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There is limited data on long-term outcomes of per-oral endoscopic myotomy (POEM). In this study, we aim to evaluate the efficacy of POEM in patients who completed a minimum follow-up of 5 years.Data of patients who underwent POEM and completed ≥ 5-year follow-up were analyzed, retrospectively. Primary outcome of study was clinical success (Eckardt ≤ 3) at ≥ 5 years after POEM. Secondary outcomes included predictors of dysphagia and symptomatic gastroesophageal reflux disease (GERD) on long-term follow-up.Three hundred nineteen patients (males 182, mean age 40.5 ± 14.2 years) completed a median of 73-(60-89) month follow-up. Esophageal motility disorders included idiopathic achalasia (type I 26.6%, type II 60.8%, type III 5.6%) and Jackhammer esophagus or distal esophageal spasm (2.8%). POEM was technically successful in 307 (96.2%) patients. Long-term success was 92.6% (overall), 92.1% (type I), 94.7% (type II), 87.5% (type III), and 75% (Jackhammer esophagus/distal esophageal spasm). Symptomatic GERD and reflux esophagitis were detected in 28.9% and 35.3% patients, respectively. On multivariate analysis, young age and female gender were independent risk factors for recurrent dysphagia (p = 0.037) and symptomatic GERD after POEM (p = 0.025), respectively. Lower post-POEM lower esophageal sphincter pressure was an independent predictor for reflux esophagitis (p = 0.016).POEM is an effective and durable treatment for achalasia and non-achalasia spastic motility disorders. Young patients and females may be at higher risk for recurrent dysphagia and symptomatic GERD, respectively.

PURPOSE OF REVIEW: Chicago Classification has standardized clinical approach to primary esophageal motility disorders. With new clinical data and advancing treatments, Chicago Classification has undergone multiple revisions to reflect updated findings and enhance diagnostic accuracy. This review will describe the recently published Chicago Classification version 4.0 (CCv4.0), which aimed to enhance diagnostic characterization and limit overdiagnosis of inconclusive esophageal motility diagnoses. RECENT FINDINGS: Key revisions outlined in CCv4.0 include (1) a modified standardized HRM study protocol performed in supine and upright positions, (2) recommended ancillary testing and manometric provocation for inconclusive manometric diagnoses (3) the required presence of obstructive symptoms for conclusive diagnoses of esophagogastric junction outflow obstruction, distal esophageal spasm and hypercontractile esophagus, and (4) requirement of confirmatory testing for esophagogastric junction outflow obstruction. SUMMARY: These key modifications aim to improve diagnostic accuracy and consistency of clinically relevant esophageal motility disorders, and subsequently clinical outcomes.

Investigations conducted using conventional manometry and, recently, using high-resolution manometry (HRM), allowed us to explore the field of esophageal motility and understand the potential link between motor features and gastroesophageal reflux disease (GERD) pathogenesis. The management of patients with nonachalasic esophageal motor disorders is often challenging, due to the clinical heterogeneous presentation and the multifactorial nature of the mechanisms underlying symptoms.Several studies, carried out using HRM, have better interpreted the esophageal motor function in patients with esophagogastric junction outflow obstruction (EGJOO), distal esophageal spasm (DES), hypertensive esophagus, and hypomotility disorders. Moreover, HRM studies have shown a direct correlation between reduced esophageal motility, disruption of the esophagogastric junction, and gastroesophageal reflux burden.Pathogenesis, clinical presentation, diagnosis, and treatment of nonachalasic esophageal motor disorders still represent a challenging area, requiring future evaluation by multicenter outcome studies carried out in a large cohort of patients and asymptomatic subjects. However, we believe that an accurate clinical, endoscopic, and HRM evaluation is, nowadays, helpful in addressing patients with nonachalasic esophageal motor disorders to optimal treatment options.

Distal esophageal spasm (DES), a relatively rare condition, is characterized by simultaneous contractions of the distal esophagus and manifested by dysphagia and chest pain. Several treatment options are recommended, such as pharmacological therapy, endoscopic interventions, and surgical myotomy. Recently, per-oral endoscopic myotomy (POEM) has been adopted as an effective and less-invasive treatment due to its excellent short-term clinical outcomes. Nevertheless, few reports describe its long-term effects. A 65-year-old woman complained of chest pain accompanied with dysphagia and weight loss for 4 months. A series of examinations suggested that she was suffering from DES and then POEM was performed. During the 2.5-year follow-up, we observed an exciting long-term outcome. Interestingly, hematoxylin and eosin staining verified a large number of eosinophils in the muscularis externa, which was absent in the mucosa of the esophagus of the patient. We herein report a case of DES who underwent POEM to eliminate persistent esophageal contractions. Eckardt score, esophageal emptying test, and high-resolution manometry were assumed to monitor the efficacy of POEM. During treatment and 2.5 years after operation, esophageal muscle biopsies and/or mucosal tissues were obtained. This case has been presented to describe that POEM could be a strategy for DES with a long-term curative effect and that eosinophils in the muscle layer of the esophagus might be involved in the pathogenesis of DES. What is more, we reviewed literature to find similar cases reported in the past.EINFüHRUNG: Diffuser Ösophagusspasmus (distal esophageal spasm DES) ist ein eher seltenes Krankheitsbild, gekennzeichnet von simultanen Kontraktionen der Muskulatur der distalen Speiseröhre, das Schluckbeschwerden und retrosternale Schmerzen verursacht. Empfohlen werden unterschiedliche Behandlungsmethoden wie medikamentöse Therapie, endoskopische Verfahren und operative Myotomie. Aufgrund der ausgezeichneten Kurzzeitergebnisse im klinischen Umfeld wird seit kurzem die perorale endoskopische Myotomie (POEM) als effektive und wenig invasive Behandlungsmethode angewandt. Es gibt jedoch nur wenige Berichte über die Langzeitergebnisse. FALLBERICHT: Eine 65-jährige Frau gab an, seit 4 Monaten unter Brustschmerzen mit Schluckbeschwerden und Gewichtsverlust zu leiden. Nach mehreren Untersuchungen wurde die Diagnose DES gestellt, woraufhin eine POEM durchgeführt wurde. Innerhalb des 2,5 Jahre langen Follow-up konnten wir ein ausgezeichnetes Langzeitergebnis feststellen. Interessanterweise ergab die Färbung mit Hämatoxylin und Eosin eine große Anzahl an Eosinophilen in der Muskelschicht, jedoch nicht in der Ösophagusschleimhaut der Patientin. SCHLUSSFOLGERUNGEN: Wir berichten hier über einen Fall von DES bei einer Patientin, bei welcher eine POEM durchgeführt wurde, um persistierende Ösophaguskontraktionen zu eliminieren. Die Wirksamkeit des Eingriffs wurde mittels Eckardt-Score, Ösophagus Entleerungstest und hochauflösender Manometrie überwacht. Während der Behandlung und 2,5 Jahre nach dem Eingriff wurden Gewebeproben aus der Muskelschicht und/oder der Ösophagusschleimhaut gewonnen. Dieser Fall wurde vorgestellt, um zu zeigen, dass POEM eine Behandlungsmethode für DES mit dauerhaft heilender Wirkung sein kann und dass Eosinophile in der Muskelschicht des Ösophagus an der Pathogenese von DES beteiligt sein können. Außerdem haben wir eine Literaturübersicht durchgeführt, um ähnliche Fälle in der Vergangenheit zu finden.

Background Esophageal motility disorders which include achalasia, esophagogastric junction outflow obstruction (EGJ outflow obstruction), jackhammer esophagus (JE), distal esophageal spasm (DES), etc. are rare disease of unknown causes. The diagnosis is based on endoscopy, barium meal, and high-resolution manometry (HRM). With the development of endoscopy, peroral endoscopic myotomy (POEM) has emerged as a standard method for the treatment of achalasia. Purpose The purpose of this article is to enable gastroenterologists to have a more comprehensive understanding of the application status, technical characteristics, clinical efficacy and future prospect of POEM in the treatment of esophageal motility disorders. Methods Through a large number of reading literature, combined with clinical practice, summary and analysis of the indications, procedure, efficacy, complications, and controversies of POEM in the treatment of esophageal motility disorders, as well as the current and future perspectives of POEM were studied. Results POEM is safe and effective in the treatment of esophageal motility disorders, but the GERD reflux rate is higher. Conclusions POEM can be a new option for the treatment of esophageal movement disorders, but large sample, multi-center, long-term study reports are needed, and it promotes the development of NOTES technology.

Background Jackhammer esophagus is a rare esophageal motility disorder that can result in dysphagia, chest pain, and gastro-esophageal reflux symptoms. High-resolution manometry is the gold standard for diagnosis, while corkscrew esophagus on upper gastrointestinal endoscopy is an uncommon manifestation. Case presentation 72-year-old man who presented with progressive dysphagia for three months without symptoms of chest pain or heartburn. Initial workup showed a corkscrew esophagus on upper gastrointestinal endoscopy; subsequently, high-resolution manometry revealed an esophago-gastric junction outflow obstruction with hypercontractile (jackhammer) esophagus. Treatment with calcium channel blockers and proton pump inhibitors was successful and relieved his symptoms near completion. Conclusions Even though the corkscrew esophagus is typically for distal esophageal spasm, the hypercontractile (jackhammer) esophagus can appear. The high-resolution manometry can help to distinguish each specific motility disorder.

Chicago Classification v4.0 (CCv4.0) is the updated classification scheme for esophageal motility disorders using metrics from high-resolution manometry (HRM). Fifty-two diverse international experts separated into seven working subgroups utilized formal validated methodologies over two-years to develop CCv4.0. Key updates in CCv.4.0 consist of a more rigorous and expansive HRM protocol that incorporates supine and upright test positions as well as provocative testing, a refined definition of esophagogastric junction (EGJ) outflow obstruction (EGJOO), more stringent diagnostic criteria for ineffective esophageal motility and description of baseline EGJ metrics. Further, the CCv4.0 sought to define motility disorder diagnoses as conclusive and inconclusive based on associated symptoms, and findings on provocative testing as well as supportive testing with barium esophagram with tablet and/or functional lumen imaging probe. These changes attempt to minimize ambiguity in prior iterations of Chicago Classification and provide more standardized and rigorous criteria for patterns of disorders of peristalsis and obstruction at the EGJ. © 2020 John Wiley & Sons Ltd, National Institute of Diabetes and Digestive and Kidney Diseases, NIDDK: P01 DK092217, National Institute of Diabetes and Digestive and Kidney Diseases, Grant/Award Number: P01 DK092217