A 43-year-old man presents to the emergency department with severe abdominal pain and substernal chest pain. The patient’s symptoms began approximately 12 hours earlier after he returned from a party where he consumed a large amount of alcohol that made him ill. Subsequently, he vomited several times and then went to sleep. A short time thereafter, he was awakened with severe pain in the upper abdomen and substernal area. His past medical history is unremarkable, and he is currently taking no medications. The patient appears uncomfortable and anxious. His temperature is 38.8°C (101.8°F), pulse rate 120 beats/min, blood pressure 126/80 mm Hg, and respiratory rate 32 breaths/min. The findings from an examination of his head and neck are unremarkable. The lungs are clear bilaterally with decreased breath sounds on the left side. The cardiac examination reveals tachycardia and no murmurs, rubs, or gallops. The abdomen is tender to palpation in the epigastric region, with involuntary guarding. The results of a rectal examination are normal. Laboratory studies reveal that his white blood count is 26,000/mm3 and that his hemoglobin, hematocrit, and electrolyte levels are normal. The serum amylase, bilirubin, aspartate transaminase (AST), alanine transaminase (ALT), and alkaline phosphatase values are within normal limits. A 12-lead electrocardiogram shows sinus tachycardia. His chest radiograph reveals moderate left pleural effusion, a left pneumothorax, and pneumomediastinum.
What is the most likely diagnosis?
What is your next step?
ANSWERS TO CASE 3: Esophageal Perforation
Summary: A 43-year-old man presents with a spontaneous thoracic esophageal perforation (Boerhaave syndrome). The patient has a left pneumothorax and exhibits a septic process from the mediastinitis.
• Most likely diagnosis: A spontaneous esophageal rupture (Boerhaave syndrome).
• Next step: Management of the airway, breathing, and circulation (ABCs), including the placement of a left chest tube, fluid resuscitation, and the administration of broad-spectrum antibiotics, followed by a water-soluble contrast study of the esophagus to confirm esophageal perforation and identify its location.
1. Recognize the clinical settings, early signs and symptoms, and complications of esophageal perforation.
2. Learn the diagnostic and therapeutic approach to a suspected esophageal perforation.
This is a young patient with forceful retching followed by severe chest and abdominal pain, and now with fever. The chest radiograph reveals pneumomediastinum, with air entering from the esophagus into the mediastinum.
This patient’s clinical presentation is classic for a spontaneous esophageal perforation; however, delay in diagnosis and treatment can still occur because many physicians do not have extensive experience in the evaluation and treatment of this problem. Maintaining a high index of suspicion and pursuing an early diagnosis and early treatment are essential.
APPROACH TO: Suspected Esophageal Perforation
BOERHAAVE SYNDROME: Spontaneous esophageal syndrome.
PNEUMOMEDIASTINUM: Air within the mediastinal space. Esophageal perforation remains a surgical emergency. A delay in diagnosis leads to increased morbidity and mortality; therefore, a high index of suspicion should be maintained. Most esophageal perforations are iatrogenic and occur during diagnostic and therapeutic endoscopic procedures. Spontaneous esophageal perforations, also referred to as Boerhaave syndrome, account for approximately 15% of all causes of esophageal perforation and carry a worse prognosis than iatrogenic perforations.
ESOPHAGEAL PERFORATION SEVERITY SCORE: A scoring system developed to help prognosticate outcomes and guide the selection of treatment for patients with esophageal perforation (based on data from Abbas et al). Patients with clinical scores of less than 2 had 23% complications and mortality of 2%; clinical scores of 3 to 5 were associated with 32% complications and 3% mortality; clinical scores of greater than 5 were associated with 21% complications and 7% mortality. This group also observed that patients with lower scores managed operatively had worse outcomes than those managed nonoperatively, thus suggesting that low-score patient may benefit from an initial course of nonoperative treatment that may include some or all of these treatments: nothing by mouth, broad-spectrum antibiotics, CT-guided drainage, and placement of covered stents by endoscopy.
The development of an acute onset of chest pain after an episode of vomiting is typical of Boerhaave syndrome. Other symptoms that may be present include shoulder pain, dyspnea, and midepigastric pain. Findings from a physical examination, screening radiographs, and laboratory results depend on (1) the integrity of the mediastinum, (2) the location of the perforation, (3) and the time elapsed since the perforation. Seventy-five percent of patients present with a pleural effusion indicating disruption of the mediastinal pleura. Contamination of the mediastinum with esophageal luminal contents often leads to mediastinitis and chest pain. A delay in treatment leads to sepsis with signs of systemic infection (tachycardia, fever, and leukocytosis). Perforation into the mediastinum leads to pneumomediastinum that can be seen on a chest radiograph and subcutaneous emphysema that can be demonstrated by physical examination. Because most spontaneous esophageal ruptures occur in the distal third of the esophagus above the GE junction, two-thirds of patients present with a left pleural effusion. The time from perforation to the time of diagnosis is of paramount importance to the selection of treatment approaches and ultimate outcome (Table 3–1).
Table 3–1 • CLINICAL PROGRESSION OF SPONTANEOUS ESOPHAGEAL PERFORATION
Traditionally, the best initial diagnostic test to confirm an esophageal rupture is a water-soluble contrast esophagogram, which identifies perforation in 90% of cases. Water-soluble contrast is preferred during the initial examination because it causes less mediastinal irritation than barium if a large leak is discovered. Water-soluble contrast (Gastrografin) esophagram should be obtained with the patient in the right lateral decubitus position to improve its diagnostic sensitivity, and, if no leak is visualized, barium contrast may be given to confirm the absence of a leak. In recent years, CT scans of the abdomen and chest are being increasingly applied as the initial diagnostic studies in many centers. Once perforation is diagnosed, the initial treatments include prompt resuscitation (directed toward the ABCs), antibiotics therapy, and preparation for operative therapy. The treatment principles for spontaneous esophageal perforation include the control of esophageal content leakage by drainage, with or without primary repair of perforation site (Figure 3–1). Over the past decade, the management of esophageal perforations has evolved toward a more selective approach, in that only some patients are selected to undergo surgical repair. Drainage of contamination from the perforations can be accomplished in some patients with CT-guided placement of drainage catheters. In some patients, the placement of covered intraluminal stents by endoscopy can be performed in addition to the drainage procedures. The selection of patients for each of these treatments has not been evaluated by any randomized controlled trials, and patient management is largely dictated by patient condition, surgeon experience, and hospital resource availability.
Figure 3–1. Algorithm for managing esophageal perforation.
3.1 A 26-year-old man is brought into the emergency center for severe chest pain and upper abdominal pain. He is diagnosed with esophageal perforation. Which of the following is the most likely etiology of his condition?
C. Iatrogenic (endoscopy)
D. Spontaneous rupture (Boerhaave syndrome)
E. Caustic injury
3.2 A 60-year-old man has a 10-year history of achalasia. His dysphagia has been worsening, and he underwent an esophageal dilation; shortly after this procedure, he develops acute chest pain, tachycardia, and fever 6 hours after esophageal dilatation for achalasia. Which of the following diagnostic procedures is most appropriate for this patient?
A. A barium esophagogram
B. A Gastrografin esophagogram
D. Chest x-ray
E. Upright abdominal x-ray
3.3 The family member of the patient in Question 3.2 is very concerned about the prognosis. Which of the following is the most important factor that determines the outcome in esophageal perforation?
A. The size of the perforation
B. The presence of fever
C. Whether a meal has been ingested recently
D. The duration between the event and the corrective surgery
3.4 After eating some stale pizza, a 21-year-old college student presents to the emergency department with a 24-hour history of nausea, vomiting, and severe chest pain. An esophageal perforation is diagnosed by a contrast study, with the best clinical impression of its onset occurring approximately 12 hours previously. Which of the following is the best treatment?
A. Primary surgical repair
B. Esophageal diversion
C. Endoscopic stent placement
D. Gastrostomy tube and observation
E. Continued observation for spontaneous healing
3.1 C. Iatrogenic causes are the most common causes of esophageal perforations. Diagnostic endoscopy is associated with the risk of cervical esophageal perforation and therapeutic endoscopy (pneumatic dilatation and variceal injections/banding) is most commonly associated with perforation of the distal esophagus.
3.2 B. Barium study is the most sensitive diagnostic method; however, barium leak is associated with mediastinitis and peritonitis. A Gastrografin (water-soluble) esophagogram is the most appropriate initial diagnostic study that is more than 90% accurate in identifying a perforation. In some centers, CT scan is done as the initial evaluation for patients with suspected thoracic/abdominal esophageal perforations.
3.3 D. The outcome of esophageal perforation is directly related to the elapsed time between the perforation and the treatment. Although the other answer choices are also influential factors in outcome, the time delay is most important as it is associated with infection and sepsis. See the esophageal perforation severity score under “Definitions” for details of other influential factors of outcome.
3.4 A. Primary esophageal repair is generally performed when the perforation is less than 24 hours in duration. In patients in good physiologic condition, surgical repair is generally used regardless of the duration of perforation. Endoscopic treatment approach is being increasingly applied for patients with esophageal perforations, but for this young and otherwise healthy individual, surgical repair should lead to good outcome and recovery.
Spontaneous esophageal perforation should be suspected in a patient with chest pain after vomiting, subcutaneous emphysema found on physical examination, and left-sided effusion demonstrated on a chest radiograph.
A high index of suspicion is needed because a delay in diagnosis directly compromises patient outcome.
Most spontaneous esophageal ruptures occur in the distal third of the esophagus.
Most iatrogenic esophageal perforations are associated with endoscopy.
Abbas G, Schuchert MJ, Pettiford BL, et al. Contemporaneous management of esophageal perforations. Surgery. 2009;146:749-756.
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Jobe BA, Hunter JG, Peters JH. Esophagus and diaphragmatic hernia. In: Brunicardi FC, Andersen DK, Billiar TR, et al, eds. Schwartz’s Principles of Surgery. 9th ed. New York, NY: McGraw-Hill; 2010: 803-887.
Sepesi B, Raymond DP, Peters JH. Esophageal perforation: surgical, endoscopic, and medical management strategies. Curr Opin Gastroenterol. 2010;26:379-383.