Esophageal Motility Disorders: Diagnosis and Treatment Strategies.

BACKGROUND: Esophageal motility disorders (EMDs) are caused by the impaired relaxation of the upper/lower esophageal sphincter and/or defective esophageal peristaltic contractions, resulting in dysphagia and noncardiac chest pain. High-resolution manometry (HRM) is essential for the diagnosis of primary EMD; however, the recognition of EMD and HRM by general practitioners in Japan is limited. This review summarizes the diagnosis of and treatment strategies for EMD. SUMMARY: HRM is a specific test for the diagnosis of EMD, whereas endoscopy and barium swallow as screening tests provide characteristic findings (i.e., esophageal rosette and bird's beak sign) in some cases. It is important to note that manometric diagnoses apart from achalasia are often clinically irrelevant; therefore, the recently updated guidelines suggest additional manometric maneuvers, such as the rapid drink challenge, and further testing, including functional lumen imaging, for a more accurate diagnosis before invasive treatment. Endoscopic/surgical myotomy, pneumatic dilation, and botulinum toxin injections need to be considered for patients with achalasia and clinically relevant esophagogastric junction outflow obstruction. KEY MESSAGE: Since the detailed pathophysiology of EMD remains unclear, their diagnosis needs to be cautiously established prior to the initiation of invasive treatment.

Malaysian joint societies' consensus recommendations on diagnosis and management of disorders of esophagogastric junction outflow.

Disorders of esophagogastric junction (EGJ) outflow, including achalasia and EGJ outflow obstruction, are motility disorders characterized by inadequate relaxation of lower esophageal sphincter with or without impaired esophageal peristalsis. Current guidelines are technical and less practical in the Asia-Pacific region, and there are still massive challenges in timely diagnosis and managing these disorders effectively. Therefore, a Malaysian joint societies' task force has developed a consensus on disorders of EGJ outflow based on the latest evidence, while taking into consideration the practical relevance of local and regional context and resources. Twenty-one statements were established after a series of meetings and extensive review of literatures. The Delphi method was used in the consensus voting process. This consensus focuses on the definition, diagnostic investigations, the aims of treatment outcome, non-surgical or surgical treatment options, management of treatment failure or relapse, and the management of complications. This consensus advocates the use of high-resolution esophageal manometry for diagnosis of disorders of EGJ outflow. Myotomy, via either endoscopy or laparoscopy, is the preferred treatment option, while pneumatic dilatation can serve as a secondary option. Evaluation and management of complications including post-procedural reflux and cancer surveillance are recommended.

Esophageal motility disorder - has Chicago classification v4.0 simplified our management?

REVIEW PURPOSE: Addressing dysphagia is vital due to its prevalence and impact on healthcare expenditure. While high resolution manometry (HRM) effectively evaluates esophageal dysphagia, its role in oropharyngeal dysphagia and upper esophageal sphincter (UES) dysfunction remains debated. The fourth iteration of the Chicago classification (CC) offers an algorithmic approach for diagnosing abnormal motor patterns via HRM. This review assesses the CC's impact on dysphagia management. RECENT INSIGHTS: The Chicago classification version 4.0 emphasizes auxiliary and provocative techniques when the algorithm falls short of a conclusive diagnosis. It introduces stricter criteria for previously ambiguous conditions like ineffective motility and esophagogastric junction outflow obstruction. This version also introduces the concept of conclusive and inconclusive classifications based on symptoms, provocation maneuvers, and supportive testing minimizing ambiguity. SUMMARY: The Chicago classification v4.0 remains a useful tool for the diagnosis of well characterized esophageal motility disorders. However, major limitations include reliance on HRM and a focus on distal esophagus contractile characteristics without considering proximal esophagus or upper esophageal sphincter, both of which can sometimes be the only evident abnormality in patients with dysphagia. Despite efforts to reduce ambiguity, diagnostic challenges persist. These limitations can be addressed in future updates.

[Esophageal Motility Disorders - What's New?] / Motilitätsstörungen des Ösophagus ­ Was ist neu?

Esophageal motor disorders are an important cause of dysphagia but can also be associated with retrosternal pain and heartburn as well as regurgitation. In extreme cases, patients are not able to eat appropriately and lose weight. Repetitive aspiration can occur and may cause pulmonological complications. Achalasia represents the most important and best-defined esophageal motor disorder and is characterized by insufficient relaxation of the lower esophageal sphincter in combination with typical disturbances of esophageal peristalsis. Additional defined motor disorders are distal esophageal spasm, hypercontractile esophagus, absent contractility and ineffective peristalsis. Patients with appropriate symptoms should primarily undergo esophagogastroduodenoscopy for exclusion of e.g., tumors and esophagitis. Esophageal high-resolution manometry is the reference method for diagnosis and characterization of motor disorders in non-obstructive dysphagia. An esophagogram with barium swallow may deliver complementary information or may be used if manometry is not available. Balloon dilatation and Heller myotomy are long established and more or less equally effective therapeutic options for patients with achalasia. Peroral endoscopic myotomy (POEM) enhances the therapeutic armamentarium for achalasia and hypertensive/spastic motor disorders since 2010. For hypotensive motor disorders, which may occur as a complication of e.g., rheumatological diseases or idiopathically, therapeutic options are still limited.

Mixed Esophageal Disease (MED): A New Concept.

We define mixed esophageal disease (MED) as a disorder of esophageal structure and/or function that produces variable signs or symptoms, simulating-fully or in part other well-defined esophageal conditions, such as gastroesophageal reflux disease, esophageal motility disorders, or even neoplasia. The central premise of the MED concept is that of an overlap syndrome that incorporates selected clinical, endoscopic, imaging, and functional features that alter the patient's quality of life and affect natural history, prognosis, and management. In this article, we highlight MED scenarios frequently encountered in medico-surgical practices worldwide, posing new diagnostic and therapeutic challenges. These, in turn, emphasize the need for better understanding and management, aiming towards improved outcomes and prognosis. Since MED has variable and sometimes time-evolving clinical phenotypes, it deserves proper recognition, definition, and collaborative, multidisciplinary approach, be it pharmacologic, endoscopic, or surgical, to optimize therapeutic outcomes, while minimizing iatrogenic complications. In this regard, it is best to define MED early in the process, preferably by teams of clinicians with expertise in managing esophageal diseases. MED is complex enough that is increasingly becoming the subject of virtual, multi-disciplinary, multi-institutional meetings.

[Therapy of esophageal motility disorders]. / Therapieoptionen von primären Motilitätsstörungen des Ösophagus.

Esophageal motility disorders are diseases in which there are malfunctions of the act of swallowing due to a change in neuromuscular structures. The main symptom is therefore dysphagia for solid and/or liquid foods, often accompanied by symptoms such as chest pain, regurgitation, heartburn, and weight loss. Esophageal manometry is the gold standard in diagnostics. Endoscopy and radiology serve to exclude inflammatory or malignant changes. With the introduction of high-resolution esophageal manometry (HRM), the diagnosis of esophageal motility disorders has improved and led to a new classification with the Chicago Classification, which has been modified several times in the last decade, most recently in 2020 with the Chicago Classification v4.0. Compared to the previous version 3.0, there are some important changes that are presented based on the most important esophageal motility disorders in everyday clinical practice.

Treatment of esophagogastric junction outflow obstruction with concomitant hypercontractile esophagus: A case series.

Hypercontractile esophagus with concomitant esophagogastric junction outflow obstruction (EGJOO) is a rare entity that is characterized by both esophageal hypercontractility and lack of relaxation of the EGJ. The clinical characteristics of these patients are not well-described and there is no strict recommendation regarding the treatment of this condition. We report four cases of patients with hypercontractile esophagus and concomitant to EGJOO. All patients underwent upper gastrointestinal (GI) endoscopy, high-resolution esophageal manometry (HRM) and barium swallow and met the criteria of Chicago Classification for both EGJOO and hypercontractile esophagus. Patients were followed up to four years from diagnosis and clinical symptoms were recorded. Four patients, who underwent evaluation for dysphagia, were found to have both EGJOO and hypercontractile esophagus on HRM. Two of them had mild symptoms and did not undergo treatment with no progression of symptoms on follow-up. Of the two patients who underwent treatment, one had botulinum toxin injection to the EGJ via upper GI endoscopy and one underwent per-oral endoscopic myotomy. Symptoms in both patients improved. Patients with concomitant hypercontractile esophagus and EGJOO present with varying degrees of symptoms and the treatment approach should be personalized according to the degree of symptoms and general clinical condition.

[Therapy of esophageal motility disorders]. / Therapieoptionen von primären Motilitätsstörungen des Ösophagus.

Esophageal motility disorders are diseases in which there are malfunctions of the act of swallowing due to a change in neuromuscular structures. The main symptom is therefore dysphagia for solid and/or liquid foods, often accompanied by symptoms such as chest pain, regurgitation, heartburn, and weight loss. Esophageal manometry is the gold standard in diagnostics. Endoscopy and radiology serve to exclude inflammatory or malignant changes. With the introduction of high-resolution esophageal manometry (HRM), the diagnosis of esophageal motility disorders has improved and led to a new classification with the Chicago Classification, which has been modified several times in the last decade, most recently in 2020 with the Chicago Classification v4.0. Compared to the previous version 3.0, there are some important changes that are presented based on the most important esophageal motility disorders in everyday clinical practice.

Functional Lumen Imaging Probe Panometry Helps Identify Clinically Relevant Esophagogastric Junction Outflow Obstruction per Chicago Classification v4.0.

INTRODUCTION: Esophagogastric junction (EGJ) outflow obstruction (EGJOO) per Chicago Classification v4.0 (CCv4.0) represents a high-resolution manometry (HRM) diagnosis with uncertain clinical significance. This study aimed to evaluate functional lumen imaging probe (FLIP) panometry among patients with EGJOO on HRM/CCv4.0 to assess clinical/manometric associations and treatment outcomes. METHODS: An observational cohort study was performed on patients who completed FLIP during endoscopy and had an HRM/CCv4.0 diagnosis of EGJOO, i.e., HRM-EGJOO (inconclusive). Abnormal FLIP panometry motility classifications were applied to identify FLIP-confirmed conclusive EGJOO. Rapid drink challenge on HRM and timed barium esophagram were also assessed. Clinical management plan was determined by treating physicians and assessed through chart review. Clinical outcome was defined using the Eckardt score (ES) during follow-up evaluation: ES < 3 was considered a good outcome. RESULTS: Of 139 adult patients with manometric EGJOO (inconclusive per CCv4.0), a treatment outcome ES was obtained in 55 after achalasia-type treatment (i.e., pneumatic dilation, peroral endoscopic myotomy, laparoscopic Heller myotomy, or botulinum toxin injection) and 36 patients after other nonachalasia-type treatment. Among patients with conclusive EGJOO by HRM-FLIP complementary impression, 77% (33/43) had a good outcome after achalasia-type treatment, whereas 0% (0/12) of patients had a good outcome after nonachalasia-type treatment. Of patients with normal EGJ opening on FLIP, one-third of patients treated with achalasia-type treatment had a good outcome, while 9 of the 10 treated conservatively had a good outcome. DISCUSSION: FLIP panometry provides a useful complement to clarify the clinical significance of an HRM/CCv4.0 EGJOO diagnosis and help direct management decisions.

How provocative tests in addition to wet swallows during high-resolution manometry can direct clinical management.

PURPOSE OF REVIEW: Provocative tests have been advocated to address limitations of high-resolution manometry (HRM) with wet swallows. We describe the commonly used provocative manometric manoeuvres [rapid drink challenge (RDC), multiple rapid swallows (MRS), solid swallows and the solid test meal (STM)], compare their diagnostic yield and accuracy to wet swallows, and outline their role in directing clinical management. RECENT FINDINGS: Provocative testing with RDC and STM identifies a proportion of achalasia cases missed by standard testing, and RDC can play a similar role to radiology in the evaluation of treatment response. In EGJOO, provocative testing with RDC and STM not only increase the diagnostic yield, but can also help differentiate between spurious cases and those representing true outflow obstruction likely to respond to achalasia-type therapies. MRS and STM can help determine the clinical relevance (or otherwise) of ineffective oesophageal motility, and in this setting, MRS may predict the likelihood of postfundoplication dysphagia. RDC and STM can help identify cases of postfundoplication dysphagia more likely to respond to dilatation. SUMMARY: Provocative testing has been shown to increase diagnostic yield of HRM, clarify inconclusive diagnoses, allows corroboration of dysmotility with patient symptoms and helps direct clinical management.

Esophageal Motility Disorders: Current Approach to Diagnostics and Therapeutics.

Dysphagia is a common symptom with significant impact on quality of life. Our diagnostic armamentarium was primarily limited to endoscopy and barium esophagram until the advent of manometric techniques in the 1970s, which provided the first reliable tool for assessment of esophageal motor function. Since that time, significant advances have been made over the last 3 decades in our understanding of various esophageal motility disorders due to improvement in diagnostics with high-resolution esophageal manometry. High-resolution esophageal manometry has improved the sensitivity for detecting achalasia and has also enhanced our understanding of spastic and hypomotility disorders of the esophageal body. In this review, we discuss the current approach to diagnosis and therapeutics of various esophageal motility disorders.

Clinical presentation and therapeutic outcome of patients with jackhammer esophagus-a multicenter cohort study in Japan.

BACKGROUND: Jackhammer esophagus (JE) is a hypercontractile esophageal motility disorder diagnosed using high-resolution manometry (HRM). We sought to determine the clinical presentation and therapeutic data of patients with JE in Japan. METHODS: The study included patients with JE, diagnosed through HRM performed for suspicious esophageal motility disorders. Demographics, esophagogastroduodenoscopy, radiology, and therapy data were collected from patient charts. RESULTS: Among the 4,412 HRM tests performed, 89 patients (61.6 ± 13.4 years; 64 males, 25 females) were diagnosed with JE (2.0%). Dysphagia was the most frequent symptom (80%), followed by chest pain (40%) and heartburn (25%). Esophagogastroduodenoscopy showed abnormal findings in 32% of patients: corkscrew/rosary beads appearance in 26%, narrowing in 11%. Eosinophilic infiltration (> 15 eosinophils/high power field) was diagnosed in 21%. Esophagography showed abnormal findings in 9% of the patients. For the initial therapy, 47 patients received medical treatment followed by peroral endoscopic myotomy (21 patients) and laparoscopic myotomy (two patients). Thirteen patients did not receive any treatment and 10 of those (77%) reported spontaneous resolution of symptoms. Patients who required invasive treatment experienced severe disability in their quality of life and greater maximal distal contractile integral than those who did not. CONCLUSIONS: HRM showed that the prevalence of JE was very low (2%). Esophagogastroduodenoscopy revealed some characteristic features of JE in patients. Some patients showed improvement of symptoms without invasive treatments. Follow-up with/without medical treatment should be considered before performing invasive treatment in patients whose distal contractile integral is relatively low and the quality of life is not impaired.

Nonachalasic esophageal motor disorders, from diagnosis to therapy.

INTRODUCTION: 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. AREAS COVERED: 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. EXPERT OPINION: 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.

Upper Gastrointestinal Functional and Motility Disorders in Children.

Children with disorders affecting the sensory and motor functions of the esophagus will present primarily with swallowing dysfunction, dysphagia, and chest pain, and those with disorders affecting the normal function of the stomach will present with symptoms like abdominal pain, nausea, and vomiting. Recent advances in the mechanisms of disease and technology have increased our understanding of gastrointestinal physiology and that knowledge has been applied to develop new diagnostic studies and therapeutic interventions. We present an overview of the clinical presentation, diagnosis, and treatment of common primary and secondary functional and motility disorders affecting the upper gastrointestinal tract in children.

Integrative Effects and Vagal Mechanisms of Transcutaneous Electrical Acustimulation on Gastroesophageal Motility in Patients With Gastroesophageal Reflux Disease.

INTRODUCTION: Impaired esophageal and gastric motilities are known to contribute to symptoms of gastroesophageal reflux disease (GERD). However, there is a lack of GERD therapy, targeting both gastric and esophageal functions. This study was designed to investigate the effects of transcutaneous electrical acustimulation (TEA) on symptoms of GERD and gastroesophageal functions and possible mechanisms in patients with GERD. METHODS: Thirty patients with GERD with ineffective esophageal motility were equally divided and randomized into a 4-week sham-TEA or 4-week TEA treatment. The GERD questionnaire (GerdQ), GERD health-related quality-of-life questionnaire, high-resolution esophageal manometry, a nutrient drink test, the electrogastrogram, and ECG were performed to assess the severity of reflux symptoms, low esophageal sphincter (LES) pressure, distal contractile integral (DCI), gastric accommodation, gastric slow waves (GSW), and autonomic functions, respectively. RESULTS: Compared with sham-TEA, the 4-week TEA treatment significantly decreased the GerdQ score (P = 0.011) and GERD health-related quality of life (P = 0.028) and improved nutrient drink-induced fullness (P < 0.001) and belching (P < 0.001) in patients with GERD. Although only acute TEA significantly enhanced LES pressure (P < 0.05), both acute and chronic TEA remarkedly increased DCI (P < 0.05) and reduced the incidence of ineffective esophageal contractions during wet swallows (P = 0.02). In addition, chronic TEA significantly increased gastric accommodation and the percentage of postprandial normal GSW compared with sham-TEA and baseline. Concurrently, TEA-enhanced vagal activity (P = 0.02) and the vagal activity positively correlated with LES pressure (r = 0.528; P = 0.003) and DCI (r = 0.522; P = 0.003). DISCUSSION: The TEA treatment performed in this study improves reflux-related symptoms, increases DCI, reduces the incidence of ineffective esophageal contractions during wet swallows, and improves gastric accommodation and slow waves. The improvement in GERD symptoms might be attributed to the integrative effects of TEA on these gastroesophageal functions mediated via the vagal mechanism.