Esophageal Perforation: Effective Options Exist for Even the Most Severe Cases

Critically ill patients with uncontained esophageal perforation have several advanced therapy options, from esophageal repair to esophagectomy and complete alimentary tract diversion with the possibility of eventual reconstruction for the most severe cases. Advanced interventions — and characteristics of 166 patients on whom they were performed at Cleveland Clinic — were recently described in the Annals of Surgery (2021;274[5]:e417-e424). The report, which covers cases over a 20-year period, represents the largest single-institution series of advanced intervention for esophageal perforations published to date.

“With more options available, the paradigm for treating esophageal perforation has changed over the years, as have outcomes,” says the paper’s senior author, Sudish Murthy, MD, PhD, Section Head of Thoracic Surgery at Cleveland Clinic. “Despite severe illness, even patients requiring esophageal diversion can now benefit from operative intervention, with excellent long-term survival.”

A critical event with no set solution

Esophageal perforation is often fatal without invasive intervention. Because of its rarity and heterogeneous etiology and presentation, optimal management strategies have not been well defined.

This study was designed to characterize patients who underwent an advanced intervention for esophageal perforation at Cleveland Clinic to identify factors associated with therapy decisions and treatment outcomes.

CT scan showing an esophageal perforation.

Cohort and interventions

Patients who had an esophageal perforation managed by advanced surgical intervention at Cleveland Clinic from 1996 to 2017 were included in the study (N = 166; mean age, 61 ± 16 years; 54% female).

The following interventions were performed:

• Primary repair with tissue flap in 74 patients (of these, three later underwent resection and five underwent resection-diversion). This group had the most iatrogenic perforations, the fewest cancer perforations and the shortest time from perforation to surgery.
• Resection (esophagectomy and gastric pull-up) in 26 patients.
• Resection-diversion (esophagectomy with complete alimentary tract diversion and planned delayed reconstruction) in 66 patients. (Among these patients, plus five of the patients with primary repair leading to resection-diversion, 39 later underwent reconstruction.) This group had more spontaneous perforations involving the lower esophagus compared with the other groups.

Illness severity was retrospectively quantified using the Pittsburgh Severity Score (PSS; range, 0-18), which takes into account risk factors at presentation that are associated with mortality (i.e., age, tachycardia, leukocytosis, pleural effusion, fever, uncontained leak, respiratory compromise, time to diagnosis, cancer, hypotension). A validation study from another institution found that a score over 5 is associated with an in-hospital mortality rate of about 40%.

The mean PSS in the entire cohort was 5.0 ± 3.3; 25% had a PSS > 7. Mean PSS was 3 points for both the repair and resection groups versus 6 points for the resection-diversion group (P = 0.002).

Intervention results

Outcomes following interventions were as follows:

• 90-day mortality was 11% with repair, 7.7% with resection and 23% with resection-diversion (P = 0.13).
• Five-year survival was 71% with repair, 63% with resection and 47% with resection-diversion (P = 0.12).

Risk of death following resection-diversion was highest within the first year. However, 52% of patients with this intervention went on to undergo reconstruction of the upper alimentary tract within two years.

“The risk of death was high for the most severe cases during the first year, but for those who survived this period and underwent reconstruction, survival was excellent,” Dr. Murthy says.

A treatment strategy

Severity of disease at presentation should determine treatment, the study authors emphasize. Dr. Murthy notes that the PSS is a reasonable method for assessing severity and can help guide intervention and management, although other factors also must be considered.

At one end of the spectrum, patients with a small contained perforation often can be managed expectantly, and endoscopic stent placement plus pleural drainage may be sufficient even for full-thickness perforations if soilage is limited.

For more severe presentations and for patients who do not respond to nonoperative management, the authors recommend the following general strategy for advanced intervention:

• For patients at the low end of severity (e.g., limited mediastinal necrosis, a salvageable esophagus): Manage with primary repair and tissue buttress. Patients should still have limited follow-up with the goal of preserving normal deglutition. More than half of patients in the series with a PSS of 2 points or less were managed this way; the rest underwent either resection or resection-diversion.
• For patients with moderately severe disease (e.g., uncontained perforation with lower severity at presentation, and possibly other esophageal pathology): Manage with primary resection and gastric pull-up. Patients should have longer follow-up and will likely need more interventions, such as anastomotic dilatation, in the future.
• For patients with very severe disease: Treat with esophagectomy and complete diversion, then reconstruction after recovery. These patients will need intensive follow-up. More than half of study patients with a PSS of more than 5 points underwent resection-diversion.

Guidance for future cases

“Repair and organ preservation are always preferred, if possible,” observes study co-author Siva Raja, MD, PhD, of the Section of Thoracic Surgery. “However, extensive mediastinal and pleural soilage, in addition to hemodynamic instability, often make this impractical.” In such cases, he says, resection-diversion can be an important life-saving option.

“Despite advances in minimally invasive and endoscopic surgery, life-threatening problems like esophageal perforations sometimes require big operations to start, as we may not have a second chance to salvage critically ill patients,” Dr. Raja concludes. “Studies such as ours can provide guidance to surgeons who are debating between the various options to treat esophageal perforations based on the clinical picture.”