Esophagogastric Junction Outflow Obstruction: A Heterogeneous Entity of Unclear Clinical Significance

Abstract

Introduction: Esophagogastric junction outflow obstruction (EGJOO), characterized by preserved peristalsis in conjunction with an elevated integrated relaxation pressure, is a topographic pattern that has recently increased in prevalence.EGJOO can result from specific anatomic variants or it may represent achalasia in evolution, but there is limited information on the clinical significance of this diagnosis. Our aim was to describe the characteristics and outcomes in our cohort of patients with EGJOO. Methods: Consecutive adult patients who had undergone high resolution esophageal manometry (HRM) between February 2013 and November 2015 with a diagnosis of EGJOO were identified. The electronic medical records were reviewed to determine: 1] secondary causes of EGJOO; 2] treatment; and 3] natural history. Improvement in symptoms noted during follow-up evaluation was considered favorable follow-up. Worsening of symptoms or no change in symptoms was considered unfavorable follow-up. Results: Of 874 HRMs performed during this time period, 83 met the criteria for EGJOO. Of these patients, 10 had secondary causes: paraesophageal hernia(4), Nissen fundoplication (2), esophageal strictures(2), prior lap band(1), and diverticulum(1). Two patients developed type II achalasia. The remaining 71 patients were categorized as primary EGJOO; presenting symptoms can be seen in Figure 1. Two of these patients had pre-operative HRMs and were asymptomatic; they were excluded from the follow-up analysis. Of the 69 primary EGJOO patients, 19 had favorable follow-up, 19 had unfavorable follow-up, and 31 were lost to follow-up. Patient characteristics are seen in Table 1. Of those with a favorable response, 6 patients underwent treatment (Figure 2). Of the 19 patients with an unfavorable response, 7 patients underwent treatment (Figure 2). The overall median follow-up time was 5 months.Figure 1Figure 2Figure 3Conclusion: This is the largest ever reported case series of EGJOO patients and their treatment response. Symptoms in patients with EGJOO do not always require treatment and treatment response is variable. The evolution of EGJOO to achalasia is rare. We believe that the challenge in managing these patients lies in distinguishing which patients will need intervention. Further studies are needed for consideration of subgrouping this syndrome or modify the categorization into clinically relevant entities.