An abrupt onset of chest pain and shock
A 71-year-old man with a past medical history of hypertension and hypothyroidism was admitted to the medical floor with right foot pain and swelling due to DVT and suspected cellulitis. On the second day of his hospital stay, he suddenly developed chest pain, diaphoresis and hypotension. He rapidly became tachycardic and hypoxic with severe respiratory distress. He had been vomiting earlier in the day. Stat ECG showed sinus tachycardia without signs of ischemia. Chest x-ray demonstrated bilateral opacities due to atelectasis versus effusion. Point-of-case ultrasound was severely limited by artifact and unable to assess cardiac function. He was transferred to the ICU for emergent intubation. Arterial and central venous catheters were inserted. Shock was refractory to escalating doses of 4 vasopressors, including norepinephrine, epinephrine, phenylephrine and vasopressin. An orogastric tube was inserted after intubation that drained over 1 liter of dark-brown-black liquid concerning for GI bleed. Three units of packed red blood cells and one unit of FFP were transfused with slight hemodynamic improvement. GI was consulted with plan for EGD the following morning.
The next morning, the critical care team noted an elevated peak inspiratory pressure (PIP) of 55 cm H20 and plateau pressure of 34 cm H20. The I:E ratio was adjusted to lengthen inspiratory time with improvement in PIP to 35 cm H20. The endotracheal tube was advanced due to a high position and a follow up chest x-ray 12 hours after intubation now demonstrated large bilateral hydropneumothorax. Preparation was made to emergently insert two pigtail thoracostomy tubes; however, the patient sustained a PEA arrest prior to insertion. CPR was initiated and bilateral needle decompression was performed at the first pulse check, producing a rush of air, and compressions were resumed. After 2 rounds of CPR, dark black liquid began to drain from the needle thoracostomy sites. ROSC was achieved followed by sterile placement of bilateral pigtail thoracostomy tubes with return of 5 liters of dark liquid. Given the appearance of the pleural fluid, there was serious concern for esophageal perforation complicated by septic shock. CT of the chest, abdomen and pelvis did not show signs of esophageal perforation, but unfortunately, the orogastric tube used for contrast administration was not withdrawn far enough to adequately evaluate the esophagus. An esophagram was subsequently performed that showed distal contrast leak, confirming the diagnosis. The amylase level of the pleural fluid was also very elevated, consistent with gastrointestinal source (3,334 U/L). Thoracic surgery was consulted, but given persistent dependence on high doses of 4 vasopressors, he was determined to be too unstable for operative intervention. Instead, after thorough discussion of the risks and benefits with the patient’s family, the gastroenterology team performed endoscopy and esophageal stent placement. EGD demonstrated ischemic changes in the middle of the esophagus as well as a full-thickness perforation measuring 4 cm located at the distal esophagus and GE junction with freely visible contamination of the mediastinum. An esophageal stent was inserted under endoscopic guidance and sutured in place. Despite this, the patient continued to deteriorate hemodynamically over the next 48 hours and subsequently died with his family present at bedside.
The life-threatening causes of chest pain are commonly discussed in the emergency department. While frequently added to the differential diagnosis, Boerhaave syndrome, or spontaneous transmural esophageal rupture, is rarely encountered. Mackler’s triad, consisting of chest pain, vomiting and subcutaneous emphysema is classically described, yet present in only 5 percent of patients with esophageal rupture. This patient’s presentation was even more unique: he was diagnosed clinically with Boerhaave syndrome during cardiac arrest after needle decompression of bilateral tension hydropneumothorax.
Esophageal rupture occurs when there is an abrupt increase in intraluminal pressure within the esophagus, typically due to forceful vomiting. When the esophagus ruptures, gastric enzymes are released that can violate the pleura leading to hydropneumothorax, as in this case. Contamination of the mediastinum with gastric contents causes local inflammation of the mediastinum and can lead to septic shock and multiorgan failure, which further increases mortality. Boerhaave syndrome should be considered when mediastinal widening, subcutaneous emphysema, mediastinal shift, pneumothorax or hydrothorax is present. The gold standard for diagnosis is esophagram or CT scan with water-soluble oral contrast. However, this is limited by a false negative rate of 15-25%, as observed in this patient who had an orogastric tube in place. Typically, mediastinal free air is observed on CT chest in > 90 percent of cases. However, it is important to note that this patient had a large, 4 cm distal esophageal tear, yet mediastinal air was absent. Instead, needle decompression and tube thoracostomy quickly established the diagnosis of Boerhaave syndrome in this patient when a large volume of biliary contents with a high amylase level was drained from the pleural cavity. Rapid diagnosis of Boerhaave syndrome is essential, as prompt antibiotic administration and surgical or endoscopic intervention is necessary for survival. When intervention is delayed frorm 12 hours to over 24 hours, mortality increases from 36 percent to greaterr than 80 percent.
A high index of suspicion for esophageal rupture is essential for diagnosis of this rare and highly fatal condition. This patient reminds us that Boerhaave syndrome should always remain on the differential diagnosis for the life-threatening causes of chest pain.
Lieu M et al. Tension hydropneumothorax as the initial presentation of Boerhaave syndrome. Respiratory Medicine Case Reports 2018;25:100-103.
Vial and Whyte. Boerhaave’s Syndrome: diagnosis and treatment. Surgical Clinics of North America 2005;85(3):515-524.