主诉 病史

A 27-year-old man presents to the emergency department (ED) with a 2-day history of hyperemesis followed by episodes of hematemesis. He complains of worsening epigastric pain, diarrhea, and myalgia. The patient also describes experiencing night sweats and rigors. No melena or hematochezia is reported. He admits to being a "binge drinker" and having consumed approximately 21 cans of beer 3 days ago. The patient's relevant past medical history includes esophagitis, which was diagnosed last year and was treated with a long-term proton pump inhibitor. He has no known drug allergies and has not taken any other medications recently. He does not admit to using any illegal substances. He has a significant family history of hypertrophic obstructive cardiomyopathy (HOCM). He works as a laborer and has smoked 10 cigarettes a day for the past 6 years. Soon after presenting in the ED, the patient collapses. On physical examination, he has a temperature of 99.5°F (37.5°C). He is in sinus tachycardia, with a heart rate of 130 bpm, and his blood pressure is noted to be 118/82 mm Hg. On examination, the patient is severely dehydrated and agitated, with tenderness to palpation noted in the upper chest and neck. The lungs are clear to auscultation, and heart sounds S1 and S2 are heard, with no murmurs or added sounds. His abdomen is soft, with a tender epigastrium.        患者男性，27岁，因剧烈呕吐后呕血2天送来急诊科。此外他还有严重的上腹痛，腹泻和肌痛，夜汗和恶寒。他承认自己是“狂饮者”，三天前喝了近21罐啤酒。他相关的过去史有食管炎，于去年诊断并接受了长效PPI治疗。无药物过敏史，近期未曾服其他药物。否认服用任何非法药物。有明确的肥厚梗阻性心肌病家族史。职业为工人，过去六年中每日吸烟10支。收入急诊科不久患者就发生了虚脱。体格检查：体温 37.5°C ，窦性心动过速，心率130/min，血压：118/82 mmHg ，严重脱水，躁动不安，胸上部和颈部触诊压痛。肺部呼吸音清晰，S1、S2存在，未闻及杂音和附加音。腹部柔软，上腹部有压痛。

查体 辅查

The initial work-up includes laboratory investigations; the clinically significant results report a raised white blood cell (WBC) count of 17.9 × 103/µL (17.9 x 109/L; normal range, 4-11 x 103/µL), sodium of 147 mEq/L (147 mmol/L; normal range, 135-145 mEq/L), potassium of 2.6 mEq/L (2.6 mmol/L; normal range, 3.5-5.0 mEq/L), blood urea nitrogen of 14.5 mg/dL (5.2 mmol/L; normal range, 7-19 mg/dL), and creatinine of 1.71 mg/dL (151 µmol/L; normal range, 0.8-1.7 mg/dL). Liver function test results are slightly deranged, with the main abnormality being a raised γ-glutamyltransferase (GGT) of 716 units/L (normal range, 11-51 units/L). An electrocardiogram (ECG) is performed and shows a prolonged QT interval, with ST depression in the inferior and anterolateral leads. The patient is diagnosed with multi-organ failure caused by sepsis, and he is transferred to the high dependency unit (HDU). The patient is treated with vigorous fluid resuscitation and broad-spectrum intravenous (IV) antibiotics. Further probing of the history reveals that he has vomited 30 times in the past 24 hours. An erect posteroanterior chest radiograph is urgently obtained (see Figure 1). An abnormality on the chest radiograph prompts a computed tomography (CT) scan of the chest (see Figure 2) and the abdomen (not pictured).       最初的有明显临床意义的实验室检查报告; WBC 17.9 × 103/µL ; Na 147 mmol/L; K 2.6 mmol/L; BUN 5.2 mmol/L; Cr 151 µmol/L 肝功能轻度受损，主要异常为GGT 716 units/L 。心电图示QT间期延长，下壁，前侧壁ST段压低。病人诊断为败血症引发的多脏器衰竭并转入高护病房。给予积极补液复苏和广谱抗生素治疗。进一步病史询问发现在过去的24小时内病人呕吐有30余次。进行了紧急胸部后前位X线摄片，胸片结果的异常促使了进一步的胸腹部CT检查。

诊断 处理

Boerhaave syndrome（布尔哈佛综合征）  This 27-year-old man presented in an acutely deteriorating state and was quickly diagnosed with multi-organ failure caused by underlying sepsis. The erect chest x-ray was a key diagnostic tool in this case, as it demonstrated air along the left heart border and the aortic knob, representing pneumomediastinum and/or pneumopericardiac air (see Figure 1). This finding led to an urgent CT scan of the chest and abdomen, which found free air within the mediastinum and the pericardial sac (see Figure 2). The patient then underwent a contrast-enhanced swallow study (not available), which failed to demonstrate a patent esophageal leak, thus indicating that the rupture had probably occurred some time earlier. 27岁男性患者入院际处于急性恶化状态，很快诊断为隐匿性败血症导致的多脏器功能衰竭。在本例中立位X线胸片是关键性的诊断工具，它显示了沿着左心边缘和主动脉球的气体，提示纵隔积气和心包积气（图1）。这一发现导致了急诊胸腹CT扫描，结果提示纵隔和心包囊的游离气体。病人进行了对比增强吞咽检查，未发现食管漏，因此表明破裂可能在早前发生。

随访 讨论

Boerhaave syndrome, or spontaneous esophageal rupture, is a rare but serious condition, with a mortality rate ranging from 25% to 89%. If treatment is instituted within 24 hours of the appearance of symptoms, the mortality rates are 25%; however, mortality rates rise to greater than 65% after 24 hours without treatment and 75-89% after 48 hours without treatment.[1] The syndrome is characterized by a complete transmural rupture of the esophagus resulting from barogenic trauma (ie, forceful vomiting).[2] A sudden rise in intraluminal pressure caused by uncoordinated vomiting with pyloric closure and diaphragmatic contraction against a contracted cricopharyngeal muscle is theorized to be the underlying cause of Boerhaave syndrome.[3] Perforation usually occurs at the weakest point of the esophagus; therefore, the most common site of rupture is the left posterolateral wall of the lower third of the esophagus. This is seen in 90% of patients.[4]         布尔哈佛综合征，又称自发性食管破裂是一种少见但很严重的情况，死亡率高达25％至89％。如果在症状出现24小时内开始治疗死亡率为25％；但24小时之后未行治疗死亡率则升高至65％以上，而48％小时后仍未行治疗死亡率可为75～89％。这一综合征是以压力性创伤（如剧烈呕吐）引发食管完全性透壁破裂为特征。理论上说，幽门关闭和膈肌收缩而伴随环咽肌也同时收缩的不协调呕吐是布尔哈佛综合征的主要原因。穿通通常发生在食管的薄弱点，因此破裂最常见的部位是食管下三分之一的左后侧壁。 Although the incidence of Boerhaave syndrome is relatively rare (accounting for only 16% of all traumatic ruptures of the esophagus), it has been associated with a very heterogeneous population, affecting all races throughout the world. There is an association between overindulgence in alcohol and food, as would be expected from a condition that is associated with vomiting.Boerhaave syndrome does, however, have a significantly increased incidence in men as compared with women, with a ratio of approximately 2:1. It is also more common in the middle-aged; 80% of patients are middle-aged men. Despite this, studies consistently report Boerhaave syndrome as affecting all age groups, from neonates to those aged 90 years.          虽然布尔哈佛综合征的发病率相对较低（占全部食管外伤性破裂的16％），但涉及各种人群，影响世界上所有的人种。发病与放任饮酒和进食有关，并常在呕吐时发生。男性发病率明显高于女性，