Dysphagia associated with esophageal wall thickening in patients with nonspecific high-resolution manometry findings: Understanding motility beyond the Chicago classification version 4.0.

BACKGROUND: Previous studies have demonstrated that 50% of patients with normal high-resolution manometry (HRM) findings or ineffective esophageal motility (IEM) may have abnormal functional luminal imaging probe (FLIP) results. However, the specific HRM findings associated with abnormal FLIP results are unknown. Herein, we investigated the relationship between nonspecific manometry findings and abnormal FLIP results. METHODS: We retrospectively analyzed 684 patients who underwent HRM at a tertiary care center in Seoul, Korea, based on the Chicago Classification version 4.0 protocol. KEY RESULTS: Among the 684 patients, 398 had normal HRM findings or IEM. Of these 398 patients, eight showed esophageal wall thickening on endoscopic ultrasonography or computed tomography; however, no abnormalities were seen during esophagogastroduodenoscopy. Among these eight patients, seven showed repetitive simultaneous contractions (RSCs) in at least one of the two positions: 61% (±29%) in 10 swallows in the supine position and 51% (±30%) in five swallows in the upright position. Four patients who underwent FLIP had a significantly decreased esophagogastric junction distensibility index (1.0 ± 0.5 m m 2 mmHg - 1 at 60 mL). Two of these patients underwent per-oral endoscopic myotomy (POEM) due to a lack of response to medication. Esophageal muscle biopsy revealed hypertrophic muscle with marginal eosinophil infiltration. CONCLUSIONS & INFERENCES: A subset of patients (2%) with normal HRM findings or IEM and RSCs experienced dysphagia associated with poor distensibility of the thickened esophageal wall. FLIP assessment or combined HRM and impedance protocols may help better define these patients who may respond well to POEM.

Characteristics of Esophageal Motility and Associated Symptom Profiles in Patients with Esophageal Diverticulum: A Study Based on High-Resolution Impedance Manometry.

BACKGROUND: Esophageal diverticulum (ED) is an uncommon structural disorder with heterogenous manifestations and elusive pathophysiology. Our aim was to investigate esophageal motility and associated symptom profiles in patients with ED based on high-resolution impedance manometry (HRIM). METHODS: Consecutive patients with ED referred to our motility laboratory between 2015 to 2022 were identified in our electronic database. All patients were evaluated based on an upper endoscopy, HRIM, and standardized symptom questionnaires. Patients with ED were further stratified into upper, middle, and lower (epiphrenic) cases. Esophageal motility was evaluated with HRIM and the updated Chicago Classification v4.0. RESULTS: Twenty-four patients with ED (9 upper, 4 middle, and 11 epiphrenic) were analyzed. Patients with ED were generally older (mean: 65 ± 13.3 years) and predominantly women (58.3%). Most ED cases were unilaterally located (95.8%) and left-side predominant (62.5%). Mean symptom duration was 20 months (range: 1-120) and the most common symptoms were dysphagia (70.8%) and regurgitation (37.5%). Erosive esophagitis was noted in 16 patients (69.6%), while barium stasis was noted in 5 patients (20.8%). Fourteen patients (58.3%) were diagnosed with esophageal motility disorders using HRIM, with achalasia being the most common diagnosis (n = 5, 20.8%). Patients with epiphrenic diverticulum had significantly higher symptom scores and achalasia prevalence. CONCLUSION: Patients with ED tended to be older and was associated with a high prevalence of EMD. A multi-disciplinary evaluation, including complete anatomical and motility surveys, may help clarify the underlying pathophysiology and tailor further treatment strategies.

Compartmentalized pressurization is a novel prognostic factor for hypercontractile esophagus.

BACKGROUND: Hypercontractile esophagus (HE) is a disorder of increased esophageal body contractile strength on high-resolution esophageal manometry (HREM). Compartmentalized pressurization (CP) is a pattern with an isobaric contour of >30 mmHg extending from the contractile front to the lower esophageal sphincter on HREM. The relevance of CP to HE has yet to be explored. METHODS: A retrospective review was performed on 830 HREM studies of patients to identify HE. HE patients' CP status and symptoms by Eckardt score (ES) were reviewed. Diagnoses were made using Chicago Classification (CC) v4.0. KEY RESULTS: Forty-seven patients (5.6%) were identified as having HE by CCv3, 30 (3.6%) of which had HE by CCv4. 11/30 HE patients had CP, and 19/30 did not. CP was associated with chronic opioid use (36.4% vs. 5.3% p = 0.047). Presenting ES was greater for HE patients with CP (7 vs. 4). Seven HE patients with CP and 11 without CP were managed medically. ES after medical therapy was higher in HE patients with CP compared to those without CP (9 vs. 0). No HE patients with CP responded to medical therapy. Kaplan-Meier analysis demonstrated significance of this association over time. 83% of all HE patients had all-cause symptom remission. CONCLUSIONS & INFERENCES: HE patients with CP are associated with a higher presenting ES. HE patients with CP do not respond to medical therapy, while HE patients without CP frequently do respond. CP in HE may have prognostic value in determination of treatment strategy for patients with HE.

[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.

Predictors of Underlying Esophageal Motility Disorders in Patients Presenting with Esophageal Candidiasis.

OBJECTIVES: Esophageal motility disorders (EMDs) are a known risk factor for esophageal candidiasis (EC), but this relation has not been described particularly well. We sought to evaluate the predictors of underlying EMDs in patients presenting with EC. METHODS: Cases of EC at a single medical center between 2010 and 2021 were identified retrospectively based on the International Classification of Diseases, Ninth Revision code. Demographic, clinical, endoscopic, and manometric data were reviewed. The diagnosis of EC was based on typical endoscopic appearance. RESULTS: In total, 130 EC patients were identified (mean age 69.5 ± 14.6; 66.2% male). Of these, 12 (9.2%) had an underlying EMD (11 cases of achalasia; 1 case of esophagogastric junction outflow obstruction). Five (41.7%) of these patients had previously been diagnosed as having an EMD, whereas 7 were newly diagnosed only after their presentation with EC. No significant differences were noted between those with or without EMDs in terms of demographics, medical comorbidities, or medication use. Patients with an EMD, however, were more likely to complain of dysphagia (91.7% vs 30.5%, P < 0.001), and on endoscopy, they were more likely to have residual food in the esophagus, residual fluid in the esophagus, a dilated esophagus, and resistance to traversing the esophagogastric junction (all P < 0.001). Sixty-one (46.9%) patients with EC died during follow-up (mean 58 months). CONCLUSIONS: EMDs are present in approximately 10% of patients presenting with EC, with half being diagnosed only after presenting with EC. Similar to non-EC patients, patients with EC with dysphagia and the typical endoscopic findings of achalasia are more likely to have an EMD and warrant prompt manometric evaluation.

Evaluating the efficacy and safety of acotiamide in patients with esophagogastric junction outflow obstruction: study protocol for an investigator-initiated, multi-center, randomized, double-blind, placebo-controlled phase II trial.

BACKGROUND: We have determined that the impaired accommodation of the lower esophageal sphincter (LES) underlies the pathogenesis of esophagogastric junction outflow obstruction (EGJOO). We have also found that acotiamide may treat EGJOO by improving impaired LES accommodation. The effects of acotiamide in patients with EGJOO need to be further confirmed in a prospective study. METHODS: This trial is a multicenter, randomized, double-blind, placebo-controlled study to compare the efficacy and safety of acotiamide (300 mg/day or 600 mg/day) with those of a placebo in the treatment of patients with EGJOO. The primary endpoint will be the proportion of patients who report an improvement in symptom of food sticking in the chest after 4 weeks of treatment period 1. The secondary endpoints will be the proportion of patients with normalized integrated relaxation pressure (IRP), the value of change from baseline in the distal contractile integral, basal LES pressure, EGJOO-quality of life score, Gastrointestinal Symptom Rating Scale, and the correlation between IRP and each symptom score. During the 2-year trial period, 42 patients from five institutions will be enrolled. DISCUSSION: This trial will provide evidence to clarify the efficacy and safety of acotiamide as a treatment for patients with EGJOO. Acotiamide might help improve the quality of life of patients with EGJOO and is expected to prevent the progression of EGJOO to achalasia. TRIAL REGISTRATION: This study was approved by the Institutional Review Board (IRB) of Kyushu University Hospital as well as the local IRBs of the participating sites for clinical trials and registered in the Japan Registry of Clinical Trials (jRCT: 2071210072). The registration date is on October 11, 2021.

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.

Evaluation of Esophageal Dysphagia in Elderly Patients.

PURPOSE OF REVIEW: While guidelines exist for the evaluation and management of esophageal dysphagia in the general population, dysphagia disproportionately affects the elderly. In this article, we reviewed the literature on evaluating esophageal dysphagia in elderly patients and proposed a diagnostic algorithm based on this evidence. RECENT FINDINGS: In older patients, dysphagia is often well compensated for by altered eating habits and physiologic changes, underreported by patients, and missed by healthcare providers. Once identified, dysphagia should be differentiated into oropharyngeal and esophageal dysphagia to guide diagnostic workup. For esophageal dysphagia, this review proposes starting with endoscopy with biopsies, given its relative safety even in older patients and potential for interventional therapy. If endoscopy shows a structural or mechanical cause, then further cross-sectional imaging should be considered to assess for extrinsic compression, and same session endoscopic dilation should be considered for strictures. If biopsies and endoscopy are normal, then esophageal dysmotility is more likely, and high-resolution manometry and additional workup should be performed following the updated Chicago Classification. Even after diagnosis of the root cause, complications including malnutrition and aspiration pneumonia should also be assessed and monitored, as they both result from and can further contribute to dysphagia. The successful evaluation of esophageal dysphagia in elderly patients requires a thorough, standardized approach to collecting a history, selection of appropriate diagnostic workup, and assessment of risk of potential complications, including malnutrition and aspiration.

[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.

Comprehensive Manometric Evaluation of Dysphagia in Patients with Down Syndrome.

Dysphagia is a common symptom in children with Down syndrome and is conventionally evaluated with imaging and endoscopy; high-resolution manometry is not routinely utilized. The aim of this study was to describe and correlate pharyngeal and esophageal manometry findings with contrast studies and endoscopy in patients with Down syndrome and dysphagia. Electronic medical records of patients with Down syndrome with dysphagia seen at our center between January 2008 and January 2022 were reviewed. Data collected included demographics, co-morbidities, symptoms, imaging, endoscopy, and manometry. Twenty-four patients with Down syndrome [median age of 14.9 years (IQR 7.6, 20.5), 20.8% female] met inclusion criteria. Common presenting symptoms of dysphagia included vomiting or regurgitation in 15 (62.5%) patients, and choking, gagging, or retching in 10 (41.7%) patients. Esophageal manometry was abnormal in 18/22 (81.2%) patients. The most common findings were ineffective esophageal motility in 9 (40.9%) followed by esophageal aperistalsis in 8 (36.4%) patients. Rumination pattern was noted in 5 (22.8%) patients. All 6 (25%) patients who previously had fundoplication had esophageal dysmotility. Strong agreement was noted between upper gastrointestinal studies and high-resolution esophageal manometry (p = 1.0) but no agreement was found between pharyngeal manometry and video fluoroscopic swallow studies (p = 0.041). High-resolution pharyngeal and esophageal manometry provide complementary objective data that may be critical in tailoring therapeutic strategies for managing patients with Down syndrome with dysphagia.

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.

Non-acid reflux and esophageal dysmotility is associated with early esophageal squamous cell carcinoma.

BACKGROUND: Studies have demonstrated that non-acid reflux (NAR) is associated with esophageal squamous cell carcinoma (ESCC). Esophageal dysmotility is associated with NAR but few studies have focused on the esophageal motility of ESCC patients. We explored the relationship between ESCC, NAR and esophageal dysmotility with the aid of multichannel intraluminal impedance and pH (MII-pH) and high-resolution manometry (HRM). METHODS: From Jan 2021 to Oct 2022, 20 patients with superficial ESCC were enrolled as the ESCC group, while 20 age and gender matched individuals without gastroesophageal reflux disease (GERD) symptoms and 20 age and gender matched patients with GERD symptoms were recruited as the control groups. Patients received 24 h MII-pH and HRM procedure before endoscopic submucosal dissection (ESD), and the data were then collected to identify the type of reflux and esophageal dysmotility. RESULTS: Prevalence of esophageal dysmotility was significantly different among the three groups, 75.0% in the ESCC group, 35.0% in the non-GERD group and 70.0% in the GERD group (P = 0.029). NAR episodes at 15 cm above the lower esophageal sphincter (LES) in the ESCC group were significantly higher than that in the non-GERD group (6.5 (3.5-9.3) vs 1.0 (0.8-4.0), P = 0.001) and were similar with that in the GERD group (6.5 (3.5-9.3) vs 5.5 (3.0-10.5), P > 0.05). NAR episodes at 5 cm above LES was significantly higher in the ESCC group than that in the non-GERD group (38.0 (27.0-60.0) vs 18.0 (11.8-25.8), P = 0.001) and was significantly higher than that in the GERD group (38.0 (27.0-60.0) vs 20.0 (9.8-30.5)), P = 0.010). Prevalence of pathologic non-acid reflux was significantly different among the three groups, 30.0% in the ESCC group, 0.0% in the non-GERD group and 10.0% in the GERD group (P < 0.001). CONCLUSION: Our study found NAR and esophageal dysfunction frequently occur in ESCC patients. NAR and esophageal dysmotility may be associated with ESCC. CLINICAL TRIAL REGISTRATION NUMBER: ChiCTR2200061456.

Infection with SARS-CoV-2 as a potential achalasia trigger.

Idiopathic achalasia is a chronic oesophageal motility disorder caused by loss of inhibitory neurons at the esophageal myenteric plexus resulting in incomplete relaxation of the lower oesophageal sphincter and abnormal peristaltism.  Among the possible causes of this, an immune response secondary to infection by some viruses has been implicated. SARS-CoV-2 could be considered among them. The therapy option should be aimed at achieving the greatest clinical effectiveness according to each patient's health status.

The emerging association between bronchiectasis and systemic sclerosis: assessing prevalence and potential causality.

BACKGROUND: Bronchiectasis has been observed in association with systemic sclerosis (SSc). Theorised aetiology includes aspiration related to oesophageal dysmotility, immunosuppressant medication use and the direct effect of collagen deposition on airway calibre. AIMS: To detail bronchiectasis prevalence in an SSc population who have had a high-resolution computed tomography (HRCT) of the thorax. We assessed whether oesophageal dysmotility, demographic variables, SSc duration or subclass were associated with bronchiectasis. METHODS: Participants in the Australian Scleroderma Cohort Study (ASCS) with a HRCT were included. The ASCS provided demographic and clinical data. HRCT studies were reviewed for bronchiectasis, oesophageal dilatation and interstitial lung disease (ILD). Traction bronchiectasis associated with ILD was recorded as a separate entity to bronchiectasis. Oesophageal dysmotility was defined by symptoms and/or oesophageal dilatation. RESULTS: Of the 256 participants, 16.4% (n = 42) had bronchiectasis. Logistic regression analysis revealed no significant association between bronchiectasis and oesophageal dysmotility (observed in 95.7%), any demographic variable, SSc duration or subclass. A negative association between bronchiectasis and ILD was observed (P = 0.009; odds ratio 0.322; 95% confidence intervals 0.137-0.756). CONCLUSION: Those with SSc appear to have an increased risk for bronchiectasis. Since bronchiectasis was not more frequent in participants with a longer duration of SSc, we hypothesise that its development is not related to immunosuppression alone. Oesophageal dysmotility was almost universal in our population such that its effect on bronchiectasis development could not be concluded. A negative association between bronchiectasis and ILD reflects that bronchiectasis occurring alongside ILD was recorded as a separate entity.

[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.

From Chicago classification v3.0 to v4.0: Diagnostic changes and clinical implications.

BACKGROUND: The Chicago Classification (CC) used to define esophageal motility disorders in high-resolution manometry (HRM) has evolved over time. Our aim was to compare the frequency of motility disorders diagnosed with the last two versions (CCv3.0 and CCv4.0) and to evaluate symptoms severity according to the diagnoses. METHODS: From June to December 2020, patients who underwent esophageal HRM with swallows in supine and sitting positions were included. HRM studies were retrospectively analyzed using CCv3.0 and CCv4.0. Symptoms severity and quality of life were assessed with validated standardized questionnaires. KEY RESULTS: Among the 130 patients included (73 women, mean age 52 years), motility disorder diagnoses remained unchanged in 102 patients (78%) with both CC. The 3 patients with esophago-gastric junction outflow obstruction (EGJOO) with CCv3.0 were EGJOO, ineffective esophageal motility (IEM) and normal with CCv4.0. Twenty-four out of 63 IEM diagnosed with the CCv3.0 (38%) turned into normal motility with the CCv4.0. Whatever the CC used, brief esophageal dysphagia questionnaire score was significantly higher in patients with EGJ relaxation disorders compared to those with IEM (25 (0-34) vs 0 (0-19), p = 0.01). Gastro-Esophageal Reflux disease questionnaire (GERD-Q) score was higher in patients with IEM with both CC compared to those who turned to normal with CCv4.0. CONCLUSIONS AND INFERENCES: While motility disorders diagnoses remained mainly unchanged with both CC, IEM was less frequent with CCv4.0 compared to CCv3.0. The higher GERD-Q score in IEM patients with CCv4.0 suggests that CCv4.0 might identify IEM more likely associated with GERD.

Dysphagia Worsens With Increasing Ineffective Swallows Among Patients With Ineffective Esophageal Motility.

INTRODUCTION: Ineffective esophageal motility (IEM) is the most common motility disorder identified on esophageal high-resolution manometry (HRM), but patients with this finding may be asymptomatic. Therefore, we aimed to identify specific HRM findings predictive of symptoms in IEM. METHODS: Adult patients (≥18 y) who underwent HRM between March 2016 and July 2019 were retrospectively evaluated and reclassified according to Chicago Classification 4.0 (CC4.0). Demographic information, HRM parameters, and gastroesophageal reflux disease Health-Related Quality of Life Questionnaire responses were captured among those with normal manometry or IEM. We evaluated the association between heartburn, regurgitation, and dysphagia, with respect to HRM findings including ineffective swallows. RESULTS: Of 379 patients, 243 (64.1%) had a normal manometry, 136 (35.9%) were on IEM spectrum, and 73 (19.3%) had conclusive IEM by CC4.0. Mean dysphagia scores were significantly higher in those with conclusive IEM compared with those with normal HRM (2.00 vs. 1.36, P =0.002), as was the percentage of individuals reporting dysphagia affecting daily activities (21.9% vs. 11.4%, P =0.02). Heartburn and regurgitation symptoms did not differ between groups. In a multivariable model of IEM patients, the percentage of ineffective swallows independently predicted a higher dysphagia burden (ß regression coefficient: 0.032, P =0.04). CONCLUSIONS: Applying the CC4.0 definition for conclusive IEM identifies a group of patients with worse dysphagia symptoms. Within this group, the percentage of ineffective swallows was an independent predictor of dysphagia severity. These findings may help practitioners and patients contextualize the heterogeneous diagnosis of IEM.

Esophageal dysmotility in patients with eosinophilic esophagitis: pathogenesis, assessment tools, manometric characteristics, and clinical implications.

Eosinophilic esophagitis (EoE) represents a growing cause of chronic esophageal morbidity whose incidence and prevalence are increasing rapidly. The disease is characterized by eosinophilic infiltrates of the esophagus and organ dysfunction. Typical symptoms include dysphagia, chest pain, and bolus impaction, which are associated to mechanical obstructions in most patients. However, up to one in three EoE patients has no visible obstruction, suggesting that a motor disorder of the esophagus may underlie symptoms. Although potentially relevant for treatment refractoriness and symptomatic burden, esophageal dysmotility is often neglected when assessing EoE patients. The first systematic review investigating esophageal motility patterns in patients with EoE was published only recently. Accordingly, we reviewed the pathogenesis, assessment tools, manometric characteristics, and clinical implications of dysmotility in patients with EoE to highlight its clinical relevance. In summary, eosinophils can influence the amplitude of esophageal contractions via different mechanisms. The prevalence of dysmotility may increase with disease duration, possibly representing a late feature of EoE. Patients with EoE may display a wide range of motility disorders and possible disease-specific manometric pressurization patterns may be useful for raising a clinical suspicion. Intermittent dysmotility events have been found to correlate with symptoms on prolonged esophageal manometry, although high-resolution manometry studies have reported inconsistent results, possibly due to the suboptimal sensitivity of current manometry protocols. Motor abnormalities may recover following EoE treatment in a subset of patients, but invasive management of the motor disorder is required in some instances. In conclusion, esophageal motor abnormalities may have a role in eliciting symptoms, raising clinical suspicion, and influencing treatment outcome in EoE. The assessment of esophageal motility appears valuable in the EoE setting.

Long-term Outcome of Peroral Endoscopic Myotomy in Esophageal Motility Disorders: A Systematic Review and Meta-analysis.

BACKGROUND AND AIM: Peroral endoscopic myotomy (POEM) is an established treatment for achalasia. In this systematic review and meta-analysis, we aimed to analyze the mid and long-term outcomes of POEM in esophageal motility disorders. METHODS: Literature search was performed in databases including PubMed, Embase, Cochrane databases, and Google scholar from January 2010 to May 2021. The primary objective of the study was the clinical success (Eckardt score ≤3 or <4) at mid-term (30 to 60 mo) and long-term (>60 mo) follow-up after POEM. Secondary objectives included post-POEM gastroesophageal reflux (GER) as evaluated by symptoms, increased esophageal acid exposure, and reflux esophagitis. RESULTS: Seventeen studies with 3591 patients were included in the review. Subtypes of motility disorders were type I (27%), type II (54.5%), type III (10.7%), distal esophageal spasm/Jackhammer esophagus (2%), and esophagogastric junction outflow obstruction (17.5%). Pooled mean follow-up duration was 48.9 months (95% CI, 40.02-57.75). Pooled rate of clinical success at mid-term follow-up was 87% (95% CI, 81-91; I2 , 86%) and long-term was 84% (95% CI, 76-89; I2 , 47%). In nonachalasia motility disorders (esophagogastric junction outflow obstruction, distal esophageal spasm, and Jackhammer esophagus), pooled rate of clinical success was 77% (95% CI, 65-85; I2 , 0%). GER as estimated by symptoms was 23% (95% CI, 19-27; I2 , 74%), erosive esophagitis was 27% (95% CI, 18-38%; I2 , 91%), and increased esophageal acid exposure was 41% (95% CI, 30-52; I2 , 88%). CONCLUSION: POEM is a durable treatment option in cases with achalasia. One-fourth of patients suffer from erosive GER in the long-term and success rates are lower in nonachalasia esophageal motility disorders.

Associations Between Patterns of Esophageal Dysmotility and Extra-Intestinal Features in Patients With Systemic Sclerosis.

OBJECTIVE: The gastrointestinal tract is commonly involved in patients with systemic sclerosis (SSc) with varied manifestations. As our understanding of SSc gastrointestinal disease pathogenesis and risk stratification is limited, we sought to investigate whether patterns of esophageal dysfunction associate with specific clinical phenotypes in SSc. METHODS: Patients enrolled in the Johns Hopkins Scleroderma Center Research Registry who completed high-resolution esophageal manometry (HREM) studies as part of their clinical care between 2011 and 2020 were identified. Associations between esophageal abnormalities on HREM (absent contractility [AC], ineffective esophageal motility [IEM], hypotensive lower esophageal sphincter [hypoLES]) and patient demographic information, clinical characteristics, and autoantibody profiles were examined. RESULTS: Ninety-five patients with SSc had HREM data. Sixty-five patients (68.4%) had AC (37 patients with only AC, 28 patients with AC and a hypoLES), 9 patients (9.5%) had IEM, and 11 patients (11.6%) had normal studies. AC was significantly associated with diffuse cutaneous disease (38.5% versus 10.0%; P < 0.01), more severe Raynaud's phenomenon, including digital pits, ulcers, or gangrene (56.9% versus 30.0%; P = 0.02), and reduced median diffusing capacity of lung for carbon monoxide (50.6% versus 72.2%; P = 0.03). AC was observed in most of the patients who died (13 of 14; P = 0.06). These findings were not seen in patients with IEM. CONCLUSION: Among patients with SSc, AC is associated with a significantly more severe clinical phenotype. IEM may associate with a milder phenotype. Further studies are needed to evaluate AC, IEM, and their clinical impact relative to the timing of other end-organ complications in SSc.