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腰硬联合麻醉下先天性盆腔多脏器畸形合并双下肢废用性萎缩剖宫产1例

2018-10-29 蒙亚珍 郭余福 田丹 中国实验诊断学

34岁女性患者,先天性盆腔多脏器畸形(膀胱外翻,马蹄肾,左侧肾萎缩,双子宫,阴道畸形,耻骨联合分离),长期坐位或卧位。既往史个月时行膀胱外翻尿道修补术,10岁时行右输尿管皮肤造口术、左输尿管结扎术,20岁时行膀胱摘除术,30岁时行右肾结石微创手术。3胎1产,26岁时孕第一胎6个半月行剖宫产,胎儿未存活。第二胎孕两个半月胎儿停止发育,行人工流产。此次孕第三胎34周+4,因产程发动行剖宫产术。术前实验

1.临床资料
 
34岁女性患者,先天性盆腔多脏器畸形(膀胱外翻,马蹄肾,左侧肾萎缩,双子宫,阴道畸形,耻骨联合分离),长期坐位或卧位。既往史个月时行膀胱外翻尿道修补术,10岁时行右输尿管皮肤造口术、左输尿管结扎术,20岁时行膀胱摘除术,30岁时行右肾结石微创手术。3胎1产,26岁时孕第一胎6个半月行剖宫产,胎儿未存活。第二胎孕两个半月胎儿停止发育,行人工流产。此次孕第三胎34周+4,因产程发动行剖宫产术。术前实验室检查无剖宫产手术及腰硬联合麻醉禁忌证。
 
患者入室后常规监测无创血压、心电、指氧与脉率,面罩给氧(5L/min),建立静脉通路,并给予乳酸林格氏液6-8ml/kg预充血容量。患者取侧卧位,选择L2-3间隙进行硬膜外置管;选择L3-4间隙,25G腰麻针进入蛛网膜下腔,脑脊液回流顺畅,以0.1ml/s的速度给予0.5%罗脈卡因10mg。在给予罗脈卡因之前栗注去甲肾上腺素0.1μg/kg?min预防低血压,在给予罗脈卡因之后根据血压调整去甲肾上腺素的泵注速度以稳定该患者的血流动力学。而后立即让患者仰卧位、子宫左侧位,并将平面控制在T6。之后手术开始,并在5min后剖出一女婴,体重1850g,Apgar评分1min7分,5min8分。术中该患者血压维持在108-122/65-82mmHg,心率在80-108次/分,输入量约1000ml,出血量约200ml。
 
术中患者没有任何不适,手术顺利,手术时间约40min。该患者术后第1天双下肢感觉与运动完全恢复,术后第3天顺利出院。术后5个月随访患者无其他并发症。
 
2.讨论
 
膀胱外翻是一种少见而复杂的先天性畸形,是胚胎期泄殖腔膜发育异常,阻碍间充质组织的移行和下腹壁的正常发育,导致膀胱外翻、尿道上裂等一系列先天性异常。新生儿发病率为1/5万。膀胱外翻特点是一个低位置的肚脐,暴露膀胱,耻骨联合分离,腹直肌的盆底分离,前异位肛门和阴道和阴茎型尿道上裂。膀胱外翻不但给患者精神和生活上带来很大痛苦,而且易并发泌尿系感染,半数患者于成年前死于并发症。
 
肾脏最常见的解剖变异是马蹄形肾,发病率为1/400-1/8002。马蹄肾发生在胚胎早期,是两侧肾脏胚胎在脐动脉之间被紧挤而融合的结果。马蹄肾由于位置、方向和血液供应的变化,其外科解剖是复杂的。包括其动脉和静脉解剖变异度也是相当高的。因此异常血供也许是肾功能异常的原因或结果[3]。部分患者可全无症状。多数患者因神经丛、血循环或输尿管受压迫而发症状。有上腹部、脐部或腰部疼痛,慢性便秘及泌尿系统症状,如慢性肾炎、肾盂炎、肾积水和结石等。80%的病例可发生肾积水。
 
双子宫、阴道畸形均属于生殖道畸形。双子宫是由于胚胎发育期两侧副中肾管发育正常但未完全融合,各自发育形成双子宫。阴道畸形是可因为两侧副中肾管完全融合异常导致双阴道畸形或称完全性阴道纵隔,以对称性阻塞为特点;也可因为两侧副中肾管部分融合异常导致单侧阴道阻塞、阴道斜隔,以非对称性阻塞为特点;若下端副中肾管融合失败导致部分性阴道纵隔,阴道纵隔常伴有双子宫、双宫颈、同侧肾脏发育不良。
 
以上器官畸形均有报道,但是合并以上器官畸形的孕产妇尚无报道。先天性多器官畸形发生率本就较低,大多数先天性多器官畸形患者在新生儿期便已死亡。本病例患者能存活至今且孕育一女,最主要是因为畸形器官仅限于盆腔。
 
先天性盆腔多脏器畸形会带来机体病理生理的改变,主要包括:
 
(1)循环系统:该患是盆腔多脏器畸形,对循环的影响较小。但由于该患者因为多脏器畸形,仅以膀胱外翻这病症就已让其从小遭受精神与生活上的巨大痛苦,长期的坐位或卧位,进而导致双下肢废用性萎缩,循环系统的血管床重新分布,导致该患者的循环系统的调节能力较正常人差。双下肢血流量减少,血容量减小,进而全身的血容量下降。除此之外,马蹄肾输尿管跨过马蹄肾的峡部或横越融合肾的前表面,此时,一根或数根异位血管就会对输尿管造成某种程度的压迫而引起梗阻,进而易引起肾积水、结石等,甚至导致该患左肾萎缩,从而使该患者对循环调节能力较常人差。
 
(2)泌尿系统:该患膀胱外翻、马蹄肾,泌尿系统畸形,易出现泌尿系统感染、肾积水、肾结石等。即使该患者曾行多次泌尿系统手术改善症状,但泌尿系统感染症状反复,对其生活带来极大的不便。而且,由于长期合并泌尿系统感染,可出现肾的淀粉样变,进而导致其左肾萎缩,但其肾功能检查未见异常。
 
而腰硬联合麻醉后,可由于交感神经节前神经纤维被阻滞,小动脉扩张,周围阻力下降,而且血液游积于周围血管系,静脉回心血量减少,心排量下降,导致血压下降。对于产妇而言,仰卧位低血压综合征一般发生在妊娠28周之后,尤其是32-36周时最易发生。本病例患者此时处于仰卧位低血压综合征最易发生的时期。仰卧位低血压综合征是在妊娠晚期,子宫本身的血流量约占全身血容量的16.67%,使回心血量减少,同时,增大的子宫压迫下腔静脉,使盆腔和下肢静脉的血液回流受阻,回心血量下降,心输出量下降,血压下降;此外,增大的子宫还会压迫横膈,引起迷走神经兴奋,心跳减慢,血压进_步下降。
 
而本病例患者长期卧位,循环已适应此体位,血压在正常范围内或稍低,但腰硬联合麻醉后由于血管床的开放,回心血量减少,心排量减少,血压下降,而对于该患者而言,循环调节能力较差,可能会导致血压骤降,而回升不易,从而导致一系列不良后果。鉴于此,我们于术前通过预充血容量即快速补液6-8ml/kg乳酸林格氏液,并通过血管活性药物即泵注去甲肾上腺素来预防低血压。
 
本病例产妇先天性盆腔多脏器畸形合并双下肢废用性萎缩,在麻醉管理上应注意其特殊的病理生理改变,预先采取适当的措施包括灵活地选取麻醉方式、预充血容量、适当使用血管活性药物来维持血流动力学的稳定,进而保证患者安全度过围术期。 

原始出处:

蒙亚珍, 郭余福, 田丹,等. 腰硬联合麻醉下先天性盆腔多脏器畸形合并双下肢废用性萎缩剖宫产1例[J]. 中国实验诊断学, 2017, 21(8):1446-1447.

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    2019-09-03 chendoc252
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    2018-10-31 huirong
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    2018-10-30 医者仁心5538

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