Esophageal perforation and leakage is a serious and potentially life-threatening medical emergency with varying etiologies (Figure 1) and clinical presentations.

Thoracic esophageal perforations are the most common (72.6%), followed by cervical (15.2%), and abdominal (12.5%).

PRESENTING CLINICAL SIGNS AND SYMPTOMS

• Pain is the most common presenting feature, present in about 80% of cases, usually referring directly to the site of perforation. In cervical perforation, there may be pain in the neck with neck stiffness, whereas thoracic perforation may present with severe chest or epigastric pain and dyspnea. In lower esophageal perforations, abdominal pain could radiate to the back. Dysphagia may be present in patients with midesophageal and distal perforations.

• Other symptoms include vomiting, hematemesis, dysphagia, tachypnea, cough and fever.

• Thoracic perforation of esophagus may present with features of mediastinitis and pneumothorax or hemothorax. In lower esophageal perforations, there may be signs of peritonitis.

• The signs for esophageal perforations are mostly nonspecific, but most of the time, there may be tachycardia, hypotension, shock and fever. Subcutaneous emphysema is present in up to 60% of perforations but requires at least an hour to develop after the initial injury.

• In cases of delayed presentation or extensive contamination, patients may be critically ill and may present with gross sepsis, respiratory failure, altered mental status and multiple organ dysfunction syndromes.

DIAGNOSTIC IMAGING OF ESOPHAGEAL PERFORATION AND LEAKAGE

Radiological studies are valuable tools in the diagnosis of esophageal perforation. (Figure 2)

X-rays : Plain chest x-rays are often suggestive of perforation, with subcutaneous emphysema, pneumothorax, pleural effusions, pneumoperitoneum and retropharyngeal swelling. (Figure 3 and Figure 4)

• In cervical esophageal perforation a lateral neck X-ray may demonstrate air in the prevertebral facial planes.

• In thoracic esophageal perforation pneumomediastinum, subcutaneous emphysema, mediastinal widening, or a mediastinal air-fluid level may be present in the chest x-ray. Pneumothorax may be present in up to 77% of the cases.

• In intra-abdominal esophageal perforation pneumoperitoneum may be apparent in abdominal xrays and hydropneumothorax on the left may be seen in patients with distal third esophageal perforations.

Fluoroscopic study of esophagus: Method of choice for confirmation and pointing the location of esophageal perforation or leak even though a negative scan always does not exclude perforation, especially in the cervical esophagus because of the rapid transit of the contrast.

Fluoroscopy:

• Reveals the primary area of leakage. (Figure 5)

• Determines whether the perforation is confined to the mediastinum or communicates freely with the pleural or peritoneal cavities.(Figure 6)

Contrast-enhanced CT : CT scan of the chest should be performed if fluoroscopy is not available in the emergency set-up or in case of a negative study despite high clinical suspicion. Perforation may be suggested by mediastinal air, extravasated luminal contrast, periesophageal fluid collections, pleural effusions, or actual communication of an air-filled esophagus with an adjacent mediastinal air-fluid collection (Figure 7). Intravenous contrast is ideally administered to delineate the esophageal wall.

GENERAL MANAGMENT

The management of esophageal perforation and leakage can be either non-operative or operative with many factors contributing in the decision made each time by the surgeon including clinical status and stability of the patient, presence of a contained perforation or a free perforation with mediastinal contamination and sepsis, any clinical deterioration, location of the perforation and presence of underlying pathology.

Conservative treatment consists of empiric antibiotics, enteral nutrition, drainage of fluid collections, and management of the perforation/leak whereas surgical revision is usually challenging and carries a high risk of secondary complications. For this reason, several minimally invasive treatments which include the application of metal clips, fibrin glue, and placement of self-expanding metal or plastic stents are coming to the fore. Endoscopic Vacuum therapy (E-Vac) is the latest addition to the management algorithm and can be applied on both adults and the pediatric population.

E-VAC TREATMENT AND IMAGING FINDINGS

Pre-procedure evaluation with esophagogram, CT scan (with oral contrast) and upper gastrointestinal endoscopy is mandatory to correctly evaluate the indication for E-Vac and to facilitate a patient-tailored treatment plan. It is important to evaluate:

• the depth, extent and composition of the extramural collection

• presence and size of fistulas or the anastomotic defects (Figure 8)

Indications and contraindications for E-Vac treatment :

Since it is still a relatively new technique, currently no standardized indications for use have been well established, although most patients with acute or chronic GI defects are candidates for E-Vac.

Possible indications include :

• Transmural Gastointestinal Defect (TGID) with associated contained undrained collection

• TGID with associated contained drainage collection in need to remove the external drain

• Acute iatrogenic perforations

• Gastrointestinal (GI) – cutaneous fistulas

• TGID with associated uncontained collection aiming to form a contained collection

• As additional therapy

Contraindications or lack of indications for E-Vac therapy remain not so clear, to date. However, it is recommended that E-Vac should be avoided in :

• patients with defects that are close to major vessels,

• patients in high risk of major bleeding

• case of inability to achieve negative pressure (GI - vaginal, vesical or trachea/bronchial fistulas and gastro-colonic fistulas

• case of inability to access TGID

• GI-cutaneous fistulas with a thin (<5 mm) and a long-epithelialized tract (>2 cm)

• patient refusal for treatment

E-Vac procedure (Figure 9, 10) includes transoral (endoluminal or intracavitary) endoscopic placement of a polyurethane sponge connected to an externalized nasogastric tube, which provides continuous negative pressure (~125mmHg). This continuous pressure provokes mechanical deformations that cause macroscopic and microscopic beneficial changes that promote healing that include:

• mechanical discloser of wound edges

• granulation of healthy tissue

• neovascularization

• control of the septic focus through active drainage of extramural collections, necrotic debris and purulent material

The drainage tube with the attached sponge needs to be replaced every 3 to 7 days. Since negative-pressure therapy results in a slow healing process, the total treatment duration usually takes several weeks (Figure 11,12, 13, 14, 15, 16).

Follow-up imaging is important to evaluate the response to the treatment and to promptly depict any potential complications.

In an uncomplicated/therapy-responsive outcome, we expect:

• reduction of the size to even complete closure of the anastomotic defect or fistula (Figure 17,18)

• reduction of the extramural collections (Figure 19)

• appearance of the wound bed and margins (endoscopic evaluation) for improvement signs of the inflammatory tissue environment (Figure 20)

E-Vac is considered in general as a safe therapeutic procedure with a small amount of reported complication such us anastomotic stricture, local bleeding, sponge dislocation and aortic fistulas.