Alykhan S. Nagji
Christine L. Lau
Presentation
A 60-year-old man with a known history of alcohol abuse and binge drinking presents to the emergency department complaining of substernal chest pain after multiple episodes of vomiting. Initial vital signs reveal sinus tachycardia along with a systolic blood pressure of 85 mm Hg. The patient is also febrile to 39.1°C. On physical examination, the patient is found to have a systolic crunching sound heard at the left sternal border (Hamman’s sign) along with subcutaneous emphysema. Laboratory tests demonstrate an elevated white blood cell count of 15,000/mm3 but are otherwise normal.
Differential Diagnosis
The combination of subcutaneous emphysema, vomiting, and chest pain comprises Mackler’s triad, a pathognomonic sign for esophageal perforation. The potential causes of esophageal perforation include the following: iatrogenic (extra- or intraluminal), trauma (i.e., penetrating or blunt injury, barotraumas, and foreign body or caustic injury), malignancy, inflammation (i.e., gastroesophageal reflux, ulceration, and Crohn’s disease), and infection. Though the most likely diagnosis in the case above is esophageal perforation secondary to barotrauma resulting in Boerhaave’s syndrome, one must be careful to rule out other cardiac, vascular, or intrathoracic pathology that may contribute to this patient’s presenting symptoms.
Presentation Continued
With respect to aforementioned case, the patient has suffered from an esophageal perforation secondary to Boerhaave’s syndrome. This is a spontaneous esophageal perforation that occurs after an episode of vomiting. It is thought to occur from a series of events that culminate in a rapid increase in intraluminal esophageal pressure, such that a transmural rupture typically occurs in the left posterolateral wall of the esophagus approximately 2 to 3 cm proximal to the gastroesophageal junction. This area of the esophagus is inherently weak as the longitudinal fibers taper before passing onto the stomach wall.
Workup
The clinical presentation of esophageal perforation is highly dependent on the etiology and location. It is important that the diagnosis be made in a timely manner. Clinical suspicion combined with imaging (i.e., plain radiography, contrast esophagography, and computed tomography [CT] with oral contrast), laboratory analysis, and in some cases direct visualization (i.e., flexible esophagoscopy) serves to confirm or refute the diagnosis of perforation.
Radiographic studies play a significant role in the establishment of the diagnosis of esophageal perforation. In the case of cervical esophageal perforations, a plain film of the lateral neck may demonstrate air in the prevertebral fascial. If a thoracic or an abdominal esophageal perforation is suspected, an upright abdominal film along with a posteroanterior (Figure 1) and lateral chest radiograph should be obtained. It stands to reason that if plain films demonstrate a pleural effusion, pneumomediastinum, subcutaneous emphysema, hydrothorax, pneumothorax, or subdiaphragmatic air, the suspicion for esophageal perforation increases. However, if the plain film is normal after a suspected esophageal injury, further workup is required.
FIGURE 1 • Chest x-ray demonstrating pneumomediastinum (arrow).
Contrast esophagography is the study of choice for the diagnosis of esophageal perforation. Two forms of contrast are available to decipher the presence and location of a perforation. Gastrograffin, being water soluble, has traditionally been the initial contrast of choice secondary to its rapid absorption after extravasation through the perforation (Figure 2). In the event that no perforation is detected with a water-soluble agent, serial dilute barium esophagography should be performed. Dilute barium should be used exclusively in patients at high risk for aspiration or if a tracheoesophageal fistula is suspected. A negative result with suspicion of perforation requires repetition of barium contrast esophagography, and possibly a CT (Figure 3) and/or esophagoscopy. Flexible esophagoscopy provides direct visualization of the perforation.
FIGURE 2 • Esophagogram demonstrating leakage of contrast (arrow) into the left chest secondary to distal esophageal perforation.
FIGURE 3 • A: Axial CT image demonstrating pneumomediastinum. B: Coronal CT image demonstrating pneumomediastinum. C: Axial CT image demonstrating air at the level of the distal esophagus and gastroesophageal junction.
Presentation Continued
After the initial assessment, history and physical examination were obtained, the patient had a posteroanterior/lateral (PA/lateral) chest radiograph that demonstrated a left pleural effusion along with mediastinal air. The patient undergoes a gastrograffin followed by dilute barium swallow, which demonstrates extravasation into the left chest consistent with a suspected esophageal perforation secondary to Boerhaave’s syndrome (Figure 2).
Diagnosis and Treatment
Therapy for esophageal perforation is dependent upon the age and health of the patient, the damage to surrounding tissues, and the underlying esophageal pathology. The goals of treatment should include prevention of further contamination, elimination of infection, restoration of gastrointestinal integrity, and establishment of nutritional support. Management of a thoracic perforation requires the following: debridement and drainage of the pleural spaces, control of the esophageal leak, complete re-expansion of the lung, prevention of gastric reflux, nutritional support, and appropriate antibiotic treatment.
Surgical Approach
Surgical intervention includes the following strategies: primary closure with or without buttressing repair, esophagectomy with immediate versus delayed reconstruction, esophageal exclusion and diversion, T-tube placement and drainage, and drainage alone. The selection of the appropriate surgical approach relies heavily on the location and degree of the perforation and the clinical situation. Though not addressed directly below, it should be noted that recently there has been an increase in both the use of esophageal stenting and occasionally nonoperative management of esophageal perforations. The intervention described below will reflect the patient in the clinical scenario who suffered a thoracic esophageal perforation.
Primary Repair
The surgical treatment of choice for an otherwise normal thoracic esophageal perforation is primary repair. The perforation in our case was approached using a left thoracotomy with the patient in the right lateral decubitus position and with single-lung ventilation. Single-lung ventilation is not required but helpful if the patient will tolerate. Once exposed, the esophagus needs to be mobilized and the necrotic esophagus is debrided carefully back to viable tissue. After proper exposure, a vertical esophageal myotomy should be performed, opening the longitudinal and the circular muscle layers to fully expose the mucosal injury. The esophageal defect is the esophageal mucosa and muscle is approximated in a two-layer closure, usually the mucosa is closed with vicryl and the muscle layer with interrupted silks. Reinforcement of the primary repair can be achieved using a variety of tissues (i.e., intercostal muscle, omental onlay graft, lattissimus dorsi, etc.), but it is imperative that the tissue be well vascularized. A nasogastric tube is inserted to just above the repair, while the closure is submerged under saline. The repair is then tested by insufflating air and occluding the distal esophagus. The nasogastric tube is then advanced into the stomach. At this time, the surgeon may consider the need to obtain enteral access (i.e., gastrostomy or jejunostomy feeding tube placement) or perform additional procedures (i.e., fundoplication for significant reflux) (Table 1).
TABLE 1. Key Technical Steps and Potential Pitfalls for Primary Repair of Perforated Esophagus
Presentation Continued
A left thoracotomy in the seventh interspace was used for exposure, during which an intercostal muscle flap was harvested. Care was taken to make sure the pleura and mediastinum were debrided and the distal esophagus was mobilized. The perforation was visualized and the esophagus was debrided. Subsequently, a myotomy was performed to expose the entire mucosal tear and a two-layer repair was performed. The repair was buttressed with an intercostal muscle flap. The thorax was irrigated and a chest tube was placed after which a feeding jejunostomy tube through a separate small upper abdominal incision was placed for enteral access.
Special Considerations
Resection and Diversion
When primary repair is not possible or the pathology makes it less desirable, surgical options include esophageal resection with immediate or delayed reconstruction, or exclusion and diversion. Resection should be considered when confronted with the following circumstances: megaesophagus from achalasia, esophageal carcinoma, caustic ingestion, or severe an esophagectomy is dictated by the surgeon’s experience and underlying pathology.
In patients with a severely devitalized esophagus or when a patient is unable to tolerate definitive repair, the surgeon should consider exclusion and diversion techniques. This includes closure of the perforation, esophageal diversion, and pleural drainage, along with the creation of a cervical esophagostomy for proximal diversion. Drainage can also be achieved via the placement of a T tube distal to the esophageal perforation with the long arm directed toward the stomach and the short arm in the esophagus proximal to the site of injury. The T tube is then brought out through a separate incision. In these circumstances, a jejunostomy should be placed for enteral access.
Postoperative Management
Postoperative management of esophageal perforation is highly dependent upon the intervention taken. If a primary repair was attempted, a follow-up esophagogram (Figure 4) is needed to evaluate the repair. In the event that a leak is present, the chest tube placed intraoperatively should suffice for drainage. However, if not adequately drained, the surgeon should consider placement of a CT-guided pigtail. The thoracostomy tube(s) should be taken off suction, as long as the lung has completely re-expanded and a repeat swallow study should be performed in 5 to 7 days to show that the perforation has healed.
FIGURE 4 • Esophagogram demonstrating absence of leak after primary repair of esophagus.
Case Conclusion
Postoperatively, the patient did well. The chest tube was taken off suction and the lung remained expanded. On postoperative day 5, the patient had an esophagogram that demonstrated no esophageal leak (Figure 4). The patient’s nasogastric tube was removed and the patient was started on a liquid diet.
TAKE HOME POINTS
· Early identification of esophageal perforation is critical.
· Employment of proper imaging and direct visualization modalities are required.
· Surgical approach dictated by location of perforation
· Complete intraoperative exposure of mucosal injury and two-layer closure
· Intraoperative recognition of circumstances that prevent primary repair
· Postoperative imaging to confirm repair of esophageal perforation
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