20210409湘雅二医院课件荟萃【胸腹膜裂孔疝(Bochdalek疝)】
● Clinical Presentation
● A 58-year-old man presents with intermittent chest pain.
● 临床表现
● 一名58岁的男子因间歇性胸痛就诊。
● 1A - Annotated Caption
● Noninfused computed tomography with oral contrast shows a moderate left pleural effusion and the herniation of peritoneal fat (arrow) and stomach (arrowhead) into the right hemithorax through a posterior diaphragmatic defect. There is atelectasis of the overlying right lung base and a small right pleural effusion.
● 口服造影剂,接着行CT扫描。显示左胸腔中等量积液,腹膜脂肪(箭头)和胃(箭头)通过后膈膜缺损疝入右胸。右肺不张,右侧少量胸腔积液。
● Further Work-up
● No Further Work-up
● 进一步处理
● 没有进一步的处理
● Imaging Findings
● Click on Annotated Thumbnail to reveal caption
● 影像表现
● 点击标记的缩略图,以查看注解
● Differential Diagnosis
● Bochdalek hernia (BH): This is the most likely diagnosis for the characteristic finding of herniated abdominal contents through a posterior diaphragmatic defect.
● Traumatic diaphragmatic hernia: This can result from laceration of the diaphragm in blunt trauma or penetrating injury, and symptoms related to herniated contents can be delayed for years after injury in some cases.
● Eventration of the diaphragm: This option is congenital hypoplasticity of the diaphragm. It is usually anteromedial but occasionally posterior, with the appearance seen in this case.
● Diaphragmatic paralysis: This can elevate abdominal structures into the chest. Paradoxical motion during a fluoroscopic sniff test would suggest this abnormality.
● 鉴别诊断
● Bochdalek 疝(BH):这是最有可能的诊断,他的特点是腹部内容物通过后膈肌缺损疝入。
● 创伤性膈疝:这可能是由于钝伤或穿透性损伤导致的膈肌撕裂导致的,并且在某些情况下外伤后疝与疝内容物相关的症状可能会延迟出现,长达几年时间。
● 膈的上下的病变:这个选项是膈先天亚塑性。它通常是前内侧的,但偶尔后面,影像表现和这个例子相同。
● 膈肌麻痹:这可以使膈膨升,腹部结构进入胸腔。在透视下膈的矛盾运动能提示这种异常改变。
● Essential Facts
● BH is a congenital hernia that occurs through the posterolateral foramen of Bochdalek. The space between the septum transversum and the pleuroperitoneal membrane normally closes, unless herniated contents maintain patency.
● This case is somewhat unusual as BHs more commonly occur on the left side, where the liver is not present to block contents from herniating into the thorax.
● Herniated contents often include peritoneal fat, omentum, and spleen as most cases are left-sided. Contents may also include stomach or liver on the right, and kidney, bowel, or pancreas.
● Clinical presentation in adulthood can include abdominal or chest pain, as in this case, recurrent pneumonia, bowel obstruction, and cardiopulmonary symptoms. In infants, large diaphragmatic hernias can present with severe cardiopulmonary insufficiency, and many of these patients die within a few years of birth.
● Complications can include pulmonary hypoplasia and strangulation or volvulus of a herniated viscus.
● 基本事实
● BH是通过Bochdalek的后外侧孔发生的先天性疝。横膈膜和胸膜腹膜之间的间隙通常是关闭的,除非疝内容物存在他才会保持通畅。
● 这个例子有些不寻常,因为BH更常见于左侧,左侧没有肝脏,不会阻止疝内容物进入胸腔。
● 疝内容物通常包括腹膜脂肪,网膜和脾脏。大多数病例是左侧的。疝内容物还可能包括胃或右侧的肝脏,以及肾,肠或胰腺。
● 在成年期,临床表现可以包括腹部或胸部疼痛。在这个病例,病史有反复发作的肺炎,肠梗阻和心肺症状。在婴儿中,巨大的膈疝可以出现严重的心肺功能不全,其中许多患者在出生后几年内死亡。
● 并发症可能包括肺发育不良和内脏疝入后扭转绞窄。
● Other Imaging Findings
● No Other Imaging Findings
● 其他影像表现
● 没有其他影像表现
● Pearls and Pitfalls
● Large congenital hernias like BHs can be life-threatening shortly after birth because of pulmonary hypoplasia, atelectasis, or mediastinal shift.
● Herniated contents in BHs are not enveloped by a hernial sac, unlike those in Morgagni hernias.
● BHs are more likely to present in infancy than Morgagni hernias, which typically present in adults.
● Eventration of the diaphragm and traumatic diaphragmatic hernias may cause the same group of structures found in BHs to project within the chest.
● Missing necrosis due to incarceration or volvulus of herniated contents can be life-threatening!
● 精华和误区
● 由于肺发育不全,肺不张或纵隔移位,BH等大型先天性疝在出生后不久可危及生命。
● BH中的疝内容物不被疝囊包裹,与Morgagni疝的不同。
● BH比Morgagni疝更容易出现在婴儿期,Morgagni疝通常在成人中出现。
● 膈肌上下病变和创伤性膈疝可能导致与BH疝出的同样类型的器官在胸腔内突出。
● 疝内容物嵌顿可致铰窄、肠扭转,漏诊他们导致的坏死,可能会危及病人生命。
● 参考文献
● Mullins ME, Stein J, Saini SS, Mueller PR. Prevalence of incidental Bochdalek’s hernia in a large adult population. AJR Am J Roentgenol. 2001;177(2):363-366.