膈疝引起的急诊手术：病例系列和综述

概要
背景
先天性膈疝（CDH）是一种先天性异常，成人罕见，频率为0.17-6％。膈肌破裂是创伤的罕见后果，约占严重闭合性胸腹部损伤的5％。临床表现范围从无症状病例到严重的呼吸道或胃肠道症状。诊断取决于病史，临床症状和放射学检查。

方法
从2013年5月至2016年6月，6例（4名女性，2名男性;平均年龄58岁）膈疝入住作者的普通外科学术部，患有呼吸和腹部症状。进行胸部X射线，钡研究和CT扫描。

结果
病例1呈现左侧膈疝，包括横结肠和降结肠。病例2显示左CDH允许胃，脾和结肠通过。病例3和6显示左半胸的胃。病例4呈左膈疝，允许脾脏，左肝叶和横结肠通过。病例5有胃和脾脏突出到胸部。总是进行紧急手术。疝气含量减少，缺损通过初次修复或网状结束。在所有情况下，术后课程都平安无事。

结论
在有或没有创伤史的患者中，重叠的腹部和呼吸道症状导致膈疝的诊断。应进行胸部X线，CT扫描和钡研究以评估膈肌缺损，大小，位置和内容。紧急手术方法是强制性的，可降低发病率和死亡率。

关键词：先天性膈疝，膈肌破裂，网状，急诊手术，剖腹手术，开胸手术

背景
先天性膈疝（CDH）是一种在1/2500新生儿中发现的异常，存活率为67％[1]。 CDH的主要特征是在胚胎发生过程中隔膜不能正确形成。隔膜的这种不完全形成允许腹部内容物突出到胸腔中，产生阻碍肺发育的质量效应。临床表现范围从无症状病例到严重的呼吸道或胃肠道症状，有时还有血液动力学不稳定。 CDH患者的广泛严重程度取决于肺发育不全和肺动脉高压的程度。后外侧疝（Bochdalek疝）是最常见的疝气类型（> 80％），大多数发生在左侧（85％），较少发生在右侧（13％）或双侧（2％）[2]。

膈肌破裂（DR）是一种罕见的创伤并发症，发生于5％的创伤，包括车辆事故[3-5]。诊断通常会延迟;创伤后多年内患者可能无症状，直至出现并发症。创伤性膈肌破裂被认为是手术修复的指征，特别是在有症状的患者中[6]。

然而，对手术的绝对适应症和时机尚无共识。并发症的发生具有最高的死亡率和发病率;因此，它强制要求紧急手术。在过去的几十年中，用合成网修复或覆盖缺损的一次缝合已成为标准程序。最近，生物网状结构被认为可有效地闭合膈肌缺损，诱导有限的炎症反应并最大限度地减少粘连形成[7]。剖腹手术或胸廓切开术是DR患者的传统治疗方法。此外，腹腔镜修复疝气的方法最近越来越受欢迎[8]。机器人方法尚未被描述为紧急情况下的有效方法，仅在一个案例[9]的文献中报道了选择性手术。

本文包括急诊手术单位先天性或创伤性膈疝的手术经验，并报道文献。

方法
作者的学术部门在紧急情况下观察到6例膈疝，伴有呼吸和腹部症状。左胸部区域未检测到呼吸音，但可听到肠鸣音。在所有病例中都进行了紧急手术。疝内容物减少，缺损通过初次修复或网状物闭合。

案例1：一名63岁的女性因肠梗阻和呼吸困难而被收治。回忆显示慢性腹痛，精神发育迟滞和斜视。在体检中，左胸区未检测到呼吸音;然而，肠鸣音是听得见的。胸部X射线和钡灌肠显示横向结肠移位到脾曲上方的左半胸。计算机断层扫描显示肺部塌陷，纵隔向右移位。左膈疝含有横结肠和降结肠（图1a）。进行急诊剖腹探查，发现左膈肌发育不全，巨结肠（直径10cm）和左肝发育不全。术中支气管镜检查显示左肺发育不全（图1b）。进行了具有回肠 - 直肠吻合术的次全结肠切除术，并完成了膈肌的一次修复。术后病程平安无事，病人于术后第15天出院。所有已知先天性综合征的核型，表型和遗传模式异常的研究均为阴性。


图1
CT扫描显示肺部塌陷，纵隔向右侧移位。左膈疝含有横结肠和降结肠。 b术中证据：膈肌缺损允许内脏移位

案例2：一名50岁女性因接受呼吸困难，胸痛和腹痛而入院。左胸部区域未检测到呼吸音。没有创伤史。胸部X光检查显示右侧纵隔移位，左侧胸腔内有肠道气体。 CT扫描显示大的环状膈肌缺损，允许胃，脾和结肠通过（图2）。采用紧急联合胸腹入路，减少内容物用Mersilenemess®修复缺损。胸廓切开术用于释放胸部和腹部内容物之间的胸部致密粘连。在放置网状物之前，麻醉师增加潮气量以扩张肺部塌陷的左下叶，并将胸腔引流管放置在左胸膜腔中。即刻术后胸部X线片显示左肺扩张，胸腔积液最少。术后病程平安无事，术后住院时间为13天。


图2
CT扫描显示左侧大膈肌缺损，允许胃，脾和结肠通过（称为Bochdalek疝），左肺完全塌陷

案例3：一名73岁的女性因呼吸困难和吞咽困难而到达。 在病史中没有明显的创伤史。 她目前的病史包括高血压和甲状腺功能减退症。 胸部X线和钡研究表明左半胸存在胃。 CT扫描显示存在大的膈疝，使胃疝入胸腔。 进行了紧急腹腔镜检查; 疝气含量减少; 并使用Proceedmess®修复缺损（图3）。 术后病程平安无事，患者在手术后7天出院。


图3
合成网状修复缺损时的腹腔镜图像

病例4：一名63岁的女性因呼吸困难而入院2天，逐渐进展并伴有左侧胸痛和干咳。 6年前有车祸的历史。 最初的胸片显示左半膈膜升高，半胸下半部存在结肠气体阴影。 CT扫描显示左膈疝允许脾脏，肝左叶和横结肠通过（图4a）。 手术在紧急情况下进行，减少内容物并用生物网修复缺损（图4b; Tutomesh，牛心包网）。 患者于术后第10天出院，无并发症。


图4
CT扫描显示左膈疝允许左胸部结肠移位。 b术中，生物网修复缺损

案例5：一名50岁的男子参与了一起工伤事故。他的管理符合Advanced Trauma Life Support协议。他到达急诊室，左侧呼吸音减弱，呼吸困难，发热，左侧心肌血肿，皮下气肿，胸腹痛。他目前的病史包括肥胖和高血压治疗。最初的胸片和钡研究显示左半胸的胃。 CT扫描显示左半胸的胃和脾，与创伤性膈肌破裂一致，所有肌层完全破裂，颈征和多发肋骨骨折，左肱骨和肩胛骨骨折（图5a，b）。在剖腹探查术中，发现左侧膈肌有创伤性缺损，左胸部有胃和脾（图5c）。减少疝内容物并用生物网（Tutomesh bovine pericardiummesh®）封闭缺损。手术后，患者被安置在重症监护病房。他于术后第8天从ICU转入，并于第20天出院。


图5
a 3D-CT扫描显示肋骨骨折。 b CT扫描显示左半胸部的胃和脾，完全破坏所有肌肉层。 c术中，修复左侧横膈膜创伤性缺损

病例6 [10]：一名51岁男性，提到5个月的呼吸困难，腹痛，恶心和呕吐史。在过去的2周内，这些症状的严重程度有所增加。回忆显示4年前左侧脾切除术治疗非霍奇金淋巴瘤。体格检查显示腹膜内中度积液，无腹膜反应。鼻胃管的引入显著改善了症状。胸部X线片显示左侧半膈膜下方有一个大的液位（图6a），假设左半膈肌破裂伴有胃疝;通过钡研究和胸腹部计算机断层扫描证实了诊断。进行了紧急左胸廓切开术，显示胃扭转，伴有一些肠袢。横向结肠的一部分通过撕裂的横膈膜嵌入疝气。疝气位于左半膜的后侧，直径为12厘米。在手术过程中，发现突出的器官和左胸膜肺之间的密集粘连，以及左肺容积显著减少和粘连隔膜的大网膜炎性肿块。因此，进行了肠扭转的减少，粘连分解和肿块的切除。最后，采用左侧膈肌缺损的直接缝合（图6b，c）。患者恢复得很顺利，组织学显示霍奇基淋巴瘤。


图6
a X射线显示胸部突出的胃。 b胸廓切开术显示大的膈肌缺损。 c修复缺陷

回顾文献
1983年至2017年，PubMed / MEDLINE使用术语“急诊手术”，“创伤性膈肌破裂”和“先天性膈疝”进行了系统评价。 搜索返回555篇论文（图7）。 排除了323篇出版物，因为这些文章不是用英文写成的（N = 87），在儿童时期（<19岁; N = 178）或不是有趣的人类（N = 58）; 32篇论文被排除在外，因为它们被认为是食管裂孔疝，40例食道疝和59例选择性疝气。 因此，对101篇文章的全文进行了资格评估：697名患者的创伤发生率为创伤性，38名患者为先天性（表1）。


图7
文献选择过程的流程图

表格1
审查显示人口统计数据，诊断和治疗的文献


发病机制
腹股沟疝的膈肌破裂首先由Sennertus在1141年描述[11]。先天性膈疝是产前或在新生儿期诊断出来的。相反，成人期的CDH非常罕见，可以通过前胸骨旁Morgagni孔或后外侧（主要是左侧），称为Bochdalek疝，首先在1848年描述[12]。病因仍在研究中，但该病是由于在妊娠第8周期间横隔膜和食道之间的管道闭合失败。 Morgagni疝是一种罕见的疾病，由胸骨附着于膈肌的缺陷发展引起。创伤性膈疝被认为是由沿着胚胎融合点的潜在弱点区域发生的胸腹膜压力梯度的突然增加而产生的[13]。

DR通常由钝性或穿透性损伤或医源性原因引起，导致腹腔中空内脏或网膜进入胸膜腔，这可能导致嵌顿甚至窒息致命结果。创伤性膈疝通常由穿透性损伤（10-19％）引起，有时由钝性胸腹部创伤（5％）[14,15]引起。此外，一些作者描述了手术或怀孕后罕见的特别是DR病例;那就是Sano A.等。报告了一例怀孕28周的孕妇，她接受了紧急剖腹产和膈肌的修复[16]; Moussa G.等人描述了患有先前窗开窗和结节病病史的患者的右DR [17]; Nakamura T.等报道了一例因射频消融治疗肝癌史患者的右DR [18]。此外，Marfan综合征与CDH之间存在关联，如Barakat等。报道[19]。

破裂的部位
左侧CDH形成率为80％[20]。此外，88-95％的膈肌破裂发生在左侧[21]，尤其是钝性创伤导致大的膈肌缺损，通常涉及（> 80％）左侧后外侧膈[22]。由于具有保护作用的肝脏大小，右侧膈肌比左侧膈肌强。由于这个原因，侧面破裂是非常罕见的，并且与高死亡率和发病率相关[23]。

本研究报道的文献综述证实左侧缺损率高达80％，仅报告了2例双侧DR。

提出症状和调查
Nayak等人。描述严重症状，46％的CDH病例，由于内脏窒息导致32％的死亡率[24]。此外，文献分析显示延迟症状的可变率（5-45.5％）[25,26]。左侧晚期CDH通常会产生急性，阻塞性，胃肠道症状，慢性呼吸困难，胸痛，反复腹痛，餐后饱胀和呕吐，演变为心肺功能衰竭[27]。事实上，右侧CDH通常只与呼吸问题有关，因为部分肝脏移位可能会阻止中空内脏的进一步突出[1]。尽管胸腔内存在肠鸣音并且没有呼吸音是与CDH相关的典型发现，但据报道误诊率为38％[28]。显然，在完全无症状的病例中，诊断非常困难。相反，当由于腹压的增加和由此导致的快速内脏移位到胸部或由于先前突出的内脏的快速扩张而发生急性表现时，诊断是明确的[29,30]。胸部X光和钡研究可用于确定哪些内脏突出到胸腔中。 CDH最常见的放射学发现是半胸的不透明性，通常与纵隔向对侧的移位有关。此外，鼻胃管在胸腔中的位置将提供重要指标并迅速正确诊断。计算机断层扫描可被视为诊断的金标准技术，为评估膈肌缺损的存在，大小和位置以及各种类型的膈疝的内容提供了独特的机会[31]，并显示了14的敏感性和特异性。 -82％和87％，分别为[32]。 MRI也很有用，但通常在紧急情况下无法执行。然而，它通常用于稳定的患者或CT扫描是模棱两可的[33]。根据文献报道，在这一报道的经验中，通过CT扫描和钡研究进行了明确的诊断。

迟发性CDH被认为是一种良性疾病，但它很快就会成为危及生命的疾病[1,27,28,31,33];因此，必须立即进行手术治疗。据报道，晚期CDH患者的相关异常，例如先天性心脏病，Fryns综合征和18号染色体，在8.6-80％的病例[1,2,27,28]中显著增加了死亡率。在这个提议中，在案例1中，即使对先天性综合症有高度怀疑，令人惊讶的是遗传研究也没有证实这一点。

手术治疗
手术修复通常涉及通过开放腹部方法对隔膜进行初级或贴片闭合。当诊断延迟时，由于怀疑内脏和胸腔之间的粘连，胸腔切开术或胸腹联合入路是首选，如报告的病例2.有些作者报道胸腔镜入路成功但疝气复发率增加无效[34-36]。此外，在胸腔镜检查期间，可发生术中肺动脉高压，随后出现血流动力学不稳定;此外，贴片的放置和管理导致显著更长的操作时间。由于这些原因，在存在小膈肌缺损和/或轻度肺动脉高压的情况下，胸腔镜下修复CDH是首选[37]。如今，腹腔镜手术对于CDH来说是安全可行的，它可能是一个很好的选择[37]，如案例3所示。

然而，紧急手术是膈肌破裂的首选治疗方法。在延迟病例中，建议采用胸腔入路减少内脏 - 胸膜粘连，避免胸内内脏穿孔伴有灾难性并发症[38]。当怀疑肠梗阻明显时，也可能需要腹部手术来控制器官。虽然用于膈疝的闭合类型仍然存在争议，但人们普遍认为大多数缺损可以主要用不可吸收的缝合线封闭[39]。当缺陷太大而不能主要关闭时，通常使用网格修复，并且使用无张力网格对于程序的成功是至关重要的。最近，由于疝复发率较低，感染抵抗力较高，移位风险较低，因此引入生物网片代替合成纤维网[7,40];然而，文献中有限的证据表明它们的优越性。事实上，在作者之前的经验中，生物网格也被用于受污染的手术领域，并取得了良好的效果[40]。然而，由于这种情况的罕见性，应该鼓励临床医生在膈疝修补术中发表生物学网的经验[7]。

结论
当诊断出膈疝时，手术是首选的治疗方法，尤其是在紧急情况下。建议在专门的中心采用多学科方法。

参考：
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