Boerhaave syndrome

特発性食道破裂

WordNet

1. a pattern of symptoms indicative of some disease
2. a complex of concurrent things; "every word has a syndrome of meanings"

PrepTutorEJDIC

1. (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2019/04/25 23:15:47」(JST)

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Boerhaave syndrome
Specialty	Gastroenterology General surgery

Esophageal rupture is a rupture of the esophageal wall. Iatrogenic causes account for approximately 56% of esophageal perforations, usually due to medical instrumentation such as an endoscopy or paraesophageal surgery. In contrast, the term Boerhaave syndrome is reserved for the 10% of esophageal perforations which occur due to vomiting.

Spontaneous perforation of the esophagus most commonly results from a full-thickness tear in the esophageal wall due to a sudden increase in intraesophageal pressure combined with relatively negative intrathoracic pressure caused by straining or vomiting (effort rupture of the esophagus or Boerhaave's syndrome). Other causes of spontaneous perforation include caustic ingestion, pill esophagitis, Barrett's esophagus, infectious ulcers in patients with AIDS, and following dilation of esophageal strictures.

In most cases of Boerhaave's syndrome, the tear occurs at the left postero-lateral aspect of the distal esophagus and extends for several centimeters. The condition is associated with high morbidity and mortality and is fatal without treatment. The occasionally nonspecific nature of the symptoms may contribute to a delay in diagnosis and a poor outcome. Spontaneous effort rupture of the cervical esophagus, leading to localized cervical perforation, may be more common than previously recognized and has a generally benign course. Preexisting esophageal disease is not a prerequisite for esophageal perforation but it contributes to increased mortality.

This condition was first documented by the 18th-century physician Herman Boerhaave, after whom it is named. A related condition is Mallory-Weiss syndrome which is only a mucosal tear.

In case of iatrogenic perforation common site is cervical esophagus just above the upper sphincter whereas spontaneous rupture as seen in Boerhaave's syndrome perforation commonly occurs in the lower (1/3)rd of esophagus.

Signs and symptoms

The classic history of esophageal rupture is one of severe retching and vomiting followed by excruciating retrosternal chest and upper abdominal pain. Odynophagia, tachypnea, dyspnea, cyanosis, fever, and shock develop rapidly thereafter.

Physical examination is usually not helpful, particularly early in the course. Subcutaneous emphysema (crepitation) is an important diagnostic finding but is not very sensitive, being present in only 9 of 34 patients (27 percent) in one series. A pleural effusion may be detected.

Mackler's triad includes chest pain, vomiting, and subcutaneous emphysema, and while it is a classical presentation, it is only present in 14% of people.

Pain can occasionally radiate to the left shoulder, causing physicians to confuse an esophageal perforation with a myocardial infarction.

It may also be audibly recognized as Hamman's sign.

Pathophysiology

Esophageal rupture, in Boerhaave syndrome is thought to be the result of a sudden rise in internal esophageal pressure produced during vomiting, as a result of neuromuscular incoordination causing failure of the cricopharyngeus muscle (a sphincter within the pharynx) to relax. As the intra-oesophageal pressure increases, the bolus within the oesophagus has nowhere to go superiorly (as the cricopharyngeus fails to relax) which causes the oesophagus to rupture. The syndrome is commonly associated with the consumption of excessive food and/or alcohol as well as eating disorders such as bulimia.

The most common anatomical location of the tear in Boerhaave syndrome is at left posterolateral wall of the lower third of the esophagus, 2–3 cm before the stomach.

Currently, the most common cause of esophageal perforation is iatrogenic. However, iatrogenic perforations, while still constituting a serious medical condition, are easier to treat and less prone to complications, particularly mediastinitis and sepsis. This is because they usually do not involve contamination of the mediastinum with gastric contents.

Diagnosis

Upright chest radiography showing mediastinal air adjacent to the aorta and tracking cephalad adjacent to the left common carotid artery. This patient presented to the Emergency department with severe chest pain after eating.

Axial CT image through the upper chest showing extraluminal air (pneumediastinum) surrounding the trachea and esophagus

Sagittal remormatted CT image showing discontinutity in the wall of the posterolateral aspect of the distal esophagus

The diagnosis of Boerhaave's syndrome is suggested on the plain chest radiography and confirmed by chest CT scan. The initial plain chest radiograph is almost always abnormal in patients with Boerhaave's syndrome and usually reveals mediastinal or free peritoneal air as the initial radiologic manifestation. With cervical esophageal perforations, plain films of the neck show air in the soft tissues of the prevertebral space.

Hours to days later, pleural effusion(s) with or without pneumothorax, widened mediastinum, and subcutaneous emphysema are typically seen. CT scan may show esophageal wall edema and thickening, extraesophageal air, periesophageal fluid with or without gas bubbles, mediastinal widening, and air and fluid in the pleural spaces, retroperitoneum or lesser sac.

The diagnosis of esophageal perforation could also be confirmed by water-soluble contrast esophagram (Gastrografin), which reveals the location and extent of extravasation of contrast material. Although barium is superior in demonstrating small perforations, the spillage of barium sulfate into the mediastinal and pleural cavities can cause an inflammatory response and subsequent fibrosis and is therefore not used as the primary diagnostic study. If, however, the water-soluble study is negative, a barium study should be performed for better definition.

Endoscopy has no role in the diagnosis of spontaneous esophageal perforation. Both the endoscope and insufflation of air can extend the perforation and introduce air into the mediastinum.

Patients may also have a pleural effusion high in amylase (from saliva), low pH, and may contain particles of food.

Differential diagnosis

Common misdiagnoses include myocardial infarction, pancreatitis, lung abscess, pericarditis, and spontaneous pneumothorax. If esophageal perforation is suspected, even in the absence of physical findings, chest xray, water soluble contrast radiographic studies of the esophagus and a CT scan should be promptly obtained. In most cases, non-operative management is administered based on radiological evidence contained in mediastinal collection.

Treatment

With the exception of a few case reports describing survival without surgery, the mortality of untreated Boerhaave syndrome is nearly 100%. Its treatment includes immediate antibiotic therapy to prevent mediastinitis and sepsis, surgical repair of the perforation, and if there is significant fluid loss it should be replaced with IV fluid therapy since oral rehydration is not possible. Even with early surgical intervention (within 24 hours) the risk of death is 25%.

Further reading

• Radiology Esophageal rupture
• Arens, Ann; Ben-Youssef, Leila; Hayashi, Sandra; Smollin, Craig (2016). "Esophageal Rupture After Ghost Pepper Ingestion". The Journal of Emergency Medicine. 51: e141–e143. doi:10.1016/j.jemermed.2016.05.061.
• Henderson, John A.M.; Péloquin, AndréJ.M. (1989). "Boerhaave revisited: Spontaneous esophageal perforation as a diagnostic masquerader". The American Journal of Medicine. 86 (5): 559–67. doi:10.1016/0002-9343(89)90385-9. PMID 2653030.
• Larrieu, AJ; Kieffer, R (1975). "Boerhaave syndrome: report of a case treated non-operatively". Annals of Surgery. 181 (4): 452–4. doi:10.1097/00000658-197504000-00015. PMC 1343787. PMID 1130863.
• Patton, Anthony S.; Lawson, Dexter W.; Shannon, James M.; Risley, Thomas S.; Bixby, Frank E. (1979). "Reevaluation of the Boerhaave syndrome". The American Journal of Surgery. 137 (4): 560–5. doi:10.1016/0002-9610(79)90131-4. PMID 426207.
• Ivey, Tom D.; Simonowitz, David A.; Dillard, David H.; Miller, Donald W. (1981). "Boerhaave syndrome". The American Journal of Surgery. 141 (5): 531–3. doi:10.1016/0002-9610(81)90040-4. PMID 6784584.

External links

Classification	D ICD-10: K22.3 ICD-9-CM: 530.4 MeSH: D004939 DiseasesDB: 9168
External resources	MedlinePlus: 000231 eMedicine: med/233

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. ブールハーフェ症候群：食道破裂 boerhaaves syndrome effort rupture of the esophagus
• 2. 食道穿孔の外科的マネージメント surgical management of esophageal perforation
• 3. 非医原性外傷性食道穿孔のマネージメント management of non iatrogenic traumatic esophageal perforations
• 4. 小児および思春期における自然縦隔気腫 spontaneous pneumomediastinum in children and adolescents
• 5. 成人における神経性大食症症および気晴らし食い症候群：内科的合併症およびそのマネージメント bulimia nervosa and binge eating disorder in adults medical complications and their management

English Journal

• Endoscopic treatment of an oesophageal rupture using an over-the-scope clip (OTSC): A case report.

• Ngo E1, Born P2, Strebel H1.
• Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology.Arab J Gastroenterol.2015 Jul 20. pii: S1687-1979(15)00041-6. doi: 10.1016/j.ajg.2015.06.005. [Epub ahead of print]
• Oesophageal rupture (Boerhaave syndrome) is a rare but serious complication after vomiting. Early intervention is mandatory in order to avoid a deleterious outcome for the patient. This case report describes the endoscopic approach using an over-the-scope clip.Copyright © 2015. Published by Elsevie
• PMID 26206430

• Spontaneous pneumomediastinum.

• Gupta A1, Rizvi I2, Srivastava VK3, Debnath J4, Kapur BN5, Khanna VN6, Aziz A7.
• Medical journal, Armed Forces India.Med J Armed Forces India.2015 Jul;71(Suppl 1):S85-8. doi: 10.1016/j.mjafi.2013.03.008. Epub 2013 Jun 6.
• PMID 26265882

• Case Report: Pulmonary Papillomatosis in a Patient Presenting with Cough and Hemoptysis.

• Zhang Z1, Chang M2, Moreta-Sainz LM3.
• The Permanente journal.Perm J.2015 Summer;19(3):e125-7. doi: 10.7812/TPP/14-192.
• An emergency ultrasound performed at bedside helped to confirm and to expedite the diagnosis of esophageal perforation in a 23-year-old man. To our knowledge, this is the first published report of using ultrasound as an aid in the diagnosis of Boerhaave syndrome by diagnosing pneumomediastinum in an
• PMID 26176580

Japanese Journal

• 外科学の古典を読む(第20回)Boerhaave症候群の原典

• 島田 謙,相馬 一亥
• 外科 74(8), 850-854, 2012-08
• NAID 40019395303

• 食道損傷の診断と治療--特発性食道破裂を中心に (特集 胸部外科診療に役立つ疾患別最新データ) -- (食道領域)

• 千野 修,小澤 壯治
• 胸部外科 64(8), 758-763, 2011-07
• NAID 40018928814

Related Links

• Boerhaave syndrome - Wikipedia, the free encyclopedia

Esophageal rupture (also known as Boerhaave's syndrome) is rupture of the esophageal wall. 56% of esophageal perforations are iatrogenic, usually due to medical instrumentation such as an endoscopy or paraesophageal surgery.

• Boerhaave Syndrome - E-Medicine - Medscape

15 Mar 2013 ... Boerhaave Syndrome. Boerhaave first described the spontaneous rupture of the esophagus in 1724.

Related Pictures

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★リンクテーブル★

リンク元	「特発性食道破裂」
関連記事	「syndrome」

「特発性食道破裂」

　　[★]

英

spontaneous esophageal rupture, spontaneous rupture of the esophagus,

同

ブールハーフェ症候群 Boerhaave症候群 Boerhaave syndrome Boerhaave's syndrome

[show details]

ブールハーフェ症候群 : 40 件
ブールハーベ症候群 : 41 件
ボールハーヴェ症候群 : 7 件

概念

• 食道内圧が急激に上昇(嘔吐、排便、分娩、咳)による有痛性の食道破裂で、特に横隔膜直上の下部食道が長軸・線状に破裂する。
• 食道下部の左壁に好発する(周囲の支持組織を欠くため)
• 食道粘膜全層の損傷　⇔　マロリー・ワイス症候群(粘膜下層まで)

疫学

医療救急パーフェクトマニュアル改訂版 p.47

• 40-50歳の男性に多い。

好発部位

医療救急パーフェクトマニュアル改訂版 p.47

• 食道下部左壁

症状

• 激烈な胸痛、心窩部痛、呼吸困難を訴え、縦隔気腫、膿気胸を認める。
• 二次的に縦隔気腫、皮下気腫、縦隔炎、胸水、気胸などが起こる。

身体所見

• 聴診：肺呼吸音減弱、音声振盪減弱

検査

• 胸腹部単純X線写真　＋　水溶性造影剤(ガストログラフィン)を用いた食道造影

• 胸腹部単純X線写真：縦隔拡大、縦隔の変異、縦隔気腫像
• 食道造影：ガストログラフィンの食道外漏出

鑑別疾患

医療救急パーフェクトマニュアル改訂版 p.47

• 急性心筋梗塞、急性大動脈解離

確定診断

医療救急パーフェクトマニュアル改訂版 p.47

• ガストログラフィンによる食道造影

治療

• 発症早期：創の閉鎖縫合

予後

• 縦隔炎は重篤
• 未治療24時間以上で死亡率70%以上(医療救急パーフェクトマニュアル改訂版 p.47)

国試

• 096A032

「syndrome」

　　[★]

• n.

• 症候群