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博雷尔哈韦综合征

Boerhaave Syndrome

作者信息

Turner Anisha R., Collier Sara A., Turner Sherry D.

机构信息

Louisiana State University Shreveport

Oklahoma Dept of Corrections

PMID:28613559
Abstract

Boerhaave syndrome typically occurs after forceful emesis and retching. Boerhaave syndrome is a transmural perforation of the esophagus and should be distinguished from Mallory-Weiss syndrome, a nontransmural esophageal tear also associated with vomiting. Since the perforation occurs with emesis, Boerhaave syndrome is usually not truly spontaneous, but this term helps distinguish it from iatrogenic perforation. Vomiting is the most common cause, but any activity that increases intraesophageal pressure can result in this syndrome. This condition can manifest in patients with a typically functioning esophagus, yet there is a subgroup where specific esophageal abnormalities or pathology are identified. Boerhaave syndrome accounts for 10% to 15% of all esophageal perforations.  Diagnosis of this condition can be challenging because the presentation can vary significantly, thus requiring an index of suspicion. Boerhaave syndrome is classically associated with the Mackler triad of vomiting, chest pain, and subcutaneous emphysema. However, patients rarely present with all of these symptoms and often have vague, nonspecific complaints. This can contribute to a delay in diagnosis and poor outcomes. Boerhaave syndrome is one of the most lethal gastrointestinal tract disorders, with a mortality rate of up to 60% with intervention, increasing to nearly 100% without intervention. Treatment is varied and depends on the time of diagnosis and the patient’s clinical condition at presentation. Management can range from conservative management to major surgical resection.

摘要

博雷尔哈夫综合征通常发生在剧烈呕吐和干呕之后。博雷尔哈夫综合征是食管的全层穿孔,应与马洛里-魏斯综合征相鉴别,后者是一种也与呕吐相关的非全层食管撕裂。由于穿孔与呕吐有关,博雷尔哈夫综合征通常并非真正的自发性,但这个术语有助于将其与医源性穿孔区分开来。呕吐是最常见的原因,但任何增加食管内压力的活动都可能导致这种综合征。这种情况可发生在食管功能正常的患者中,但也有一部分患者存在特定的食管异常或病变。博雷尔哈夫综合征占所有食管穿孔的10%至15%。这种疾病的诊断可能具有挑战性,因为临床表现差异很大,因此需要有怀疑指数。博雷尔哈夫综合征典型地与呕吐、胸痛和皮下气肿的麦克勒三联征相关。然而,患者很少同时出现所有这些症状,且常常有模糊、非特异性的主诉。这可能导致诊断延迟和预后不良。博雷尔哈夫综合征是最致命的胃肠道疾病之一,接受干预时死亡率高达60%,若不干预则死亡率几乎升至100%。治疗方法多样,取决于诊断时间和患者就诊时的临床状况。处理范围可从保守治疗到大型手术切除。