Unusual Presentation of Boerhaaveʼs Syndrome: Self-Limiting Spontaneous Perforation of the Mid-Esophagus

Abstract

Spontaneous rupture of the esophagus as described by Dr. Boerhaave in 1724, is associated with high mortality, 36 to 40%. We report a patient who suffered an unusual spontaneous perforation of the midesophagus and survived with conservative therapy. An 84-year-old man presented with persistent chest pain; 2 hours earlier while eating pork he felt that it got stuck in his lower chest. He noted that anything he drank came back up though he could swallow his saliva. He had no history of heartburn, chronic cough, or regurgitation. His dentition was good. There was no history of hematemesis, melena, fever, or chills. There was no significant surgical history. Examination: bp-155/76, p-83, T-36.8, r-18/min, wt-120 lbs. He was alert and oriented. The neck, heart, lungs, and abdomen were unremarkable. Labs: WBC-7,100, Hg-15.3. At EGD, immediately upon entering the esophagus, a deep linear rent with exposed muscle was seen leading down to two lumens (photo A). A pediatric endoscope passed through a lumen into the stomach which appeared normal with no injury at the cardia. CT scan demonstrated a large hiatal hernia and a significant amount of gas within the mediastinum, but no effusion. Water-soluble contrast esophagogram showed extravasation of contrast in the mid esophagus slightly inferior to the aortic arch (photo B). The patient was treated conservatively with intravenous antibiotics. On day 6, repeat esophagogram revealed a smooth focal narrowing at the level of the aortic arch but no contrast extravasation (photo C). The patient was discharged on day 11 tolerating clear liquids and off antibiotics. The Dutch admiral described by Dr. Boerhaave had a history of being a glutton. In that era, it was common to induce vomiting to allow immediate indulgence in more food. The admiral was found to have olive oil and roast duck in his left pleural cavity. Spontaneous rupture of the esophagus is rare and occurs predominantly in the distal esophagus. In the past it was almost universally fatal. The first drainage of mediastenitis was recorded in 1941. Successful surgical closure of esophageal perforation was performed by Dr.Barrett in 1947. We described a patient who had an even more unique presentation: spontaneous perforation of the mid-esophagus. Surprisingly he did very well with just conservative therapy and was discharged after only eleven days.1808_A Figure 1. Esophageal wall rent with perforation1808_B Figure 2. Contrast esophagogram with contrast extravasation (day 0)1808_C Figure 3. Contrast esophagogram without contrast extravasation (day 6)