Acute oesophageal necrosis syndrome

An 89-year-old woman with a known hiatus hernia presented to the accident and emergency department with acute onset epigastric pain. CT of the thorax and abdomen revealed a large hiatus hernia with mesentero-axial volvulus but no evidence of strangulation. A large aneurysmal aortic arch and descending aorta were visible with associated mural thrombus. As the pain was worsening, following discussion with the patient, the decision to operate was taken. The hiatus hernia was successfully reduced and the stomach looked healthy. The oesophagus, however, appeared black almost throughout its entire length consistent with acute oesophageal necrosis syndrome, a rare and lethal disease. Left lateral thoracotomy followed by cervicotomy was performed to retrieve a healthy oesophageal segment, which was anastomosed to the cardiac end of the stomach. Despite treatment in the intensive care unit, the patient's condition progressively deteriorated and she died of multiorgan failure 12 days later.