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Prenatal diagnosis of an acardiac twin

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Boerhaave’s syndrome is the spontaneous perforation of the esophagus resulting from a sudden increase in intraesophageal pressure combined with negative intrathoracic pressure. It is a rare condition, with an annual… Click to show full abstract

Boerhaave’s syndrome is the spontaneous perforation of the esophagus resulting from a sudden increase in intraesophageal pressure combined with negative intrathoracic pressure. It is a rare condition, with an annual incidence of only 3.1 cases/ 1,000,000 population. Approximately 15% of esophageal perforations occur spontaneously, and the mortality rate exceeds 40%. Although Boerhaave’s syndrome-related perforation occurs most commonly in the posterolateral intrathoracic aspect of the esophagus, it can also occur in the cervical and intra-abdominal regions. The condition results in mediastinal contamination by gastric contents, precipitating chemical mediastinitis, with the possibility of evolution to bacterial infection and necrosis. Patients with Boerhaave’s syndrome typically develop signs and symptoms of severe chest pain and subcutaneous emphysema. However, one third of such patients develop atypical symptoms or are admitted to the hospital with respiratory failure or shock. Patients with cervical perforations can present with local pain, dysphagia, and dysphonia, as well as tension on sternocleidomastoid muscle palpation and crackles due to subcutaneous emphysema. In addition to Boerhaave’s syndrome, the differential diagnoses of chest and abdominal pain should include myocardial infarction, pulmonary embolism, aortic dissection, and pancreatitis. Conventional radiography, barium swallow, and, especially, contrast-enhanced CT are of great value for the timely detection of Boerhaave’s syndrome. CT shows the lungs, mediastinum, pleura, and aorta in greater detail, as well as having greater sensitivity in the detection of fluid collections. The findings corroborating rupture include esophageal edema with parietal thickening; perilesional fluid collections with or without a gaseous component; mediastinal widening; and fluid or air in the pleural and retroperitoneal spaces. In cases of esophageal rupture, the basic therapeutic options include conservative treatment, endoscopic procedures, and surgery. The conservative treatment consists in the interruption of oral food intake, together with fluid administration, enteral nutrition, antibiotic therapy, the use of beta-blockers, and drainage of the perilesional collections. Endoscopic therapy with stent placement can be reserved for cases in which there is early diagnosis, without contamination. Finally, the indication for surgical treatment, which varies from local debridement to the extensive resection of the esophagus, depends on factors such as the extent of the rupture, concomitant diseases, and the presence of contamination or signs of sepsis.

Keywords: boerhaave syndrome; diagnosis; diagnosis acardiac; prenatal diagnosis; esophagus; acardiac twin

Journal Title: Radiologia Brasileira
Year Published: 2018

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