Esophagogastric Junction Outflow Obstruction Is Likely to Be a Local Manifestation of Other Primary Diseases: Analysis of Single-Center 4-Year Follow-Up Data

Abstract

Background: Whether esophagogastric junction outflow obstruction (EGJOO) is a variant of achalasia cardia (AC) or an esophageal motility state of certain organic or systemic diseases remains controversial. We aimed to investigate the differences between EGJOO and AC in clinical characteristics and outcomes through a 4-year follow-up.

Methods: Patients diagnosed with primary EGJOO or AC were included. Based on the presence of concomitant disease, EGJOO patients were divided into a functional and an anatomical EGJOO group; similarly, patients with AC were divided into an AC with organic disease group and a true AC group. Disease characteristics and high-resolution manometry (HRM) parameters were retrospectively compared between the groups, and the development of organic diseases that could affect esophageal motility disorders and responses to treatment were examined during the follow-up. Symptom relief was defined as an Eckardt score of ≤3 after the treatment.

Results: The study included 79 AC patients and 70 EGJOO patients. Compared with patients with AC, EGJOO patients were older, had shorter disease duration, a lower Eckardt score, and were more likely to have concurrent adenocarcinoma of the esophagogastric junction (AEG) and autoimmune disease (p < 0.05 for all). The severity of dysphagia and Eckardt scores were higher in the anatomical EGJOO group than in the functional EGJOO group. Significant differences were seen in HRM parameters (UES residual pressure, LES basal pressure, and LES residual pressure) between AC and EGJOO patients. However, no significant differences in HRM parameters were observed between the functional EGJOO and anatomical EGJOO groups. Sixty-seven (95.71%) patients with EGJOO and sixty-nine (87.34%) patients with AC experienced symptom relief (p = 0.071). Among patients achieving symptom relief, a relatively large proportion of patients with EGJOO had symptom relief after medications (37/67, 55.22%), the resolution of potential reasons (7/67, 10.45%), and spontaneous relief (15/67, 22.39%), while more patients with AC had symptom relief after POEM (66/69, 95.65%). Among EGJOO patients achieving symptom relief, more patients (7/20, 35%) with anatomical EGJOO had symptom relief after the resolution of potential reasons for EGJOO, while more patients (32/47, 68.09%) with functional EGJOO had symptom relief with medications.

Conclusions: Concurrent AEG and autoimmune diseases are more likely in EGJOO than in AC. A considerable part of EGJOO may be the early manifestation of an organic disease. Anatomical EGJOO patients experience symptom improvement with the resolution of primary diseases, while most functional EGJOO patients experience symptom relief with pharmacotherapy alone or even without any treatment.

Keywords: achalasia cardia; esophagogastric junction outflow obstruction; high-resolution manometry; per-oral endoscopic myotomy; pneumatic dilation.

Figure 1

Enrollment of study patients. EGJOO, esophagogastric junction outflow obstruction.

Figure 2

Endoscopic and computed tomography (CT) scan images of two patients with EGJOO and subsequent adenocarcinoma of the esophagogastric junction (AEG). Patient 1 was diagnosed with cardia inflammation using endoscopy (A) and pathology. Thickened cardiac wall and slight stricture of the esophagus are seen on CT (B) performed at the initial visit. Six months later, AEG (T3N0M1) was diagnosed. Endoscopy shows coarse, hyperemic, and easily bleeding cardia mucosa (C). CT shows an obviously thickened cardiac wall (D). In Patient 2, endoscopy performed at the initial visit (E) shows cardia mucosa hyperemia; CT (F) shows a slightly thickened cardiac wall. Endoscopy (G) performed one-and-a-half years later shows high-grade localized intraepithelial neoplasia in the gastric mucosa; this was confirmed on pathology. CT shows thickened and enhanced cardiac wall (H).

Figure 3

Proportion of patients achieving symptom relief with different therapies in the EGJOO and AC groups. EGJOO, esophagogastric junction outflow obstruction; AC, achalasia cardia; POEM, per-oral endoscopic myotomy.

Figure 4

Diagnosis and treatment algorithm for patients with EGJOO and AC. EGJOO, esophagogastric junction outflow obstruction; AC, achalasia cardia; HRM, high-resolution manometry; EUS, endoscopy ultrasound; CT, computed tomography; MRI, magnetic resonance imaging; PPIs, proton pump inhibitors; POEM, per-oral endoscopic myotomy.