Spontaneous perforation of the esophagus - Boerhaave syndrome

History

Imaging

Discussion



Clinical History


A heavily drunk 35 year-old man was first admitted at a general hospital for an acute epigastric pain following an episode of emesis. Clinical examination found signs of hypoxemia and neck crepitation. Lung sounds was bilaterally diminished. There was no fever. CT was performed in emergency. It showed bilateral pleural effusion, left pneumothorax and subcutaneous emphysema. Perforation of the esophagus was suspected. An hour later,the patient was transferred in our hospital and a water soluble EG series confirmed the diagnosis. Surgery was immediately performed. A primary closure was practiced, combined with both a gastrostomy and a jejunostomy. Chest-tubes were placed. Then the patient was transferred to the intensive care unit for hyperalimentation and intraveinous antibiotics.



Imaging 



Discussion 


Boerhaave syndrome is a rare disease resulting from a spontaneous esophageal perforation after an effort of vomiting. In most cases, patients are men around 50, with a past history of alcohol intoxication or a neurologic affection. Usual site of perforation is the postero-lateral left wall of the distal esophagus (anatomical zone of weakness) The pathogenesis of rupture may be a disturbance of esophageal mobility : upper esophagus sphincter remains contracted during vomiting. This leads to increased intraesophageal pressure, then rupture occurs. Diagnosis is based on both clinical and radiological findings. The triad of Mackler includes : emesis, strong sudden chest pain and subcutaneous emphysema. Plain film findings are pneumomediastinum(V-Naclerio sign), hydropneumothorax and subcutaneous emphysema. Esophageal rupture may be confused with myocardial infarction, dissecting anevrysm or a perforated ulcer. In atypical clinical presentation, CT is specific and confirms the diagnosis. A water soluble contrast EG series can also be requested by the surgeon to confirm the site of the perforation, communicating with the left pleural space. This is a life-threatening condition, because mediastinum contamination by microorganism, gastric acid and digestive enzymes may complicate by mediastinitis and septic shock. Early diagnosis and prompt treatment are essential for survival. Without any treatment death occurs in 90% cases during the first 48 hours. It occurs in 36% with surgical treatment. Many surgical strategies have been described. Optimal treatment can be performed in less than 24 hours perforations. It includes closure of the perforation, pleural aspiration, mediastinal toilet and drainage, and a long-term antibiotic therapy. This seems to give the patient the best chance for complete healing.