Clinical presentation and disease course of patients with esophagogastric junction outflow obstruction

Abstract

Esophagogastric junction outflow obstruction, characterized by preserved peristalsis in conjunction with an elevated integrated relaxation pressure, can result from specific anatomic variants or may represent achalasia in evolution. There is limited information on the clinical significance of this diagnosis. The aim of this study is to describe the clinical characteristics and outcomes in our cohort of patients with esophagogastric junction outflow obstruction.Consecutive adult patients who had undergone high-resolution esophageal manometry between February 2013 and November 2015 with a diagnosis of esophagogastric junction outflow obstruction were identified. Electronic medical records were reviewed to determine: (1) secondary causes of esophagogastric junction outflow obstruction; (2) treatment; and (3) natural history. Improvement in symptoms noted during follow-up evaluation was considered to be a favorable outcome. Worsening of symptoms or no change in symptoms was considered to be an unfavorable outcome.Of 874 manometries performed during this time period, 83 met the criteria for esophagogastric junction outflow obstruction. Of these patients, 11 had secondary causes: paraesophageal hernia (4), Nissen fundoplication (2), esophageal stricture (3), prior laparoscopic band placement (1), and diverticulum (1). All of these secondary causes were identified by barium esophagram. The remaining 72 patients were categorized as idiopathic esophagogastric junction outflow obstruction. Two patients developed type II achalasia on follow-up. An additional two patients had no symptoms as testing was performed for preoperative evaluation prior to bariatric surgery, leaving 68 patients for symptom follow-up analysis. Of these, 19 had a favorable outcome, 18 had an unfavorable outcome, and 31 were lost to follow-up. Of those with a favorable outcome, 6 patients underwent treatment: medication (3), botulinum toxin injection followed by laparoscopic Heller myotomy (1), botulinum toxin injection and medication (1), and bougie dilation (1). Of the 18 patients with an unfavorable outcome, 6 patients underwent treatment: botulinum toxin injection (5) and medication (1). Computed tomography scan or endoscopic ultrasound was performed in 40% of patients with available follow-up and none of these studies revealed secondary causes. The overall median follow-up time was 5 months.Esophagogastric outflow obstruction is a manometric finding of unclear significance. Secondary causes should first be excluded with structural studies. The evolution of esophagogastric junction outflow obstruction to achalasia is rare. Symptoms in patients with esophagogastric junction outflow obstruction do not always require treatment and treatment response is variable. The challenge in managing these patients lies in distinguishing which patients will need intervention. Further studies are needed for consideration of subgrouping this disease or modifying the categorization into clinically relevant entities.

Pneumatic dilation improves esophageal emptying and symptoms in patients with idiopathic esophago‐gastric junction outflow obstruction

“…In our experience, we observed 2/14 patients with idiopathic EGJOO who were treated with Botox (14%) progress to type 3 achalasia within 20 months of treatment . There have been four patients with EGJOO treated with surgical myotomy who had positive outcomes …”

“…Table summarizes all currently published data in regards to EGJOO treatment outcomes . Somewhat surprising are the three reports (Van Hoeij, Perez‐Fernandez and Lynch) suggesting that 44%–52% improve with no therapy …”

Esophagogastric junction outflow obstruction on manometry: Outcomes and lack of benefit from CT and EUS

“…Their conclusion is that performing an EUS in all patients with EGJOO may not be warranted given its low yield at detecting pathology not seen with other less aggressive modalities. Further, Lynch et al reported that in their series of 83 EGJOO cases, 15 patients had either a CT or EUS, none of which revealed any abnormalities; all secondary causes were revealed on BE (paraesophageal hernia, Nissen fundoplication, esophageal stricture, prior laparoscopic band placement, and diverticulum). Finally, DeLay et al reported that in their series of 32 patients with EGJOO, 21 had anatomic abnormalities as potential causes.…”

“…One of the most significant advantages that HRM provides compared to conventional manometry is its ability to accurately identify and diagnose the different contractile patterns of aperistalsis seen in achalasia . However, this increased sensitivity has also led to the observation of other manometric abnormalities with unknown clinical significance; one such example is an entity known as esophagogastric junction outflow obstruction (EGJOO) …”

Pharmacologic interrogation of patients with esophagogastric junction outflow obstruction using amyl nitrite

“…Proposed etiologies of EGJOO in CCv3 include mechanical obstruction, cardiovascular compression, early achalasia, and potentially hiatal hernia . In addition, outflow obstruction with normal lower esophageal sphincter (LES) function can result from a tight fundoplication wrap or extrinsic compression of the EGJ, such as from a paraesophageal hernia or gastric laparoscopic band placement …”