Symptom or sign:
The esophagus spans three distinct anatomic regions: neck, thorax, and abdomen. As such, symptoms of esophageal perforation vary, based on the location and severity of the perforation. Clinical symptoms and signs are not always specific and can be commonly mistaken for other much common conditions, such as myocardial infarction or peptic ulcer disease.
In a 50-year review of esophageal perforation, Nesbit et al. found four most commonly occurring symptoms to be pain (71%), fever (50%), dyspnea (24%), crepitus (22%).
Cervical esophagus. Esophageal perforation within the neck, commonly presents as neck pain, dysphagia, or odynophagia and worsening with neck flexion and swallowing. Palpable crepitus, if present, is usually easily palpable on exam.
Thoracic esophagus. Perforation of the upper portion of the intra-thoracic esophagus results in a right-sided pleural effusion associated with fever. Mid-thoracic esophageal perforation presents with subternal or epigastric pain; mediastinal crunch is rarely auscultated.
Mackler’s triad. Thoracic pain, vomiting, subcutaneous emphysema, and contamination of the left pleural space characterize perforation of the distal thoracic esophagus.
Abdominal esophagus. Severe epigastric pain radiating to the back and/or left shoulder with associated peritonitis suggests perforation of the abdominal esophageal segment.
Depending on the time elapsed from the injury and degree of cavitary contamination, signs of systemic inflammatory response, overt sepsis, or shock (tachycardia, tachypnea, hypotension, fever etc.) may appear.
Perforation of the esophagus is a relatively uncommon condition. Its occurrence alarms clinicians due to historically quoted high patient mortality (>50%) associated with this condition. While still a dangerous and potentially life-threatening situation, perforation of the esophagus has become increasingly more manageable, thanks to the advancements in imaging technology, image guided interventions, endoscopic and stent therapy, and improved surgical and intensive perioperative care.
The most common etiology of esophageal perforation has shifted from spontaneous esophageal perforation – known as Boerrhave’s syndrome after Hermann Boerrhave for his infamous clinical and pathological description of the demise of the Grand Admiral of Dutch fleet – to iatrogenic perforation incurred during diagnostic or therapeutic endoscopic procedures.
Regardless of the etiology, achievement of optimal outcomes in the management of esophageal perforation is facilitated by early diagnosis and the impeccable judgment of clinicians experienced in the medical, surgical, and endoscopic management alternatives. Care of each patient with esophageal perforation is always individualized, taking into account (1) the patient’s condition and comorbidities, (2) time interval to diagnosis, (3) cause and location of perforation, and (4) underlying esophageal pathology.
Also known as:
Perforation of the esophagus, Boerrhave’s syndrome, spontaneous esophageal perforation, iatrogenic esophageal perforation
What disease states can produce this sign or symptom?
Many acute diseases of the chest and upper abdominal organs may produce symptoms similar to esophageal perforation. Acute coronary syndrome, aortic dissection, pulmonary embolus but also esophagitis, peptic ulcer disease, gastritis, acute cholecystitis, or pancreatitis can all present with symptomatology similar to esophageal perforation.
The frequency of these diagnoses in emergency departments is much greater than the uncommon patient with esophageal perforation. The increasing use of imaging technology in the evaluation of patients with acute chest or abdominal pain in most emergency rooms, in particular CT scans, often leads to previously unexpected findings suggestive of esophageal perforation.
What urgent or emergent measures should be initiated even before the diagnosis is established?
As soon as the diagnosis of esophageal perforation is entertained, the patient should be made nothing per o.s., and intravenous fluids and antibiotics covering both aerobic and anaerobic microbes started ASAP. Assessment of the mental status, respiratory status, hemodynamic parameters, and laboratory values (renal function, acid-base disturbances) then serves as a guide in the decision for further evaluation and treatment.
The overall care of a patient with esophageal perforation is guided by following principles:
Timely and accurate diagnosis
Resuscitation and optimization of cardiopulmonary function
Institution of broad spectrum antimicrobial coverage
Control of extraluminal contamination
Debridement of nonviable tissue
Restoration of gastro-intestinal continuity
What is the appropriate initial diagnostic approach to identify the specific underlying disease?
In a patient with clinically suspected esophageal perforation, the first order of importance is to assess the clinical state of the patient.
A hemodynamically unstable patient manifesting signs of sepsis is best first admitted to the intensive care unit for volume resuscitation, respiratory support, and optimization of the hemodynamic status. If a plain anterior-posterior chest X-ray reveals large pleural effusion, tube thoracostomy should be placed and fluid sent for gram stain, cultures, lactate dehydrogenase (LDH), and amylase. The presence of food particles in the chest tube confirms the diagnosis of esophageal perforation.
Consideration should be given in the critically ill patient regarding whether further diagnostic imaging (contrast CT) should be performed or simply to proceed to the operating room for a judicious diagnostic endoscopy, followed by a therapeutic stent placement, primary surgical repair, with drainage and debridement of contaminated spaces. This decision is best made by a thoracic or esophageal surgeon experienced in the management of esophageal perforation.
Diagnostic evaluation of a stable patient with suspected esophageal perforation should proceed in a systematic manner, beginning with chest X-rays, followed by a contrast esophagogram with Gastrografin and subsequent thin barium swallow. Positioning a patient in a prone or right lateral decubitus position decreases the transit time of a contrast bolus traversing the esophagus, compared to the upright swallow; this increased transit time for the contrast agent in the esophagus facilitates identification of more subtle perforations.
The goal of the contrast study is to diagnose, localize, and establish whether the perforation is contained or freely communicating with the mediastinum or pleural cavity. Information learned from the contrast study, coupled with the overall patient status, serves as a guide in selecting the appropriate therapeutic strategy.
If a patient is unable to swallow, a neck, chest, abdomen CT scan may suffice in therapeutic planning. In addition, a carefully performed flexible esophagoscopy provides valuable information about the extent and location of the perforation, and of any associated esophageal pathology. Endoscopy is best performed in the operating room, with available set up for immediate tube thoracostomy should a tension pneumothorax develop from overt insufflation; alternatively, chest tube can be placed prior to endoscopy on the side of pleural effusion.
The clinical presentation of patients with esophageal perforation varies, depending on the mechanism and location of injury, time elapsed from the injury, and underlying comorbid conditions.
Cervical perforation is associated with dysphagia and odynophagia; the latter worsening with neck flexion. Palpable neck crepitus is often palpable.
Upper thoracic perforation commonly presents with right-sided pleural effusion, but may also be associated with substernal and epigastric pain; audible mediastinal crunch is a rather rare finding.
Perforation of the lower esophagus, common in spontaneous perforation, is classically characterized by the presence of left-sided pleural effusion and Mackler’s triad: thoracic pain, vomiting, and subcutaneous emphysema.
Severe epigastric pain, abdominal rigidity with peritonitis, and, occasionally, pain radiating to the back or left shoulder are hallmarks of the perforation of the abdominal portion of the esophagus.
Other signs of systemic inflammatory response or sepsis such as fever, tachypnea, tachycardia, leukocytosis, and oliguria may be present, depending on the degree of cavitary contamination. In extreme circumstances, a patient’s clinical status may evolve into circulatory instability and multiorgan dysfunction.
The nature and unpredictability of esophageal perforation precludes the study of this condition in a prospective randomized manner. Virtually all reports on treatment and outcome of esophageal perforation are retrospective series, making direct comparison between treatment strategies difficult.
Traditionally, primary surgical repair, resection, or diversion was the mainstay of treatment. While surgical therapy continues to play an important role in the treatment of esophageal perforation, excellent outcomes including mortality, morbidity, and esophageal healing have been achieved with nonoperative treatment with or without placement of intraluminal stent. Treatment of esophageal perforation must therefore be individualized in each patient, taking into account all available modern treatment modalities. Often a hybrid approach of medical, endoscopic, and surgical options offers the best change for a successful result.
Strict medical treatment with careful patient observation, antibiotics, intravenous antibiotics, and nothing per o.s. may be utilized in patients with a documented, contained perforation and minimal clinical symptoms. This is particularly true in those with perforation detected immediately following an intervention, where the time of perforation is known and the patient has been NPO.
The original characteristics favoring nonoperative management were defined by Cameron and later expanded by Altorjay. They include early diagnosis, perforation within the neck or mediastinum with drainage back into the esophagus, absence of esophageal disease (benign or malignant), and minimal symptoms. The outcome in a patient population with these favorable clinical and radiographic characteristics is superior with nonoperative management compared to the outcome of surgical repair.
Endoscopic deployment of a removable, covered esophageal stent has revolutionized treatment of esophageal perforation. Multiple reports have documented the feasibility of stent use in esophageal perforation. The greatest utility of stents is in the perforation of mid to distal esophagus. Optimally, the proximal and distal landing zones for the stent should be within normal esophageal tissue. Ultimately, a covered stent intra-luminally bypasses the perforation, prevents further contamination of the mediastinum and pleura, and allows healing of the esophageal wall. However, stent alone is not always sufficient as a sole therapy for esophageal perforation. Adequate debridement and drainage of contaminated spaces and nonviable tissue is also of paramount importance.
Regardless of the technical approach chosen for the management of esophageal perforation, the guiding principles of therapy remain the same. Depending on institutional resources and experience of the clinician, drainage of infested pleural space or mediastinum may be achieved via image-guided techniques or thoracoscopically; in severe instances, formal thoracotomy and decortication of the lung may be required to achieve adequate lung expansion.
The main advantage of surgical exploration of esophageal perforation is the opportunity to address all aspects associated with perforation: visualization of perforation, repair, resection, or diversion; drainage and debridement of surrounding devitalized tissues, underlying esophageal pathology; and access for enteral nutrition. The choice of surgical therapy, however, requires experience and judgment in patient selection and surgical approach. Accurate preoperative localization of the injury and optimization of cardiopulmonary function are essential in planning the appropriate operative strategy and exposure. Importantly, the time from perforation and the presence of any esophageal disorder (achalasia, stricture, or tumor) further challenge the surgeon’s operative planning.
While general principles of primary repair of esophageal perforation remain the same for all segments of the esophagus, the extent of intervention differs in the cervical, thoracic, and abdominal esophageal perforation. Following the exposure of esophageal injury, myotomy is extended proximally and distally to ensure full inspection of the mucosal perforation. The mucosa is then approximated with interrupted fine absorbable sutures. The muscular layer is then closed over the mucosal repair as a second layer.
Drainage alone may suffice for cervical perforation, particularly those that are difficult to expose or repair. Primary repair of the thoracic or abdominal segment of the esophagus is more challenging, depending on the time from perforation, adequacy of the patient’s tissues, and any esophageal pathology.
A two-layer closure of the thoracic esophagus is often buttressed with an intercostal muscle flap, pericardium, or omentum, and abdominal esophagus can be reinforced with gastric fundus by constructing a Dor, Toupet, or Nissen fundoplication. The complexity of surgical decision making is augmented in the presence of esophageal disease such as achalasia, esophageal motility disorders, strictures, or malignancy. In the presence of severe esophageal dysfunction or malignancy prior to perforation, it may be best to proceed with esophageal resection and subsequent reconstruction with a suitable conduit. Involvement of an experienced esophageal surgeon in this setting is invaluable.
What is the diagnostic approach if this initial evaluation fails to identify the cause?
Cross-sectional visualization of the neck, mediastinum, and upper abdomen during a contrast esophagogram is highly sensitive (>90%) in identifying extra-luminal air and fluid collections, and therefore should be the initially study of choice in a stable and cooperative patient. Computed tomography is very useful in patients unable to undergo formal contrast swallow.
If the diagnosis of esophageal perforation is still in question, carefully performed flexible endoscopy has been associated with 100% sensitivity and 83% specificity in diagnosing esophageal perforation. Moreover, endoscopy has the advantage of direct visualization of mucosal tears, perforations, pathologic lesions, and strictures, which further aids in the management of patients with esophageal perforation.
What's the evidence?
Nesbit, JC, Sawyers, JL. “Surgical Management of Esophageal Perforation”. Am Surg. vol. 53. 1987. pp. 183-91.
Brinster, CJ, Singhal, S, Lee, L. “Evolving options in the management of esophageal perforation”. Ann Thorac Surg. vol. 77. 2004. pp. 1475-83. (The most comprehensive review of esophageal perforation within the past decade. Reviews literature of esophageal perforation from diagnosis to treatment and provides outcome data of various treatments.)
Vogel, SB, Rout, WR, Martin, TD, Abbitt, PL. “Esophageal perforation in adults: aggressive, conservative treatment lowers morbidity and mortality”. Ann Surg. vol. 241. 2005. pp. 1016-21. (One of the first reports documenting the feasibility of nonoperative management of esophageal perforation. Notably, an aggressive interventional approach was used with multiple imagine studies and image-guided procedures.)
Sutcliffe, RP, Forshaw, MJ, Datta, G. “Surgical management of Boerhaave's syndrome in a tertiary oesophagogastric centre”. Ann R Coll Surg Engl. vol. 91. 2009. pp. 374-80. (Article outlines modern surgical therapy of spontaneous esophageal perforation.)
Shaker, H, Elsayed, H, Whittle, I, Hussein, S, Shackcloth, M. “The influence of the "golden 24-h rule'' on the prognosis of oesophageal perforation in the modern era”. Eur J Cardiothorac Surg. vol. 38. 2010. pp. 216-22. (The importance of early diagnosis and intervention is discussed in this article. Diagnosing esophageal perforation and instituting treatment within first 24 hours of the event has been the most consistent predictor of favorable outcome in the literature.)
Kavic, SM, Basson, MD. “Complications of endoscopy”. Am J Surg. vol. 181. 2001. pp. 319-32. (Iatrogenic perforation has become the most common cause of esophageal perforation. This article discusses complications of endoscopy.)
Abbas, G, Schuchert, MJ, Pettiford, BL. “Contemporaneous management of esophageal perforation”. Surgery. vol. 146. 2009. pp. 749,55; discussion 755-6. (More recent large-series retrospective review of outcomes of esophageal perforation in a single institution. Authors attempt to construct a clinical score predicting outcome and serving as a guide to therapy.)
Freeman, RK, Van Woerkom, JM, Ascioti, AJ. “Esophageal stent placement for the treatment of iatrogenic intrathoracic esophageal perforation”. Ann Thorac Surg. vol. 83. 2007. pp. 2003-7.
Kim, AW, Liptay, MJ, Snow, N. “Utility of silicone esophageal bypass stents in the management of delayed complex esophageal disruptions”. Ann Thorac Surg. vol. 85. 2008. pp. 1962-7.
Leers, JM, Vivaldi, C, Schafer, H, Bludau, M. “Endoscopic therapy for esophageal perforation or anastomotic leak with a self-expandable metallic stent”. Surg Endosc. vol. 23. 2009. pp. 2258-62. (Freeman, van Woerkom, Ascioti (2007); Kim, et al. (2008); and Leers, Vivaldi, and Schafer, et al. (2009) refer to the latest approach to esophageal perforation and outline the success of endoscopic and stent management of patients with esophageal perforation.)
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- Esophageal perforation
- What disease states can produce this sign or symptom?
- What urgent or emergent measures should be initiated even before the diagnosis is established?
- What is the appropriate initial diagnostic approach to identify the specific underlying disease?
- What is the diagnostic approach if this initial evaluation fails to identify the cause?