Spontaneous Esophageal Rupture without Risk Factors

Boerhaave syndrome is a spontaneous rupture of the esophagus. It results from a sudden increase in esophageal pressure combined with negative intrathoracic pressure as seen with severe straining or vomiting. Esophageal perforation is extremely rare with an incidence of approximately 3.1 per 1,000,000 per year with a mortality rate estimated to be 20-50%. The rare occurrence and fatal nature of an esophageal perforation makes this syndrome a difficult yet important diagnosis to consider in the clinical setting. This case presents a previously healthy 47-year-old gentleman who presented to a local emergency department with syncope, vomiting, and chest pain sequentially. He reported a 24-hour history of dark stools followed by syncope, and when he regained consciousness he had three bouts of retching and expulsion of coffee-ground vomitus. Shortly after emesis, the patient developed severe and continuous pain located in his central chest that radiated to his back. Upon arrival to the hospital he was tachycardic. Blood tests revealed metabolic acidosis, increased white blood cell count, elevated lactate, and significant anemia. The patient’s hemoglobin was 7.7 gm/dL, a significant drop from the patient’s baseline level of 14. The patient received 1 unit of packed red blood cells and was sent for imaging studies. Chest X-ray and CT imaging revealed free air in the mediastinum, and a subsequent Gastrografin esophagogram study revealed a lower esophageal perforation proximal to gastroesophageal junction. The patient was rushed to the operating room for emergency surgery. An esophagogastroduodenoscopy showed no active bleeds and confirmed a distal mucosal defect suggestive of perforation. The surgery team then performed a left thoracotomy with intercostal muscle harvest and esophageal repair, with the patient needing an additional 4 units of packed red blood cells during surgery. He tolerated the surgery well and received appropriate post-op care in the ICU. A repeat Gastrografin and barium swallow esophagogram revealed no evidence of leak, strictures, or complications from the operation. A follow up CT image of the chest, abdomen, and pelvis was performed one month after hospital discharge, and results showed complete resolution of mediastinal air without evidence of esophageal leak. This case demonstrates how a spontaneous esophageal perforation can occur in previously healthy, middle aged patients with no significant GI history or identifiable risk factors. Given this information, clinicians should consider Boerhaave Syndrome when a patient of any age presents with chest pain after an episode of emesis