(hereditary angioedema,HAE)”。
 
查体:意识清楚,表情痛苦,颜面部皮肤黏膜轻中度水肿,伴腹痛。血常规、胸片、心电图基本正常。眼眶CT示:右侧眼球破裂伤;右侧上颌窦各壁、右侧眼眶内外侧壁、双侧鼻骨、右侧颧弓多发骨折。血清补体C30.63g/L(正常值0.9~1.8g/L)和C40.068g/L(正常值0.1~0.4g/L)。诊断:多部位损伤、眼球破裂(右)、眼眶骨折(右)、创伤性玻璃体积血(右)、遗传性血管性水肿。
 
经麻醉科、耳鼻咽喉科、眼科、风湿免疫科等多学科会诊后,每天给予雄性激素达那唑600mg预防治疗,输注新鲜冰冻血浆(FFP)400ml和动态监测血清补体,术中备好气管切开。经以上处理后,3d后患者颜面部皮肤黏膜水肿减轻,无腹痛,复查C30.83g/L和C40.092g/L。入室后开放外周静脉输液通道,入室BP120/70mmHg,SpO298%。
 
术前用药:阿托品0.3mg。术前备好400mlFFP和气管切开包后,开始全麻诱导:丙泊酚60mg和芬太尼0.1mg。诱导平稳,面罩持续给氧,FiO2 80%,氧流量5L/min。常规消毒铺巾,眼科医师予2%利多卡因行右侧球后神经阻滞。麻醉维持:丙泊酚1.5~2mg·kg-1·h-1和瑞芬太尼0.1μg·kg-1·min-1。维持SpO2≥95%,RR12~20次/分,BP90~120/60~70mmHg,BIS40~60。手术顺利,历时1h,输注400mlFFP,术中无喉头水肿和眼心反射等并发症发生。患者清醒后无呼吸困难,送回ICU,术后因腹痛再次输注FFP200ml。1d后转入普通病房,4d后顺利出院。围术期总共输注FFP1000ml,术后输注FFP时出现荨麻疹,肌内注射异丙嗪50mg治疗。
 
讨论
 
HAE是一种罕见的遗传性疾病,由基因缺陷引起,目前还没有统一诊断标准和治愈方法,只能够控制发病症状。主要临床表现为反复发生的皮肤、呼吸道和内脏器官肿胀,当水肿发生于气道时,可致喉头水肿,如果抢救不及时,1/3患者可因窒息死亡。当水肿发生于肠道,出现腹痛等类似急腹症的表现,常误诊为阑尾炎,导致急诊进行剖腹探查。其病因与患者血清中C1脂酶抑制因子(C1 inhibitor,C1INH))减少或功能缺损,以致C1过度活化,C4和C2的裂解失控,所生成的缓激肽增多,以致使微血管通透性增高,引起水肿有关。与过敏性或药物性血管性水肿不同,糖皮质激素或抗组胺药对HAE引起的水肿无效。
 
HAE患病率约为1/10000~1/50000。国内最常用的药物是达那唑,维持剂量取决于病情,每天范围从50~600mg。由于血浆来源的C1INH尚未进入中国,故仍然缺乏用于HAE急性发作的治疗药物。临床以应用FFP治疗HAE引起的喉头水肿、呼吸困难、呕吐和腹痛,输液量在200~1000ml,个别患者在输注早期腹痛加重,输注30~90min后症状改善并且在2~12h后完全消退。少数患者可出现荨麻疹,肌内注射异丙嗪50mg可减少过敏反应发生。输注FFP可缩短HAE患者急性加重的过程并减轻全身水肿症状。但是,该方法有引起症状恶化的风险,输注过程中需密切观察。
 
本例患者因外伤致HAE急性发作,临床表现以颜面部皮肤黏膜轻中度水肿和腹痛为主,暂无呼吸困难和喉头水肿。由于局麻无法抑制拟行眼科手术的应激刺激,且手术刺激有诱发喉头水肿的可能。BIS监测下联合输注丙泊酚和瑞芬太尼可产生满意的麻醉效果,为眼科手术创造良好的操作环境。因此,此次选择了全身麻醉,但未行气管插管,也未放置喉罩和口咽通气道等声门上通气装置,以避免刺激患者呼吸道而诱发喉头水肿,而是通过面罩给氧这一无创通气方式控制患者呼吸道。
 
在充分保证球后神经阻滞效果的基础上,减少麻醉药物的应用剂量,保证充足的氧供和避免CO2潴溜,维持适当的麻醉深度。但是,仅面罩给氧的气道管理方法值得探讨,术中麻醉风险较大。此类患者麻醉管理要点:(1)术前详细了解患者的病史、发病情况、用药情况以及最近有无气道和喉头水肿发作;(2)务必多学科联合会诊,评估手术和麻醉风险,进行充分的术前准备;(3)在无C1INH等特效药物情况下,谨慎选择气管插管全麻,术中备好气管切开工具应对喉头水肿急性发作,术后重症监护。综上所述,HAE发病率低,误诊率和死亡率高,临床医师对其认识尚不全面,国内缺乏急性发作期的治疗药物,临床上以达那唑和FFP预防喉头水肿发作为主,麻醉风险较大。一旦诱发严重喉头水肿,应行气管切开。

原始出处:

邓伟,曹草,郭丽伟,徐国海,余树春.遗传性血管性水肿患者眼科手术麻醉一例[J].临床麻醉学杂志,2018,34(11):1144.

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    2019-12-15 147b245cm61暂无昵称

    学习了,很好的病例

    0

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    2019-11-16 14794e5bm67(暂无昵称)

    非常受益,很好的研究

    0

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    2019-11-14 小华子
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