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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

High-resolution manometry (HRM) is a gastrointestinal motility diagnostic system that measures intraluminal pressures using closely aligned sensors. Multiple rapid swallows (MRS) are used in conjunction with HRM to assess esophageal physiology prior to anti-reflux and hiatal hernia procedures. A retrospective, qualitative study was conducted on 90 patients who underwent HRM with MRS in a single community clinic. 80 patients met the inclusion criteria. MRS testing consisted of rapid 2 mL swallows in 2–3 s intervals with patients in a seated, upright position. Clinical information was reviewed including indications for HRM, prior diagnostic workup, manometry, distal contractile integral (DCI), and integrated residual pressure (IRP). HRM studies were visualized using Manoview Analysis Software v3.0 (Medtronic). Certain esophageal dysmotility and pressurization manometry patterns were previously undetected on HRM alone. In our study, the addition of MRS was clinically helpful in (1) assessing contraction reserve, (2) highlighting features of jackhammer, (3) stimulating esophageal spasm, and (4) visualizing distal esophageal pressurization pattern with mechanical obstruction. Additionally, abnormal pathophysiology such as (5) paradoxical LES contraction (achalasia) and (6) loss of deglutition inhibition were identified. MRS had a diagnostic utility of 21.25% (n = 17) among the 80 patients. An intolerance rate of 7.7% (n = 7) was observed in patients unable to complete the protocol. Augmentation of HRM with MRS produces unique manometric features that have clinical utility in uncovering esophageal disorders. MRS provocation testing is a practical, inexpensive, well-tolerated addition to HRM that may yield useful clinical information to guide complicated diagnoses and medical management.

Purpose of review Chronic opioid use is common and can cause opioid-induced esophageal dysfunction (OIED). We will discuss the pathophysiology, diagnosis, and management of OIED. Recent findings OIED is diagnosed based on symptoms, opioid use, and manometric evidence of distal esophageal spasm, esophagogastric junction outflow obstruction, achalasia type III, or jackhammer esophagus. Chronic opioid use appears to interfere with inhibitory signals involved in control of esophageal motility, allowing for unchecked excitatory stimuli, and leading to spastic contractility and impaired esophagogastric junction relaxation. Patients may present with dysphagia and chest pain. OIED is significantly more prevalent in patients taking the stronger opioids oxycodone and hydrocodone compared with the weaker opioid tramadol. Based on 24-h morphine equivalent doses, patients with OIED take higher opioid doses than those without OIED. Impaired inhibitory signaling was recently demonstrated in a study showing reduced deglutitive inhibition during multiple rapid swallows in patients taking opioids. Summary OIED is frequent in chronic opioid users undergoing manometry for esophageal symptoms, especially at higher doses or with stronger opioids. OIED appears to be due to impaired inhibitory signals in the esophagus. Opioid cessation or dose reduction is recommended, but studies examining management of OIED are lacking.

Distal esophageal spasm (DES) is a rare motility disorder characterized by premature and rapidly propagated contractions of the distal esophagus. Treatment options are limited and often poorly effective. Peroral endoscopic myotomy (POEM) seems an effective and attractive new treatment option for DES. In this case report we describe some of the difficulties that could arise. A 84-year old man with therapy-refractory DES and complaints of severe dysphagia and chest pain underwent a POEM procedure under general anesthesia. A longer myotomy was performed to cleave the circular muscle layer from start till end of the spastic contractions. The length of the myotomy was 16 cm. Hyperactive spastic contractions during the procedure complicated the creation of the submucosal tunnel, extended the duration (134 vs. 60-90 min for achalasia), increased postoperative pain and prolonged hospital admission. Intravenously nitroglycerin peroperative diminished spastic contractions. Postoperative a remnant of spastic contractions was present, proximal to the myotomy, causing persistent symptoms. Performing POEM for DES is challenging due to reactive hyperactive spastic contractions during the procedure causing technical difficulties and an extended procedure. A long myotomy, several centimeters above the proximal border of the spastic region, is essential to prevent remnants of spasticity

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.

Summary Distal esophageal spasm (DES) is a motility disorder characterized by premature contraction of the esophageal body during single swallows. It is thought to be due to impairment of esophageal inhibitory pathways, but studies to support this are limited. The normal response to multiple rapid swallows (MRS) is deglutitive inhibition of the esophageal body during the MRS sequence. Our aim was to compare the response to MRS in DES patients and healthy control subjects. Response to MRS during HRM was evaluated in 19 DES patients (8 with and 11 without concomitant esophagogastric junction outflow obstruction [EGJOO]) and 24 asymptomatic healthy controls. Patients with prior gastroesophageal surgery, peroral endoscopic myotomy, pneumatic dilation, esophageal botulinum toxin injection within 6 months of HRM, opioid medication use, and esophageal stricture were excluded. Response to MRS was evaluated for complete versus impaired inhibition (esophageal body contractility with distal contractile integral [DCI] > 100 mmHg-sec-cm during MRS), presence of post-MRS contraction augmentation (DCI post MRS greater than single swallow mean DCI), and integrated relaxation pressure (IRP). Impaired deglutitive inhibition during MRS was significantly more frequent in DES compared to controls (89% vs. 0%, P

Objective: to evaluate esophageal dysmotility (ED) and the extent of Barrett’s esophagus (BE) before and after laparoscopic Nissen fundoplication (LNF) in patients previously diagnosed with BE and ED. Methods: twenty-two patients with BE diagnosed by upper gastrointestinal (GI) endoscopy with biopsies and ED diagnosed by conventional esophageal manometry (CEM) were submitted to a LNF, and followed up with clinical evaluations, upper GI endoscopy with biopsies and CEM, for a minimum of 12 months after the surgical procedure. Results : sixteen patients were male (72.7%) and six were females (27.3%). The mean age was 55.14 (± 15.52) years old. and the mean postoperative follow-up was 26.2 months. The upper GI endoscopy showed that the mean length of BE was 4.09 cm preoperatively and 3.91cm postoperatively (p=0.042). The evaluation of esophageal dysmotility through conventional manometry showed that: the preoperative median of the lower esophageal sphincter resting pressure (LESRP) was 9.15 mmHg and 13.2 mmHg postoperatively (p=0.006). The preoperative median of the esophageal contraction amplitude was 47.85 mmHg, and 57.50 mmHg postoperatively (p=0.408). Preoperative evaluation of esophageal peristalsis showed that 13.6% of the sample presented diffuse esophageal spasm and 9.1% ineffective esophageal motility. In the postoperative, 4.5% of patients had diffuse esophageal spasm, 13.6% of aperistalsis and 22.7% of ineffective motor activity (p=0.133). Conclusion: LNF decreased the BE extension, increased the LES resting pressure, and increased the amplitude of the distal esophageal contraction; however, it was unable to improve ED.

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.

POEM has been successfully performed in patients with spastic esophageal disorders (SED), such as diffuse esophageal spasm, jackhammer esophagus, and type 3 achalasia. We performed a systematic review and meta-analysis to evaluate its efficacy in these patients and if total average myotomy length and prior medical or endoscopic treatments affected clinical success. PubMed, EMBASE, Google-Scholar, Scopus, and Cochrane Review were searched for studies on POEM in SED from 2008 to September 2018. Clinical success was determined by Eckardt score (≤ 3) at follow-up. Sub-group analysis was performed based on myotomy length and evaluates the effect of prior treatments on clinical success. 9 studies with 210 patients were included in the final analysis. We found that the pooled rate of clinical success for POEM was 89.6% (95% CI 83.5–93.1, 95% PI 83.4–93.7, I2 = 0%). In three studies (50 patients), where total myotomy length was 10 cms and the pooled rate of clinical success was 89.1% (95% CI 83.0–93.2, I2 = 0%). The difference between these results was not statistically significant (p = 0.69). Additionally, a meta-regression analysis showed that prior treatment status did not significantly affect the primary outcome (p = 0.43). While it is well known that POEM is a safe and effective treatment for spastic esophageal disorders, we conclude that variation in total myotomy length and prior endoscopic or medical treatments did not have a significant effect on clinical success.

Spastic esophageal disorders (SEDs) include spastic achalasia (type III), diffuse esophageal spasm (DES), and nutcracker/jackhammer esophagus (JH). Per-oral endoscopic myotomy (POEM) has demonstrated efficacy and safety in the treatment of achalasia. Recently, POEM has been indicated for the treatment of SEDs. We conducted a systematic review and meta-analysis to determine the clinical success and safety of POEM in SEDs. We searched several databases from 01/01/2007 to 01/10/2016 to identify studies (with five or more patients) on POEM for the treatment of SEDs. Weighted pooled rates (WPRs) for clinical success and adverse events (AEs) were calculated for all SEDs. Clinical success was defined as Eckardt scores of ≤3 and/or improvement in severity of dysphagia based on achalasia disease-specific health-related quality of life questionnaire. The WPRs for clinical success and AEs were analyzed using fixed- or random-effects model based on heterogeneity. The proportionate difference in clinical success and post-procedure adverse event rates among individual types of SEDs was also calculated. A total of eight observational studies with 179 patients were included in the final analysis. Two studies were of good quality and six were of fair quality based on the National Institutes of Health quality assessment tool. The WPR with 95% confidence interval (CI) for cumulative clinical success of POEM in all SEDs was 87% (78, 93%), I 2 = 37%. The total number of patients for individual disorders, i.e., type III achalasia, JH, and DES, was 116, 37, and 18, respectively. The WPRs for clinical success of POEM for type III achalasia, DES, and JH were 92, 88, and 72%, respectively. Proportion difference of WPR for clinical success was significantly higher for type III achalasia in comparison with JH (20%, P = 0.01). The WPR with 95% CI for AEs of POEM in all SEDs was 14% (9, 20%), I 2 = 0%. The WPRs for post-procedure adverse events for type III achalasia, DES, and JH were 11, 14, and 16%, respectively. There was no difference in safety of POEM among individual SEDs. POEM is an effective and safe therapeutic modality for the treatment of spastic esophageal disorders.

Background In the past, ambulatory 24-h manometry has been shown useful for the evaluation of patients with non-cardiac chest pain (NCCP). With the diagnostic improvements brought by pH-impedance monitoring and high-resolution manometry (HRM), the contribution of ambulatory 24-h manometry to the diagnosis of esophageal hypertensive disorders has become uncertain. Our aim was to assess the additional diagnostic yield of ambulatory manometry to HRM and ambulatory pH-impedance monitoring in this patient population. Methods All patients underwent 24-h ambulatory pressure-pH-impedance monitoring and HRM. Patients had retrosternal pain as a predominant symptom and no explanation after cardiologic and digestive endoscopic evaluations. Diagnostic measurements were analyzed by two independent physicians. Key Results Fifty-nine patients met the inclusion criteria; 37.3% of the patients had their symptoms explained by abnormalities on pH-impedance monitoring and 6.8% by ambulatory manometry. Functional chest pain was diagnosed in 52.5% of the patients. High-resolution manometry, using the Chicago Classification v3.0 criteria alone, did not identify any of the four patients with esophageal spasm on ambulatory manometry. However, taking into account other abnormalities, such as simultaneous (rapid) or repetitive contractions, HRM had a sensitivity of 75% and a specificity of 98.2% for the diagnosis of esophageal spasm. Conclusions & Inferences In the work-up of NCCP, ambulatory 24-h manometry has a low additional diagnostic yield. However, it remains the best technique to identify esophageal spasm as the cause of symptoms. This is particularly useful when an unequivocal diagnosis is needed before treatment.

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. 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.

Acute oesophageal symptoms include acute dysphagia or food bolus impaction (most commonly due to strictures, Schatzki ring and eosinophilic oesophagitis), acute chest pain with odynophagia due to oesophageal infections, motility disorders and acute oesophageal rupture (of which oesophageal intramural haematoma is a subtype). Acute full thickness oesophageal rupture carries a high mortality if not recognised early; the clinical features and conditions with which this may be confused are presented and discussed.

Background Limited data exist on the use of peroral endoscopic myotomy (POEM) for therapy of spastic esophageal disorders (SEDs). Objective To study the efficacy and safety of POEM for the treatment of patients with diffuse esophageal spasm, jackhammer esophagus, or type III (spastic) achalasia. Design Retrospective study. Setting International, multicenter, academic institutions. Patients All patients who underwent POEM for treatment of SEDs refractory to medical therapy at 11 centers were included. Interventions POEM. Main Outcome Measurements Eckardt score and adverse events. Results A total of 73 patients underwent POEM for treatment of SEDs (diffuse esophageal spasm 9, jackhammer esophagus 10, spastic achalasia 54). POEM was successfully completed in all patients, with a mean procedural time of 118 minutes. The mean length of the submucosal tunnel was 19 cm, and the mean myotomy length was 16 cm. A total of 8 adverse events (11%) occurred, with 5 rated as mild, 3 moderate, and 0 severe. The mean length of hospital stay was 3.4 days. There was a significant decrease in Eckardt scores after POEM (6.71 vs 1.13; P = .0001). Overall, clinical response was observed in 93% of patients during a mean follow-up of 234 days. Chest pain significantly improved in 87% of patients who reported chest pain before POEM. Repeat manometry after POEM was available in 44 patients and showed resolution of initial manometric abnormalities in all cases. Limitations Retrospective design and selection bias. Conclusion POEM offers a logical therapeutic modality for patients with SEDs refractory to medical therapy. Results from this international study suggest POEM as an effective and safe platform for these patients.

Distal esophageal spasm is a rare motility disorder presenting principally with nonobstructive dysphagia and noncardiac chest pain. In symptomatic patients, the manometric diagnosis is made when >10% of the wet swallows have simultaneous and/or premature contractions intermixed with normal peristalsis. We characterize manometry and barium as complementary diagnostic approaches, and given the intermittent nature of the disorder, one should be always aware that it is almost impossible to rule out spasm. Treatment is difficult; we propose an approach beginning with the least invasive intervention.

Distal esophageal spasm (DES) is a primary esophageal motility disorder. We encountered a rare case of DES accompanied by multiple esophageal diverticula. A 72-year-old woman complained of prolonged dysphagia and chest pain. A barium esophagogram showed multiple esophageal diverticula and significant contraction of the lower esophagus just above the cardia. Esophagogastroduodenoscopy revealed a corkscrew-like appearance, with spiral contractions and diverticula. High-resolution manometry revealed that the integrated relaxation pressure was normal; premature contractions were observed in ≥20% of the swallowing wave; the distal contractile integral was normal. She was diagnosed with DES according to the Chicago classification v 3.0. As smooth muscle relaxants were not effective, we decided to perform peroral endoscopic myotomy (POEM) to eliminate persistent esophageal contraction. After POEM treatment, her symptoms were markedly improved, and the Eckardt score significantly decreased from 11 points to 1. An esophagogram after POEM showed that barium flowed promptly into the stomach. The multiple esophageal diverticula were considered to be the result of false pulsion diverticulosis caused by excessive internal esophageal pressure, and this represented the most severe form of DES. POEM could be a new curative strategy for the most severe DES cases with multiple diverticula.

In this issue of the journal, Mion and coworkers describe 23 patients with dysphagia and/or thoracic pain and hypercontractile esophageal motility disorders who were either treated with botulinum toxin 100 U or underwent a sham procedure. The surprising outcome of the study was that both active botulinum toxin treatment and sham treatment resulted in a significant reduction in symptoms and manometric abnormalities after 3 months, with no difference between the two arms. One can interpret the lack of effect of botulinum toxin over placebo as indicative of the benign natural history of hypercontractile esophageal motility disorders or be convinced that many patients with hypercontractility on manometry actually have functional symptoms not related to the manometric findings, and thus, treatment of hypercontractility is not more effective than placebo. Either way, invasive, irreversible treatments with potential risks for complications seem difficult to justify in these patients.

The concept of esophageal spastic disorders encompasses spastic achalasia, distal esophageal spasm, and jackhammer esophagus. These are conceptually distinct in that spastic achalasia and distal esophageal spasm are characterized by a loss of neural inhibition, whereas jackhammer esophagus is associated with hypercontractility. Hypercontractility may also occur as a result of esophagogastric junction outflow obstruction or inflammation. The diagnosis of jackhammer esophagus as a primary motility disorder is based on the characteristic manometric findings after ruling out mechanical obstruction and eosinophilic esophagitis. Despite the differences in pathophysiology among the esophageal spastic disorders, their management is similar.

Purpose of Review High-resolution manometry (HRM) is increasingly performed worldwide, to study esophageal motility. The Chicago classification is subsequently applied to interpret the manometric findings and facilitate a diagnosis of esophageal motility disorders. This review will discuss new insights regarding the diagnosis and management using the Chicago classification. Recent Findings Recent studies have demonstrated that high-resolution manometry is superior to conventional manometry, and has a higher sensitivity to diagnose achalasia. Furthermore, the subclassification of achalasia as used in the Chicago classification has prognostic value and can be used to direct treatment. Diagnosis of esophageal spasm has been improved by using the distal latency as diagnostic criterion. Recently, criteria for minor disorders of peristalsis have been sharpened, leading to a lower rate of patients with abnormal results, thereby increasing the relevance of a diagnosis. Summary High-resolution manometry is now considered the gold standard for diagnosis of esophageal motility disorders. The Chicago classification provides a standardized approach for analysis and categorization of abnormalities that has led to a significant increase in our knowledge regarding the diagnosis and management of motility disorders. Further refinement of the classification will be required.

Autoimmune autonomic ganglionopathy (AAG) is an immune-mediated disorder that leads to various autonomic failures associated with anti-ganglionic acetylcholine receptor antibodies (anti-gAChR-Abs). Diffuse esophageal spasm (DES) is an uncommon esophageal motility disorder. We herein report the case of a 68-year-old woman with DES as a partial symptom of AAG. She presented with chronic esophageal transit failure, constipation, and numbness of the hands and feet, Adie's pupil, thermal hypoalgesia, and decreased deep tendon reflexes. Right sural nerve biopsy showed significantly decreased numbers of small myelinated fibers. Barium swallowing X-ray showed repetitive simultaneous contractions indicating DES in the esophagus. Gastrointestinal endoscopy and CT image showed a dilated esophageal lumen and liquid effusion. Simultaneously, serum anti-gAChR-α3-Ab indicating AAG was detected. After pulse intravenous methylprednisolone (IVMP) and intravenous immunoglobulin therapy (IVIg), the bolus progression and liquid effusion improved, suggesting that DES is an important gastrointestinal symptom of AAG.

Corkscrew esophagus (also referred as rosary bead esophagus) is a classic finding of diffuse esophageal spasm (DES) in barium studies reflecting abnormal contractions, leading to compartmentalization and curling of the esophagus, ultimately giving an appearance similar to a corkscrew or rosary beads. We review the pathophysiology of this finding, correlating it to corkscrew and rosary images that originated this classic description.

The best-defined primary esophageal motor disorder is achalasia. However, symptoms such as dysphagia, regurgitation and chest pain can be caused by other esophageal motility disorders such as Diffuse Esophageal Spasm (DES), Nutcracker Esophagus (NE) and the Hypertensive Lower Esophageal Sphincter (HTN-LES). Most patients with DES and HTN-LES who complain of dysphagia improve after a myotomy. Patients with NE whose main complaint is chest pain, often do not have relief of the pain and can even develop dysphagia as a consequence of the myotomy. POEM is a relatively new procedure, and there are no studies with long-term follow-up and no prospective and randomized trials comparing it to surgical myotomy. Overall, the key to success is based on a complete evaluation and a careful patient selection. The best results, regardless of the technique, are in fact obtained in patients with outflow obstruction and impaired esophageal emptying, a picture similar to achalasia.

Distal oesophageal spasm is a rare and under-investigated motility abnormality. Recent studies indicate effective bolus transit in varying percentages of distal oesophageal spasm patients.Explore functional aspects including contraction onset velocity and contraction amplitude cut-off values for simultaneous contractions to predict complete bolus transit.We re-examined data from 107 impedance-manometry recordings with a diagnosis of distal oesophageal spasm. Receiver operating characteristic analysis was conducted, regarding effects of onset velocity on bolus transit taking into account distal oesophageal amplitude and correcting for intra-individual repeated measures.Mean area under the receiver operating characteristic curve for saline and viscous swallows were 0.84±0.05 and 0.84±0.04, respectively. Velocity criteria of30 cm/s when distal oesophageal amplitude100 mmHg and 8 cm/s when distal oesophageal amplitude100 mmHg for saline and 32cm/s when distal oesophageal amplitude100 mmHg and7 cm/s when distal oesophageal amplitude100 mmHg for viscous had a sensitivity of 75% and specificity of 80% to identify complete bolus transit. Using these criteria, final diagnosis changed in 44.9% of patients. Abnormal bolus transit was observed in 50.9% of newly diagnosed distal oesophageal spasm patients versus 7.5% of patients classified as normal. Distal oesophageal spasm patients with distal oesophageal amplitude100 mmHg suffered twice as often from chest pain than those with distal oesophageal amplitude100 mmHg.The proposed velocity cut-offs for diagnosing distal oesophageal spasm improve the ability to identify patients with spasm and abnormal bolus transit.

We studied the spectrum of motor dysphagia in a northern Indian tertiary referral center. In this retrospective study, consecutive patients with motor dysphagia referred to the Gastrointestinal Pathophysiology and Motility Laboratory from 2002 to 2007 were evaluated clinically and with eight-channel water-perfusion manometry. Causes of dysphagia were diagnosed using standard criteria. Of 250 patients (age 41.3 [15.0] years, 146 men), 193 (77%) had achalasia cardia (AC) and 57 (23%) had other causes (11, 4.4%: diffuse esophageal spasm [DES]; 9, 3.6%: hypertensive lower esophageal sphincter [Hy LES]); manometry was normal in 37 patients. Twenty-seven patients (14%) had vigorous AC. Duration of dysphagia at presentation was longer in those with AC and Hy LES than in normal manometry (NM) (21 months [1–180] vs. 6 [1–360], p = 0.000; 24 months [7–48] vs. 6 [1–360], p = 0.015). Regurgitation and bolus obstruction were more frequent in those with AC than in NM (89/154, 57.79% vs. 3/27, 11.11%, p = 0.000001). Heartburn was less frequent in patients with AC than in others (AC: 4/146, 2.73% vs. normal: 4/27, 14.8% [p = 0.02] and others: 3/15, 20% [p = 0.018]). Chest pain was reported by 74/135 (54.8%) classic and 12/19 (63.2%) vigorous AC (p = NS). Patients with NM had lower LES pressure than those with classic AC, Hy LES and vigorous AC (p < 0.0001 in each case). Patients with DES had lower LES pressure than in classic AC, Hy LES and vigorous AC (p = 0.043, p < 0.0001, and p = 0.002, respectively). Patients with classic AC had lower LES pressure than in Hy LES and vigorous AC (p = 0.024, p = 0.001, respectively). Classic AC was the commonest cause of motor dysphagia in our center. AC was associated with higher LES pressure, longer duration of dysphagia, frequent regurgitation and bolus obstruction.

ObjectiveTo evaluate the utility of computerized manometry (CM) to identify pharyngoesophageal segment (PES) spasm during tracheoesophageal speech.Study DesignProspective clinical, controlled study.Subjects and MethodsIntraluminal pressures of the PES were collected in 12 tracheoesophageal speakers without spasm and 8 tracheoesophageal speakers with PES spasm before and after localized injection of botulinum toxin to the PES. All subjects underwent voice analysis and videofluoroscopy in addition to CM before and after treatment.ResultsAll tracheoesphageal speakers with PES spasm presented with mean intraluminal pressures greater than 16 mmHg (mean, 25.36 mmHg). In contrast, mean intraluminal pressures of subjects without spasm was 11.76 mmHg (P < 0.05). The negative predictive value associated with the use of 16 mmHg as a threshold value for spasm was 100%.ConclusionCM is a clinically useful tool to aid in speech rehabilitation for tracheoesophageal speakers. Intraluminal pressures of greater than 16 mmHg...

he treatment of noncardiac chest pain (NCCP) is challenging due to the eterogeneous nature of the disorder. NCCP may be caused or associated ith a spectrum of clinical conditions, including gastroesophageal reflux, isceral hyperalgesia, esophageal motility disorders, and psychiatric onditions. It is also possible that many patients may suffer from more han one source of pain, although this has not been critically studied. election of therapy is frequently aimed at the suspected underlying isorder. The purpose of this paper is to provide a comprehensive review f available treatment modalities for NCCP.

By far, GERD is the most common cause of NCCP, accounting for up to 60% of the patients. 3 Among patients with non-GERD-related NCCP, up to 30% have an esophageal motor disorder. 4‐6 However, 70% of those with non-GERD-related NCCP have normal esophageal motility but may demonstrate alteration in esophageal pain perception (visceral hyperalgesia). Thus, the main esophageal underlying mechanisms for NCCP include GERD, esophageal motility abnormalities, and visceral hypersensitivity. Differentiating between the underlying mechanisms of NCCP on a clinical basis has not been a gratifying experience. A recent study could not find a significant difference when comparing the quality and characteristics of chest pain between patients with and without GERD-related NCCP. 7 That included chest pain site, radiation, and relationship to food, exercise, and sleep. Only chest pain that was relieved by antacids and the presence of classic GERD symptoms (heartburn and acid regurgitation) were predictive of GERDrelated NCCP. The latter findings were recently supported by another study from Korea demonstrating increased GERD-related findings on invasive diagnostic tools in patients with GERD-related symptoms as compared with those without GERD-related symptoms. 8 Presently, diagnosis of the underlying cause of NCCP is driven by availability of treatment and diagnostic

Background Obesity is now one of the world’s major chronic diseases. The etiology of the severe comorbid conditions associated with morbid obesity is not fully understood, and in particular the relationship between gastroesophageal reflux and obesity. Methods Sixty-seven patients were enrolled in this prospective study. Patients and control individuals were divided into four subgroups according to their body mass index (BMI). Esophageal motility was assessed using a conventional water-perfused esophageal manometry catheter, and 24-h pH-metry was carried out using multichannel intraluminal impedance equipment. Results In the group with the highest BMI (>50), contraction amplitudes in the middle and distal esophagus were significantly higher in comparison both with the control group and groups with a lower BMI (P

Summary In tracheo esophageal puncture (TEP), we carry out a myotomy of the pharynx constrictor muscle; however, about 9 to 79% of patients need such procedure. The consequence of such procedure is an increase in salivary fistula rates in the postoperative. Botulin toxin is used in an outpatient basis. Aim analyzing the efficacy of botulin toxin (BT) use in the rehabilitation of totally laryngectomized patients with tracheoesophageal voice (TEV) with spasms (S) of the pharyngoesophageal segment (PES) without myotomy. Materials and Methods We analyzed eight patients submitted to total laryngectomy (TL), rehabilitated with TEV, with speech prosthesis (SP) and struggle to utter voice because of PES spasms. They were all submitted to treatment of such motor alteration with the injection of 100 units of BT in the PES. The evaluation was based on perceptive voice analysis, video fluoroscopy (VF) of the PES, acoustic voice analysis and computerized manometry (CM) of the PES, all before and after BT injection. Study design prospective Results There was a reduction in PES CM pressure after BT injection. Acoustic analysis showed an improvement in harmonics quality after treatment. There was smoother voice utterance and spasm improvement after BT. Conclusion all patients with PES spasms presented vocal improvement after BT injection in the PES.

Background According to the Chicago classification of esophageal motility disorders, distal esophageal spasm (DES) is defined as premature esophageal contractions (distal latency [DL]

Named primary esophageal motility disorders (PEMD) present with specific manometric patterns classified as: (1) hypertensive lower esophageal sphincter, (2) nutcracker esophagus (also hypercontratile, hypertensive, or hypercontracting esophagus), (3) diffuse esophageal spasm, and (4) achalasia. These conditions, with the exception of achalasia, are rare, poorly understood, and inadequately studied. Treatment of these conditions is based on symptoms and aimed at symptomatic improvement. The authors reviewed current literature on surgical treatment of non-achalasia PEMD. The review shows that: (a) surgical therapy may be an attractive alternative in patients with PEMD; (b) proper selection of patients based on symptoms evaluation and esophageal function tests is essential; (c) laparoscopic myotomy with proximal extent tailored to manometric findings seems to be the ideal surgical therapy; and (d) esophagectomy may be necessary as a last resource due to multiple failures of surgical conservative treatment.

Most studies investigating esophageal motility among the morbidly obese have focused on the relationship between lower esophageal sphincter (LES) pressure and gastroesophageal reflux disease (GERD). Very few studies in the literature have examined motility disorders among the morbidly obese population in general outside the context of GERD. This study aimed to determine the prevalence of esophageal motility disorders in obese patients selected for bariatric surgery.A total of 116 obese patients (81 women and 35 men) selected for laparoscopic gastric banding underwent manometric evaluation of their esophagus from January to March 2003. Tracings were retrospectively reviewed for the end points of LES resting pressure, LES relaxation, and esophageal peristalsis.The study patients had a body mass index (BMI) of 42.9 kg/m2, and a mean age of 48.6 years. The following abnormal manometric findings were demonstrated in 41% of the patients: nonspecific esophageal motility disorders (23%), nutcracker esophagus (peristaltic amplitude180 mmHg) (11%), isolated hypertensive LES pressure (35 mmHg) (3%), isolated hypotensive LES pressure (12 mmHg) (3%), diffuse esophageal spasm (1%), and achalasia (1%). Only one patient with abnormal esophageal motility reported noncardiac chest pain.Despite a high prevalence of esophageal dysmotility in our morbidly obese study population, there was a conspicuous absence of symptoms. Although the patients in this study were not directly questioned with regard to esophageal symptoms, several studies in the literature support our conclusion.

Background and Aims Eight years have passed since the introduction of the per-oral endoscopy myotomy (POEM) procedure. POEM was initially received as an investigational procedure, but since the revelation of promising safety and efficacy data, it is becoming the preferred treatment for achalasia. With the recent completion of our 1000th POEM procedure, we share our experience and knowledge through the discussion of clinical pearls, pitfalls, and practical considerations. Methods The various aspects of the procedure and conditions that warrant special attention are discussed from our perspective, with a focus on areas in which there is currently limited evidence. Results The key points on patient position, submucosal tunneling, myotomy, closure, intraprocedural bleeding, and advanced sigmoid achalasia are presented. Conclusions The dissemination of this information serves as a foundation for new POEM operators and as a catalyst for more-experienced operators to further refine and advance their POEM skills and stimulate international discourse and collaboration.

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.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.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.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.Цель исследования. Сравнительный анализ клинических проявлений, результатов инструментального и психодиагностического обследования больных первичным эзофагоспазмом (ЭС) и его сочетанием с гастроэзофагеальной рефлюксной болезнью (ГЭРБ). Материалы и методы. Обследовали 104 больных с клиническими и манометрическими признаками ЭС и разделили на 2 группы: 1-я группа - 42 больных первичным ЭС, 2-я группа - 62 больных ЭС в сочетании с ГЭРБ. Обследование включало манометрию пищевода, эзофагогастродуоденоскопии, суточную рН-метрию, опрос с применением опросника для выявления вегетативных нарушений, сокращенной методики обследования личности. Результаты. При первичном ЭС по сравнению с ЭС в сочетании с ГЭРБ достоверно чаще выявляются значительная интенсивность болевого синдрома (р=0,009) и парадоксальный характер дисфагии (р=0,03); по данным манометрии, чаще констатируется несогласованность моторики всего пищевода (р=0,001). При сравнении статистических рядов показателей амплитуды и длительности сокращений в дистальном отделе пищевода у больных обеих групп достоверных различий не выявлено. Вегетативные нарушения определены у 76% больных ЭС, но межгрупповые различия недостоверны. Нарушения психической адаптации отмечены у 81,7% больных в отсутствие межгрупповых различий. Заключение. Этиопатогенетическим фактором развития ЭС является психовегетативная реакция на хронический стресс как при первичном ЭС, так и при его сочетании с ГЭРБ. Создается впечатление, что рефлюкс желудочного содержимого в пищевод не относится к ведущим факторам развития ЭС. При первичном ЭС моторная функция пищевода в целом нарушена в значительно большей степени, чем при ЭС в сочетании с ГЭРБ. Степень выраженности моторных нарушений находит отражение в особенностях клинических проявлений заболевания.

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.

We report the case of a 60-year-old woman with metastatic breast cancer whose intractable nausea and vomiting were effectively managed with a hyoscine hydrobromide (scopolamine) patch. Contrast swallow revealed oesophageal spasm to be the underlying cause. Symptom relief may be attributed to the antimuscarinic properties of the patch, allowing lower oesophageal sphincter relaxation. Following patch use she was able to enjoy small meals and fluids without symptoms. This is the first time this mechanism of action of scopolamine for alleviating nausea and vomiting has been described in the literature.

SUMMARY. Laparoscopic Heller myotomy for achalasia has a 10–20% failure rate and may require re-operation to control persistent or recurrent symptoms. We report follow-up of 15 patients who underwent laparoscopic re-operation for failed Heller myotomy. Between 1993 and 2004, 15 patients underwent laparoscopic re-operation for failed Heller myotomy at our center. The mean duration between procedures was 23 months. Follow-up was completed at a mean duration of 30 months in 14 patients (93%) via a telephone questionnaire. Our overall failure rate for primary surgery (n = 106) was 5.6%. The mechanisms of failure were incomplete myotomy (33%), myotomy fibrosis (27%), fundoplication disruption (13%), too tight fundoplication (7%) and a combination of myotomy fibrosis and incomplete myotomy (20%). Significant symptom improvement was observed with postoperative symptom resolution seen in 71% of patients with dysphagia, 89% for regurgitation, 58% for heartburn and 40% for chest pain. Fifty percent reported excellent results and 79% would recommend the procedure to a friend. Subsequent dilations were performed in four patients (29%). Two patients required conversion to open surgery (13%). Three patients (20%) failed the re-operation and required further revisional surgery. Complications included intraoperative perforation in three (none of which resulted in postoperative morbidity) and a pneumothorax in one patient. Prior endoscopic therapies (pneumatic dilation or Botulinum toxin) were not associated with poor results. Laparoscopic re-operation for failed Heller myotomy is feasible and results are encouraging.

Mechanisms underlying coronary spasm are still poorly understood. The aim of the study was to assess the hypothesis that fluctuations in the development of coronary spasm might reflect inputs from the adjacent esophageal system. We enrolled patients admitted to the coronary care unit for episodes of nocturnal angina. Seven patients with variant angina and five with coronary artery disease (CAD) had concurrent ECG and esophageal manometric monitoring. ECG monitoring documented 28 episodes of ST elevation in variant angina patients and 16 episodes of ST depression in CAD patients. Manometric analysis showed that esophageal spasms resulted remarkably more frequently in variant angina patients (143 total spasms; individual range 9–31) than in CAD patients (20 total spasms; individual range 0–9; P < 0.01). Time series analysis was used to assess fluctuations in the occurrence of abnormal esophageal waves and its relationship with spontaneous episodes of ST shift. Episodes of esophageal spasm in CAD were sporadic (1 into 5 min before ECG-recorded ischemia) ( P < 0.05). A bidirectional analysis of causal effects showed that the influence processes between esophageal and coronary spasms were mutual and reciprocal (transfer function model, P < 0.05) in variant angina. We concluded that in variant angina patients, episodes of esophageal spasms and myocardial ischemia influenced each other. Mechanisms that cause esophageal spasm can feed back to produce coronary spasm. Coronary spasm may feed forward to produce additional episodes of esophageal spasm.

Oesophageal spasm is a common empiric diagnosis clinically applied to patients with unexplained chest pain. In contrast it is an uncommon manometric abnormality found in patients presenting with chest pain and/or dysphagia and diagnosed by >or=20% simultaneous oesophageal contractions during standardized motility testing. Using Medline we searched for diagnostic criteria and treatment options for oesophageal spasm. While the aetiology of this condition is unclear, studies suggest the culprit being a defect in the nitric oxide pathway. Well-known radiographic patterns have low sensitivities and specificities to identify intermittent simultaneous contractions. Recognizing that simultaneous contractions may result from gastro-oesophageal reflux this diagnosis should be investigated or treated first. Studies have documented improvements with proton-pump inhibitors, nitrates, calcium-channel blockers and tricyclic antidepressants or serotonin reuptake inhibitors. Small case series reported benefits after botulinium toxin injections, dilatations and myotomies. Uncertainties persist regarding the optimal management of oesophageal spasm and recommendations are based on controlled studies with small numbers of patients or on case series. Acid suppression, muscle relaxants and visceral analgetics should be tried first. Botulinium toxin injections should be reserved for patients who do not respond. Pneumatic dilatations or myotomies represent rather heroic approaches for non-responding patients.

Diffuse esophageal spasm (DES) and achalasia share both clinical and manometric characteristics. Some reports support the notion of progression of DES to achalasia. However, there are currently no prospective data in support of this theory. To assess prospectively the rate of manometric progression of DES to achalasia. Manometry tracings of DES patients diagnosed between 1992 and 2003 were independently reviewed blindly and agreed on by two esophageal experts. Patients with DES who agreed to undergo repeat esophageal manometry constituted the study cohort. Follow-up manometry tracings were evaluated blindly and independently by the same two interpreters to determine the rate of manometric progression to achalasia. Predictors of manometric progression were assessed. A total of 32 patients were diagnosed with DES between 1992-2003. Twelve patients (9M/3F; median age 62 years) agreed to participate and underwent second manometry (mean +/- SD follow-up of 4.8 +/- 3.4 years). Achalasia was diagnosed on follow-up manometry in one patient (8%), seven (58%) patients continued to have DES, three (25%) had normal motility, and one (8%) had nutcracker esophagus. There were no predictors of progression to achalasia based on the initial manometry parameters. A subgroup of DES patients with initial low esophageal body amplitude developed increase in esophageal simultaneous contractions on follow-up similar to the patient who evolved to achalasia. Following were the results. 1) Progression from DES to achalasia is uncommon. 2) DES patients with low esophageal body amplitude may develop increased simultaneous contractions over time. 3) DES remains an elusive diagnosis clinically and manometrically.