Esophageal Motility Disorders.

Esophageal motility disorders can cause chest pain, heartburn, or dysphagia. They are diagnosed based on specific patterns seen on esophageal manometry, ranging from the complete absence of contractility in patients with achalasia to unusually forceful or disordered contractions in those with hypercontractile motility disorders. Achalasia has objective diagnostic criteria, and effective treatments are available. Timely diagnosis results in better outcomes. Recent research suggests that hypercontractile motility disorders may be overdiagnosed, leading to unnecessary and irreversible interventions. Many symptoms ascribed to these disorders are actually due to unrecognized functional esophageal disorders. Hypercontractile motility disorders and functional esophageal disorders are generally self-limited, and there is considerable overlap among their clinical features. Endoscopy is warranted in all patients with dysphagia, but testing to evaluate for less common conditions should be deferred until common conditions have been optimally managed. Opioid-induced esophageal dysmotility is increasingly prevalent and can mimic symptoms of other motility disorders or even early achalasia. Dysphagia of liquids in a patient with normal esophagogastroduodenoscopy findings may suggest achalasia, but high-resolution esophageal manometry is required to confirm the diagnosis. Surgery and advanced endoscopic therapies have proven benefit in achalasia. However, invasive interventions are rarely indicated for hypercontractile motility disorders, which are typically benign and usually respond to lifestyle modifications, although pharmacotherapy may occasionally be needed.

Opioid-Induced Esophageal Dysfunction: Differential Effects of Type and Dose.

OBJECTIVE: Data regarding opioid effects on esophageal function are limited. We previously demonstrated an association between chronic opioid use and esophageal motor dysfunction characterized by esophagogastric junction outflow obstruction, distal esophageal spasm, achalasia type III, and possibly Jackhammer esophagus. Our aim was to characterize the influence of different opioids and doses on esophageal dysfunction. METHODS: Retrospective review of 225 patients prescribed oxycodone, hydrocodone, or tramadol for >3 months, who completed high-resolution manometry from 2012 to 2017. Demographic and manometric data were extracted from a prospectively maintained motility database. Frequency of opioid-induced esophageal dysfunction (OIED, defined as distal esophageal spasm, esophagogastric junction outflow obstruction, achalasia type III, or Jackhammer esophagus on high-resolution manometry, was compared among different opioids. The total 24-hour opioid doses for oxycodone, hydrocodone, and tramadol were converted to a morphine equivalent for dose effect analysis. RESULTS: OIED was present in 24% (55 of 225) of opioid users. OIED was significantly more prevalent with oxycodone or hydrocodone use compared with tramadol (31% vs 28% vs 12%, P = 0.0162), and for oxycodone alone vs oxycodone with acetaminophen (43% vs 21%, P = 0.0482). There was no difference in OIED for patients taking hydrocodone alone vs hydrocodone with acetaminophen. Patients with OIED were taking a higher median 24-hour opioid dose than those without OIED (45 vs 30 mg, P = 0.058). DISCUSSION: OIED is more prevalent in patients taking oxycodone or hydrocodone compared with tramadol. There is greater likelihood of OIED developing with higher doses. Reducing the opioid dose or changing to tramadol may reduce OIED in opioid users.

It's not just a heartburn and reflux disease: a case report of distal oesophageal spasm and review of literature.

Distal oesophageal spasm is a rare condition that affects the motility of the oesophagus. It can be diagnosed by highresolution oesophageal manometry and the diagnosis is supported by other modalities such as barium swallow and esophagogastroduodenoscopy examinations. Treatment options include pharmacological therapy, endoscopy and surgical interventions. We described a case of distal oesophageal spasm in an elderly patient who presented with chronic dyspepsia.

The Relevance of Spastic Esophageal Disorders as a Diagnostic Category.

PURPOSE OF REVIEW: This review addresses the similarities and differences between the spastic esophageal disorders, including jackhammer esophagus, distal esophageal spasm (DES), and type III (spastic) achalasia. The pathophysiology, diagnosis, and treatment of each separate disorder are discussed herein, with an emphasis on overlapping and discordant features. RECENT FINDINGS: The Chicago Classification is a hierarchical organizational scheme for esophageal motility disorders, currently in its third iteration, with direct impact on the definitions of these three disorders. Complementary diagnostic tools such as impedance planimetry and novel manometric parameters continue to evolve. The suite of potential treatments for these disorders is also expanding, with progressive interest in the role of peroral endoscopic myotomy alongside established pharmacologic and mechanical interventions. Although jackhammer esophagus, distal esophageal spasm, and type III achalasia frequently overlap in terms of their clinical presentation and available management approaches, the divergences in their respective diagnostic criteria suggest that additional study may reveal additional mechanistic distinctions that lead in turn to further refinements in therapeutic decision-making.

The Chicago Classification 3.0 Results in More Normal Findings and Fewer Hypotensive Findings With No Difference in Other Diagnoses.

OBJECTIVES: High-resolution manometry (HRM) is the preferred method for the evaluation of motility disorders. Recently, an update of the diagnostic criteria (Chicago 3.0) has been published. The aim of this study was to compare the performance criteria of Chicago version 2.0 (CC2.0) vs. 3.0 (CC3.0) in a cohort of healthy volunteers and symptomatic patients. METHODS: HRM studies of asymptomatic and symptomatic individuals from several centers of Spain and Latin America were analyzed using both CC2.0 and CC3.0. The final diagnosis was grouped into hierarchical categories: obstruction (achalasia and gastro-esophageal junction obstruction), major disorders (distal esophageal spasm, absent peristalsis, and jackhammer), minor disorders (failed frequent peristalsis, weak peristalsis with small or large defects, ineffective esophageal motility, fragmented peristalsis, rapid contractile with normal latency and hypertensive peristalsis) and normal. The results were compared using McNemar's and Kappa tests. RESULTS: HRM was analyzed in 107 healthy volunteers (53.3% female; 18-69 years) and 400 symptomatic patients (58.5% female; 18-90 years). In healthy volunteers, using CC2.0 and CC3.0, obstructive disorders were diagnosed in 7.5% and 5.6%, respectively, major disorders in 1% and 2.8%, respectively, minor disorders in 25.2% and 15%, respectively, and normal in 66.4% and 76.6%, respectively. In symptomatic individuals, using CC2.0 and CC3.0, obstructive disorders were diagnosed in 11% and 11.3%, respectively, major disorders in 14% and 14%, respectively, minor disorders in 33.3% and 24.5%, respectively, and normal in 41.8% and 50.3%, respectively. In both groups of individuals, only an increase in normal and a decrease in minor findings using CC3.0 were statistically significant using McNemar's test. DISCUSSIONS: CC3.0 increases the number of normal studies when compared with CC2.0, essentially at the expense of fewer minor disorders, with no significant differences in major or obstructive disorders. As the relevance of minor disorders is questionable, our data suggest that CC3.0 increases the relevance of abnormal results.

Distal esophageal spasm.

PURPOSE OF REVIEW: Distal esophageal spasm (DES) is a rare esophageal motility disorder associated with dysphagia and chest pain. In 2011, the diagnosis of DES was refined based on the occurrence of premature (rather than rapid) contractions by high-resolution manometry. New therapeutic options have also been recently proposed. Thus, a review on DES incorporating publications since 2012 is timely because of these revisions in definition and management. RECENT FINDINGS: DES remains a heterogeneous clinical disorder. Its pathophysiology is still debated and DES might be related to achalasia. Alternatively, it might be secondary to medications, especially opiates. Endoscopic ultrasound might be informative diagnostically by demonstrating muscularis propria hypertrophy and thickening. Botulinum toxin injection in the esophageal body has been shown superior to placebo to relieve symptoms associated with DES. Finally, per oral endoscopic myotomy is a promising therapeutic approach, but may be less effective in DES than in achalasia. SUMMARY: The diagnosis of DES should lead to a systematic search for medication that might promote the occurrence of esophageal dysmotility. Endoscopic treatment of DES (botulinum toxin injection or per oral endoscopic myotomy) should be further evaluated in controlled studies using current diagnostic criteria by high-resolution manometry.

[Clinical and pathogenetic features of esophageal spasm].

AIM: To comparatively analyze clinical manifestations in patients with primary esophageal spasm (ES) and its concurrence with gastroesophageal reflux disease (GERD) and the results of their instrumental examinations and psychodiagnostic tests. SUBJECTS AND METHODS: A total of 104 patients with the clinical and manometric signs of ES were examined and divided into two groups: 1) 42 patients with primary ES; 2) 62 patients with ES concurrent with GERD. The examination encompassed esophageal manometry, esophagogastroduodenoscopy, 24-hour pH metry, and an interview using a questionnaire to identify autonomic disorders, and the Mini-Mult test. RESULTS: The patients with primary ES compared to those with ES concurrent with GERD significantly more frequently showed severe pain syndrome (p = 0.009) and a paradoxical dysphagia pattern (p = 0.03); manometry revealed an incoordination in the motility of the entire esophagus (p = 0.001). Comparison of the statistical series of values for contraction amplitude and duration in the distal esophagus found no significant difference in the patients of both groups. Autonomic disturbances were detected in 76.0% of the patients with ES; but the intergroup differences were insignificant. Mental maladaptation was observed in 81.7% of the patients in the absence of intergroup differences. CONCLUSION: The etiopathogenetic factor of ES is a psychoautonomic response to chronic stress in both primary ES and its concurrence with GERD. The reflux of gastric contents into the esophagus does not appear to be one of the leading causes of ES. In primary ES, esophageal motor function is generally impaired to a much greater extent than that in ES concurrent with GERD. The degree of motor disorders is embodied in the specific clinical features of the disease.

[Diffuse oesophageal spasms: results of a retrospective manometric study]. / Maladie des spasms diffuse de l'asophage: résultats d'une etude manométrique retrospective.

BACKGROUND: Diffuse esophageal spasms is a primary motor disrder of the esophagus of unknown etiology characterized by intermettent peristalsis. This is rare condition which represents 3-5% if primary disorders of the esophagus. Diagnosis and treatment of this entity are difficult. AIMS: To evaluate the frequency and the clinical and the manometric features of diffuse esophageal spasms. METHODS: We conducted a retrospective study related to esophageal manometry performed between January 2000 and December 2011 regardless of the indication. Patients with meeting criteria for diffuse esophageal spasms (> 20 % simultaneous waves with greater than 30 mmHg pressure in the esophagus) were included. RESULTS: Out of 1188 patients, 13 (1,09 %) met the manometric criteria for diffuse esophageal spasms. It was 8 women and 5 men with a median age of 57 years. Dysphagia was the most relevant symptom and chest pain was only noted in 1 patient. The frequency of simultaneous waves was between 20 and 80 %. The pressure of the lower esophageal sphincter was normal in most cases. CONCLUSION: In this Tunisian manometric study, the diffuse esophageal spasms is rare. Dysphagia was the most relevant symptom and the pressure of the lower esophageal sphincter was normal in most cases.

Diffuse esophageal spasm in children referred for manometry.

Diffuse esophageal spasm (DES) causes chest pain and/or dysphagia in adults. We reviewed charts of 278 subjects 0 to 18 years of age after esophageal manometry to describe the frequency and characteristics of DES in children. Patient diagnoses included normal motility (61%), nonspecific esophageal motility disorder (20%), DES (13%, n=36), and achalasia (4%). Of patients with DES, the most common chief complaint was food refusal in subjects younger than 5 years (14/24, 58%) and chest pain in subjects older than 5 years (4/12, 33%). Comorbid medical conditions, often multiple, existed in 33 subjects. DES should be considered when young children present with food refusal.

Progression of diffuse esophageal spasm to achalasia: incidence and predictive factors.

The progression of certain primary esophageal motor disorders to achalasia has been documented; however, the true incidence of this decay is still elusive. This study aims to evaluate: (i) the incidence of the progression of diffuse esophageal spasm to achalasia, and (ii) predictive factors to this progression. Thirty-five patients (mean age 53 years, 80% females) with a manometric picture of diffuse esophageal spasm were followed for at least 1 year. Patients with gastroesophageal reflux disease confirmed by pH monitoring or systemic diseases that may affect esophageal motility were excluded. Esophageal manometry was repeated in all patients. Five (14%) of the patients progressed to achalasia at a mean follow-up of 2.1 (range 1-4) years. Demographic characteristics were not predictive of transition to achalasia, while dysphagia (P= 0.005) as the main symptom and the wave amplitude of simultaneous waves less than 50 mmHg (P= 0.003) were statistically significant. In conclusion, the transition of diffuse esophageal spasm to achalasia is not frequent at a 2-year follow-up. Dysphagia and simultaneous waves with low amplitude are predictive factors for this degeneration.

Distal esophageal spasm in high-resolution esophageal pressure topography: defining clinical phenotypes.

BACKGROUND: The manometric diagnosis of distal esophageal spasm (DES) uses "simultaneous contractions" as a defining criterion, ignoring the concept of short latency distal contractions as an important feature. Our aim was to apply standardized metrics of contraction velocity and latency to high-resolution esophageal pressure topography (EPT) studies to refine the diagnosis of DES. METHODS: Two thousand consecutive EPT studies were analyzed for contractile front velocity (CFV) and distal latency to identify patients potentially having DES. Normal limits for CFV and distal latency were established from 75 control subjects. Clinical data of patients with reduced distal latency and/or rapid CFV were reviewed. RESULTS: Of 1070 evaluable patients, 91 (8.5%) had a high CFV and/or low distal latency. Patients with only rapid contractions (n = 186 [17.4%] using conventional manometry criteria; n = 85 [7.9%] using EPT criteria) were heterogeneous in diagnosis and symptoms, with the majority ultimately categorized as weak peristalsis or normal. In contrast, 96% of patients with premature contraction had dysphagia, and all (n = 24; 2.2% overall) were ultimately managed as spastic achalasia or DES. CONCLUSIONS: The current DES diagnostic paradigm focused on "simultaneous contractions" identifies a large heterogeneous set of patients, most of whom do not have a clinical syndrome suggestive of esophageal spasm. Incorporating distal latency into the diagnostic algorithm of EPT studies improves upon this by isolating disorders of homogeneous pathophysiology: DES with short latency and spastic achalasia. We hypothesize that prioritizing measurement of distal latency will refine the management of these disorders, recognizing that outcomes trials are necessary.

Esophageal motor disorders: how to bridge the gap between advanced diagnostic tools and paucity of therapeutic modalities?

High-resolution manometry has added significantly to our current understanding of esophageal motor function by providing improved detail and a data analysis paradigm that is more akin to an imaging format. Esophageal pressure topography provides a seamless dynamic representation of the pressure profile through the entire esophagus and thus, is able to eliminate movement artifact and also assess intrabolus pressure patterns as a surrogate for bolus transit mechanics. This has led to improved identification of anatomic landmarks and measurement of important physiological parameters (esophagogastric junction relaxation, distal latency, and contractile integrity). This research has bridged the gap into clinical practice by defining physiologically relevant phenotypes that may have prognostic significance and improve treatment decisions in achalasia, spasm, and hypercontractile disorders. However, more work is needed in determining the etiology of symptom generation in the context of normal or trivial motor dysfunction. This research will require new techniques to assess visceral hypersensitivity and alterations in central modulation of pain and discomfort.

Esophageal spasm: demographic, clinical, radiographic, and manometric features in 108 patients.

Diffuse esophageal spasm (DES) remains insufficiently understood. Here we aimed to summarize the demographic, clinical, radiographic, and manometric features in a large cohort of patients with DES. We identified all consecutive patients diagnosed with DES from 2000 to 2006 at Mayo Clinic Florida. The computerized records of these patients were reviewed to extract relevant information. We performed 2654 esophageal motilities during that period. There were 108 patients with esophageal spasm, and 55% were female. Median age was 71 years. The most common leading symptom was dysphagia in 55, followed by chest pain in 31. Weight loss occurred in 28 patients. The median of time from onset of symptoms to diagnosis was 48 months (range 0-480), with a median of time from the first medical consultation to diagnosis of 8 months (range 0-300). The most frequent comorbidities were hypertension and psychiatric problems. At presentation, 81 patients were taking acid-reducing medications, and 49 patients were taking psychotropic drugs. An abnormal esophagogram was noted in 46 of 76 patients with this test available, but most radiographic findings were nonspecific with the typical 'corkscrew' appearance seen in only three patients. Gastroesophageal reflux disease (GERD) was diagnosed by pH testing or endoscopy in 41 patients. We did not find any difference between the rate of simultaneous contractions or esophageal amplitude between patients with a leading symptom of dysphagia and those with chest pain. DES is an uncommon motility disorder that often goes unrecognized for years. Physicians should be aware of the clinical heterogeneity of DES and consider motility testing early in the course of unexplained esophageal symptoms. Given the high prevalence of GERD in DES, the role of GERD and the impact of acid-reducing therapy in DES deserve further study.

Distal esophageal spasm.

Distal esophageal spasm (DES) is an uncommon esophageal motility disorder associated with dysphagia and/or chest pain. Its pathophysiology implies an impairment of esophageal inhibitory neural function. Using conventional manometry, DES was defined by the presence of simultaneous esophageal contractions. With the introduction of high-resolution manometry and esophageal pressure topography (EPT) in clinical practice, rapidly propagated contractions are nonspecific of esophageal spasm. Hence, a more physiological and clinically relevant definition was proposed. Distal latency (DL) measures the period of inhibition that precedes contraction in the distal esophagus immediately proximal to the esophagogastric junction (EGJ). Premature contractions, defined as reduced DL, appeared to be much more specific for DES in EPT. Premature contractions with normal EGJ relaxation constitute DES, while premature contractions with impaired EGJ relaxation are diagnostic of spastic achalasia. Because of the interaction between DES and gastroesophageal reflux disease, 24-h esophageal pH monitoring should also be considered in patient evaluation. Medical treatment of DES aims to compensate for the deficient inhibitory neural function. Sildenafil, which blocks nitric oxide degradation and thus prolongs esophageal muscle relaxation, is a promising treatment. Endoscopic injection of botulinum toxin in the esophageal muscle is also an interesting therapeutic option. Finally, extended surgical myotomy might be discussed in extreme cases after failure of other therapeutic options.

An audit of combined multichannel intraluminal impedance manometry in the assessment of dysphagia.

BACKGROUND: Multichannel Intraluminal Impedance (MII) Monitoring is a method of examining oesophageal bolus transit without the need for radiation. In combination with oesophageal manometry it allows correlation of bolus transit with peristaltic activity. The clinical application of impedance manometry is still being refined. This audit looked to examine whether impedance manometry had advantages over standard manometry in assessment of patients with dysphagia. METHODS: 41 patients with the presenting symptom of dysphagia were assessed by combined MII and oesophageal manometry at a Wellington Hospital between February 2008 and December 2009. Each underwent manometry and MII using standardised techniques. FINDINGS: Achalasia was diagnosed in 23 patients (56.1%), Ineffective oesophageal motility (IEM) in 5 patients (12.2%), Diffuse oesophageal Spasm (DES) in 7 patients (17.1%), and Nutcracker oesophagus in 2 patients (4.9%). 4 patients had normal manometry studies (9.8%). All patients with achalasia, IEM, and DES had abnormal bolus transit. All patients with normal manometry had abnormal bolus transit. Both patients with nutcracker oesophagus had normal bolus transit. 4 patients with achalasia had undergone previous Hellers myotomy. Two of these patients (50.0%) now had normal LES relaxation pressures, but all four still had abnormal oesophageal peristalsis and abnormal bolus transit. INTERPRETATION: Multichannel Intraluminal Impedance manometry has advantages over standard manometry in characterising the physiological abnormalities associated with dysphagia. Patients in this study had severe defects including achalasia where bolus transit was invariably poor meaning little further information was gained. Extension of this study to include a wider group of patients with dysphagia may yield different results.

Diffuse esophageal spasm: has the term lost its relevance? Analysis of 217 cases.

Diffuse esophageal spasm (DES) has been reported as a potential cause of dysphagia or chest pain; however, the patho-physiology of DES is unclear. The aim of this study was to examine the manometric correlates of dysphagia and chest pain in this patient population. All patients undergoing manometry at our institution are entered into a prospectively maintained database. After institutional review board approval, the database was queried to identify patients meeting criteria for DES (≥20% simultaneous waves with greater than 30 mm Hg pressure in the distal esophagus). The patient-reported symptoms and manometric data, along with the results of a 24-hour pH study (if done), were extracted for further analysis. Out of 4923 patients, 240 (4.9%) met the manometric criteria for DES. Of these, 217 patients had complete manometry data along with at least one reported symptom. Of the patients with DES, 159 (73.3%) had dysphagia or chest pain as a reported symptom. Patients reporting either dysphagia or chest pain had significantly higher lower esophageal sphincter (LES) pressure than patients without these symptoms (P= 0.007). Significant association was noted between reported dysphagia and percentage of simultaneous waves. Chest pain did not correlate with percent of simultaneous waves, mean amplitude of peristalsis, or 24-hour pH score. The origin of reported chest pain in patients with DES is not clear but may be related to higher LES pressure. Simultaneous waves were associated with reported dysphagia. Using current diagnostic criteria, the term DES has no clinical relevance.

Managing diffuse oesophageal spasm.

Non-cardiac chest pain is common,1 affecting around 25% of the population during their lifetime and accounting for about 2-5% of presentations to hospital accident and emergency departments.2 Around 10% of patients presenting with such pain, and up to 13% of those presenting with functional dysphagia, have diffuse oesophageal spasm (DOS), an oesophageal motility disorder.3,4 DOS is often recognised and treated only after patients have attended hospital emergency departments and specialist clinics for years, because of the non-specific nature of their symptoms and difficulty in diagnosis (features common to motility disorders).5 Here we discuss the diagnosis and further management of patients with DOS.

Chicago Classification Update (v4.0): Technical review on diagnostic criteria for distal esophageal spasm.

Distal esophageal spasm (DES) is defined as a manometric pattern of at least 20% of premature contractions in a context of normal esophago-gastric junction relaxation in a patient with dysphagia or non-cardiac chest pain. The definition of premature contraction requires the measurement of the distal latency and identification of the contractile deceleration point (CDP). The CDP can be difficult to localize, and alternative methods are proposed. Further, it is important to differentiate contractile activity and intrabolus pressure. Multiple rapid swallows are a useful adjunctive test to perform during high-resolution manometry to search for a lack of inhibition that is encountered in DES. The clinical relevance of the DES-manometric pattern was raised as it can be secondary to treatment with opioids or observed in patients referred for esophageal manometry before antireflux surgery in absence of dysphagia and non-cardiac chest pain. Further idiopathic DES is rare, and one can argue that when encountered, it could be part of type III achalasia spectrum. Medical treatment of DES can be challenging. Recently, endoscopic treatments with botulinum toxin and peroral endoscopic myotomy have been evaluated, with conflicting results while rigorously controlled studies are lacking. Future research is required to determine the role of contractile vigor and lower esophageal sphincter hypercontractility in the occurrence of symptoms in patients with DES. The role of impedance-combined high-resolution manometry also needs to be evaluated.

Esophageal motility disorders on high-resolution manometry: Chicago classification version 4.0©.

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.