Characterization of idiopathic esophagogastric junction outflow obstruction

Esophagogastric junction (EGJ) outflow obstruction is a manometric diagnosis, characterized by an elevated relaxation pressure (IRP4) of the lower esophageal sphincter (LES) and intact or weak peristalsis. The etiology and preferred treatment remain unknown. We describe a large patient cohort in detail, for a better understanding of this rare disorder. We included 47 patients, diagnosed with EGJ outflow obstruction on high-resolution manometry (HRM) between 2012 and December 2014. Idiopathic EGJ outflow obstruction was diagnosed in 34 patients. The majority (91%) of patients presented with retrosternal pain or dysphagia. The median (IQR) for various HRM parameters was IRP4, 18.9 mmHg (18-23); intrabolus pressure (IBP), 8.3 mmHg (5-12) and basal LES pressure, 27.5 mmHg (22-33). Peristaltic breaks were seen in 88% and elevated IBPmax in 74% of patients. No patients had stasis, difficult LES passage or esophageal dilation on endoscopy. Only 7/25 patients (28%) had stasis on barium esophagography. In 26 patients (82%), no treatment was required: 18 had symptoms judged unrelated to outflow obstruction, 5 had spontaneous symptom relief, and 3 declined therapy. Eight patients were treated: five received botox injections with a good but short-lived effect, three received pneumatic dilatation, of which one was successful. Three patients were diagnosed with achalasia on a subsequent manometry. Primary EGJ outflow obstruction has an unclear clinical significance. A substantial part of patients has unrelated symptoms, spontaneous symptom relief, or no stasis. Treated patients showed a beneficial response to botox injections. A small proportion develops achalasia at follow-up