Navigating reflux disease after achalasia treatments: Balancing risks and benefits.

The peroral endoscopic myotomy (POEM) procedure has revolutionized the management of achalasia in many centres around the world as it offers patients a minimally invasive endoscopic solution to their dysphagia caused by achalasia. Alongside its success in alleviating dysphagia, concerns regarding postoperative gastroesophageal reflux disease have emerged as a pertinent issue which are not fully resolved. In this study, Nabi et al have comprehensively reviewed the topic of the prediction, prevention and management of gastroesophageal reflux after POEM. POEM is a purely endoscopic procedure which is usually performed without any anti-reflux procedure. Certain patients may be better served by a laparoscopic Heller's myotomy and fundoplication and it is important that gastroenterologists and surgeons provide comprehensive risks and benefits of each achalasia treatment option so that patients can decide what treatment is best for them. This article by Nabi et al provides a comprehensive review of the current status of this issue to allow these discussions to occur.

The evolution of the treatment of esophageal achalasia. Chronicle of a 35-year journey.

Over the last few decades, significant improvement has been made in both the evaluation and treatment of esophageal achalasia. The Chicago classification, today in version 4.0, is now the standard for diagnosis of achalasia, providing a classification into 3 subtypes with important therapeutic and prognostic implications. Therapy, which was at first mostly limited to pneumatic dilatation, today includes minimally invasive surgery and peroral endoscopic myotomy, allowing for a more tailored approach to patients and better treatment of recurrent symptoms. This review chronicles my personal experience with achalasia over the last 35 years, describing the progress made in the treatment of patients with achalasia.

Effect of esophageal body recoil on clinical outcomes in non-spastic achalasia.

BACKGROUND: Despite the established efficacy of achalasia treatments on symptomatic outcomes, there are limited data evaluating the treatment effect on esophageal dilatation. This study aimed to assess the effect achalasia treatment on esophageal dilatation and the effect of esophageal width reduction ("recoil") on clinical outcomes. METHODS: Patients with type I or type II achalasia that completed high-resolution manometry (HRM), functional lumen imaging probe (FLIP), and timed barium esophagram (TBE) pre and post treatment were included. Esophageal width was measured using TBE. Focused subgroup analysis was performed on patients with normal posttreatment EGJ opening on FLIP. Good clinical outcomes were defined as barium column height of <5 cm at 5 min and Eckardt Score ≤3. KEY RESULTS: Sixty-nine patients (41% type I and 59% type II) were included. Esophageal width decreased from pre to post treatment mean (SD) 4.2 (1.3) cm-2.8 (1.2) cm; p < 0.01. In the normal post treatment EGJ opening subgroup, esophageal width was less in patients with good TBE outcome compared to poor outcome mean (SD) 2.2 (0.7) cm versus 3.2 (1.4) cm (p < 0.01), but did not differ in good versus poor symptomatic outcome groups. Esophageal width recoil >25% posttreatment was associated with a greater rate of good TBE outcome (71% vs. 50%, p = 0.04) and good symptomatic outcome (88% vs. 50%; p = 0.04). CONCLUSIONS AND INFERENCES: Esophageal recoil was associated with good achalasia treatment outcome in patients without posttreatment EGJ obstruction. This suggests that mechanical properties of the esophageal wall, likely associated with tissue remodeling, play a role in clinical outcomes following achalasia treatment.

Measuring and improving quality in esophageal care and swallowing disorders.

Evaluating clinical care through quality-related metrics is increasingly common. There are now numerous quality statements and indicators related to the medical management of benign and pre-malignant esophageal diseases. Expert consensus leveraging evidence-based recommendations from published society guidelines has been the most frequently used basis for developing esophageal quality statements. While surgical care of patients with esophageal malignancies, including squamous cell carcinoma, has also been developed, those related to benign esophageal disease now include domains of diagnosis, treatment, and monitoring for gastroesophageal reflux disease, eosinophilic esophagitis (EoE), achalasia, and Barrett's esophagus (BE). Several recent studies evaluating adherence to quality metrics affirm substantial variation in practice patterns with opportunities for improvement in care across esophageal diseases. In particular, patient education regarding treatment options in achalasia, frequency of esophageal biopsies among patients with dysphagia to evaluate for EoE, and endoscopic evaluation within a BE segment are areas identified to have need for improvement. As the management of esophageal diseases becomes more complex and interdisciplinary, adherence to quality metrics may be a source of standardization and improvement in delivery and ultimately patient outcomes. Indeed, the development of national quality databases has resulted in a significant growth in the use of these metrics for quality improvement activities and may form the basis for future inclusion in quality reporting and payment programs.

Modern insights into the pathophysiology and treatment of pseudoachalasia.

BACKGROUND: Secondary achalasia or pseudoachalasia is a clinical presentation undistinguishable from achalasia in terms of symptoms, manometric, and radiographic findings, but associated with different and identifiable underlying causes. METHODS: A literature review was conducted on the PubMed database restricting results to the English language. Key terms used were "achalasia-like" with 63 results, "secondary achalasia" with 69 results, and "pseudoachalasia" with 141 results. References of the retrieved papers were also manually reviewed. RESULTS: Etiology, diagnosis, and treatment were reviewed. CONCLUSIONS: Pseudoachalasia is a rare disease. Most available evidence regarding this condition is based on case reports or small retrospective series. There are different causes but all culminating in outflow obstruction. Clinical presentation and image and functional tests overlap with primary achalasia or are inaccurate, thus the identification of secondary achalasia can be delayed. Inadequate diagnosis leads to futile therapies and could worsen prognosis, especially in neoplastic disease. Routine screening is not justifiable; good clinical judgment still remains the best tool. Therapy should be aimed at etiology. Even though Heller's myotomy brings the best results in non-malignant cases, good clinical judgment still remains the best tool as well.

Medical management of painful achalasia: a patient-driven systematic review.

Achalasia is a rare esophageal disorder characterized by abnormal esophageal motility and swallowing difficulties. Pain and/or spasms often persist or recur despite effective relief of the obstruction. A survey by UK charity 'Achalasia Action' highlighted treatments for achalasia pain/spasms as a key research priority. In this patient-requested systematic review, we assessed the existing literature on pharmacological therapies for painful achalasia. A systematic review of the literature using Medline, Embase and Cochrane databases was performed to identify studies evaluating pharmacological therapies for achalasia. Methodological quality of included randomized controlled trials was assessed using the Cochrane Risk of Bias tool. In total, 70% (40/57) of survey respondents reported experiencing pain/spasms. A range of management strategies were reported. Thirteen studies were included in the review. Seven were randomized controlled trials. Most studies were >30 years old, had limited follow-up, and focussed on esophageal manometry as the key endpoint. Generally, studies found improvements in lower esophageal pressures with medications. Only one study evaluated pain/spasm specifically, precluding meta-analysis. Overall risk of bias was high. The achalasia patient survey identified that pain/spasms are common and difficult to treat. This patient-requested review identified a gap in the literature regarding pharmacological treatments for these symptoms. We provide an algorithm for investigating achalasia-related pain/spasms. Calcium channel blockers or nitrates may be helpful when esophageal obstruction and reflux have been excluded. We advocate for registry-based clinical trials to expand the evidence base for these patients.

Esophageal Motility Disorders: Diagnosis and Treatment Strategies.

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

Incidence, prevalence and clinical management of achalasia since the introduction of high-resolution manometry in Western Australia.

BACKGROUND: Epidemiological studies in achalasia and its clinical management in Australia are limited. AIMS: To determine the prevalence and trends in incidence rates and describe the types of treatment stratified by subtypes of achalasia. METHODS: A retrospective observational study was conducted at a single site that offers a state-wide high-resolution manometry (HRM) service in Western Australia (WA). Patients (aged ≥ 18 years) newly diagnosed with achalasia based on HRM findings between 2012 and 2021 were extracted from the HRM database. The crude incidence rate and age-standardised incidence rate (ASIR) along with the 2021-point prevalence were calculated. Trends were assessed by the Kendall τb test. The patients' initial and subsequent treatment modalities were described. RESULTS: A total of 296 new cases were identified, and the median age at diagnosis was 56 years. The patient's median age, sex and year of the first treatment did not vary significantly with the subtypes. The lowest and highest ASIR (cases/100 000 person-years) were 0.8 in 2012 and 2.1 in 2021, respectively. Only type 2 achalasia showed a significant increasing trend (P = 0.009). The 2021-point prevalence was 16.9 cases/100 000 people and increased with age. Pneumatic balloon dilatation (PBD) was the most common treatment for types 1 and 2, while laparoscopic Heller myotomy was most common for type 3. Peroral endoscopic myotomy (POEM) has become common in the past 5 years. CONCLUSION: The ASIR of type 2 achalasia significantly increased in WA. PBD was most commonly performed, although peroral endoscopic myotomy has recently increased as a preferred treatment option.

Assessing the diagnostic yield of achalasia using provocative testing in high-resolution esophageal manometry: Serial diagnostic study.

BACKGROUND: Chicago Classification v4.0 recommends that if achalasia is demonstrated with single water swallows (SWS); provocative testing is not required. We determine whether provocative testing in patients with suspected achalasia can change manometric findings and reproduce symptoms. METHODS: Between 2016 and 2022, 127 consecutive manometry studies of patients with achalasia were retrospectively analyzed. All patients underwent SWS, a solid meal (SM) and/or a rapid drink challenge (RDC). Demographic data, fluoroscopy, gastroscopy, and pre-and post-treatment Eckardt scores were collated. KEY RESULTS: Of 127 achalasia patients (50.6 ± 16.6 years and 54.6% male), all completed a SM and 116 (91.3%) completed RDC; overall 83 were naïve (65.4%) to previous therapy. 15.4% patients with normal integrated relaxation pressure (IRP) on SWS demonstrated obstruction with RDC. SM gave a different achalasia phenotype in 44.9% of patients (p ⟨ 0.001). Twelve patients with normal IRP during SWS had persistent/recurrent obstruction during provocative testing; 83.3% had previous achalasia therapy. None of 13 patients with Type III (TIII) achalasia with SWS exhibited a change in manometric findings with provocative testing. Impedance bolus heights were lower in patients with TIII achalasia and those with normal IRP with SWS. During the SM, symptoms were reproduced in 56.7% of patients. Forty-six of 103 patients (44.7%) underwent therapy based upon the final achalasia subtype which was defined by the provocative test result of the high-resolution manometry (HRM) study. All treatments were effective, regardless of the achalasia subtype. CONCLUSIONS AND INFERENCES: Manometric findings remain unchanged when TIII achalasia is diagnosed with SWS. In patients with normal IRP, Type I, or Type II achalasia during SWS, provocative testing can alter achalasia phenotype or uncover achalasia where diagnosis is unclear. Further, it can reproduce symptoms. Such findings can personalize and guide effective therapeutic decisions.

[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.

True peristaltic recovery is uncommon following treatment, particularly endoscopic dilation for achalasia cardia, though pseudo-recovery often occurs.

BACKGROUND: Relieving esophagogastric junction (EGJ) obstruction has been the focus of treatment for achalasia cardia. The recovery of peristalsis has been an elusive goal. Studies analyzing post-intervention peristaltic recovery have several limitations such as the use of conventional manometry or lack of standard definitions of peristalsis. Accordingly, we undertook this study to analyze frequency and pattern of peristaltic recovery following treatment for achalasia cardia on high-resolution manometry (HRM) and standard Chicago definition of peristalsis. METHODS: Pre and post-intervention HRM records of 71 treatment-naive patients diagnosed as achalasia cardia were retrospectively analyzed. Records with pre and post-intervention HRM on different systems (e.g. solid state and water perfusion) and those with inadequate information were excluded. All HRMs were interpreted as per Chicago classification version 3.0. After pneumatic dilation (PD) or laparoscopic Heller's myotomy (LHM), pseudorecovery of peristalsis was defined as any contraction at least 3 cm in length along 20 mmHg isobaric contour with a distal latency of less than 4.5 seconds. True recovery and premature contractions were defined by standard Chicago classification v3.0 criteria. RESULTS: Change in diagnosis was observed in 38 of 71 (53.5%) patients after intervention. While pseudo-peristaltic recovery occurred in 11 of 71 (15.5%) patients, only three (4.2%) had a true recovery. Another nine (12.7%) patients showed new premature contractions. CONCLUSION: True peristaltic recovery is uncommon in achalasia cardia following intervention, particularly PD. Pseudo-peristaltic recovery is more common. Further research is warranted on this issue.

Achalasia cardia sub-types in children: Does it affect the response to therapy?

BACKGROUND: Achalasia sub-types affect treatment response in adults, but there is no similar data in children. We studied the differences in clinico-laboratory features and response to therapy between achalasia sub-types in children. METHODS: Forty-eight children (boys:girls-25:23, 14 [0.9-18] years) with achalasia (clinical, barium, high-resolution manometry [HRM], gastroscopy) were evaluated. The sub-type was determined by Chicago classification at HRM. Pneumatic dilatation (PD) or surgery was the primary therapy. Success was defined as Eckhardt score of ≤ 3. RESULTS: Dysphagia (95.8%) and regurgitation (93.8%) were the most common symptoms. Forty of 48 cases had an adequate HRM study: Type I (n-19), II (n-19) and III (n-2). Types I and II had similar clinical profile. Type II had higher basal lower esophageal sphincter (LES) pressure (30.5 [16.5-46] vs. 22.5 [13-43] mmHg; p = 0.007) and less dilated esophagus on timed barium esophagogram (TBE, 25 [13-57] vs. 34.5 [20-81] mm; p = 0.006) than type I. Both types had similar success (86.6% [13/15] vs. 92.8% [13/14]; p = 1) after first PD and need of post-PD myotomy (5/17 vs. 1/16; p = 0.1) in follow-up. Twenty-three cases had TBE before and after PD; 15 (65.2%) had good clearance. These subjects required myotomy (1/15 vs. 4/8; p = 0.03) and repeat PD (5/15 vs. 4/8; p = 0.08) less often than those with poor clearance on TBE. CONCLUSION: Types I and II achalasia have similar frequency and clinical profile. Type II has higher LES pressure and less dilated esophagus than Type I. Both respond equally well to initial PD. Type I required post-PD myotomy more often, though not significantly. TBE is useful for assessing therapeutic response.

Clinical characteristics and manometric findings of esophageal achalasia-a systematic review regarding differences among three subtypes.

Esophageal achalasia is classified into three subtypes according to manometric findings. Since several factors, including clinical characteristics and treatment response, have been reported to differ among the subtypes, the underlying pathogenesis may also differ. However, a comprehensive understanding regarding the differences is still lacking. We therefore performed a systematic review of the differences among the three subtypes of achalasia to clarify the current level of comprehension. In terms of clinical features, type III, which is the least frequently diagnosed of the three subtypes, showed the oldest age and most severe symptoms, such as chest pain. In contrast, type I showed a higher prevalence of lung complications, and type II showed weight loss more frequently than the other types. Histopathologically, type I showed a high loss of ganglion cells in esophagus, and on a molecular basis, type III had elevated serum pro-inflammatory cytokine levels. In addition to peristalsis and the lower esophageal sphincter (LES) function, the upper esophageal sphincter (UES) function of achalasia has attracted attention, as an impaired UES function is associated with severe aspiration pneumonia, a fatal complication of achalasia. Previous studies have indicated that type II shows a higher UES pressure than the other subtypes, while an earlier decline in the UES function has been confirmed in type I. Differences in the treatment response are also crucial for managing achalasia patients. A number of studies have reported better responses in type II cases and less favorable responses in type III cases to pneumatic dilatation. These differences help shed light on the pathogenesis of achalasia and support its clinical management according to the subtype.

Influence of Anxiety and Depression on the Symptoms of Achalasia Patients: A Retrospective Cohort Study in China during COVID-19.

BACKGROUND: Idiopathic achalasia (AC) may be affected by anxiety and/or depression; however, reliable evidence is still lacking. The present retrospective cohort study aimed to explore the influence of psycho-mental factors on the severity of AC. METHODS: All patients in the AC database of the Tianjin Medical University General Hospital from 2012 to 2020 were divided into two subgroups, intervention (n = 202) and medication (n = 84), according to previous treatments. Healthy people (n = 300) who underwent gastrointestinal endoscopy comprised the control group. The severity of symptoms and the anxiety and depression score of AC patients and controls were monitored by telephone and compared before and during COVID-19. In addition, the factors of AC symptoms during the COVID-19 were discussed by multiple linear regression. RESULTS: During COVID-19, the anxiety and depression levels of AC patients and healthy individuals were deteriorated. For AC patients, before and after COVID-19, symptoms, anxiety, and depression scores in the medication group were more serious than those in the intervention group. Furthermore, previous therapy, depression, and gender were found to be significantly related to the severity of AC symptoms during COVID-19. CONCLUSIONS: The outbreak of COVID-19 made AC patients and healthy people anxious and depressed. Depression rather than anxiety might worsen the AC symptoms. Interventional therapy might protect AC patients against psychological abnormalities during COVID-19.

Achalasia, from diagnosis to treatment.

INTRODUCTION: Achalasia is an uncommon esophageal motility disorder and is characterized by alterations of the motility of the esophageal body in conjunction with altered lower esophageal sphincter (LES) relaxation. The clinical presentation of patients with achalasia may be complex; however, the most frequent symptom is dysphagia. The management of patients with achalasia is often challenging, due to the heterogeneous clinical presentation. AREAS COVERED: The diagnosis and management of achalasia has significantly improved in the last years due to the growing availability of high-resolution manometry (HRM) and the implementation in the therapeutic armamentarium of new therapeutic endoscopic procedures. Traditional therapeutic strategies include botulinum toxin injected to the LES and pneumatic balloon dilation. On the other hand, surgical treatments contemplate laparoscopic Heller myotomy and, less frequently, esophagectomy. Furthermore, in the last few years, per oral endoscopic myotomy (POEM) has been proposed as the main endoscopic therapeutic alternative to the laparoscopic Heller myotomy. EXPERT OPINION: Diagnosis and treatment of achalasia still represent a challenging area. However, we believe that an accurate up-front evaluation is, nowadays, necessary in addressing patients with achalasia for a more accurate diagnosis as well as for the best treatment options.

Achalasia: investigation and management.

Achalasia, characterised by the absence of peristalsis and failure of relaxation of the lower oesophageal sphincter, is an uncommon degenerative condition that results in dysphagia. If left untreated it can lead to aspiration, oesophageal perforation, oesophagitis and malnutrition. It has a range of immune, allergic, viral and genetic aetiological causes. Successful diagnosis relies on the use of oesophagogastroduodenoscopy, barium swallow and oesophageal manometry to characterise the severity of the disease and to rule out underlying malignancy. Although no treatment can reverse the degenerative process, therapeutic strategies including lifestyle modification, medication, endoscopic and operative intervention can help to reduce symptoms. This article reviews the latest methods used to investigate and manage achalasia.

Rapid Drink Challenge During High-resolution Manometry for Evaluation of Esophageal Emptying in Treated Achalasia.

BACKGROUND & AIMS: Incomplete esophageal emptying is a key variable predicting symptom relapse after achalasia treatment. Although optimally evaluated using the timed barium esophagogram (TBE), incomplete esophageal emptying can also be identified on rapid drink challenge (RDC) performed during high-resolution manometry. METHODS: We evaluated if RDC differentiates complete from incomplete esophageal emptying in treated patients with achalasia, against a TBE gold standard. Unselected treated patients with achalasia with both TBE (200 mL of low-density barium suspension) and RDC (200 mL of water in sitting position) were enrolled in 5 tertiary referral centers. TBE barium column height at 1, 2, and 5 minutes were compared with RDC variables: pressurizations >20 mmHg, maximal RDC pressurization, proportion of RDC time occupied by pressurizations, trans-esophagogastric junction gradient, and integrated relaxation pressure. RESULTS: Of 175 patients recruited (mean age, 59 years; 47% female), 138 (79%) were in clinical remission. Complete TBE emptying occurred in 45.1% at 1 minute, 64.0% at 2 minutes, and 73.1% at 5 minutes. RDC integrated relaxation pressure correlated strongly with TBE column height, and a 10-mmHg threshold discriminated complete from incomplete emptying at all 3 TBE time points with area under receiver operating characteristic curves of 0.85, 0.87, and 0.85, respectively. This threshold had high negative predictive values for complete emptying (88% at 2 minutes, 94% at 5 minutes), and modest positive predictive values for incomplete emptying (77% at 2 minutes, 62% at 5 minutes). CONCLUSIONS: RDC during high-resolution manometry is an effective surrogate for TBE in assessing esophageal emptying in treated patients with achalasia.

Development of a Preliminary Question Prompt List as a Communication Tool for Adults With Achalasia: A Modified Delphi Study.

BACKGROUND: Question prompt lists (QPLs) are structured sets of disease-specific questions that enhance patient-physician communication by encouraging patients to ask questions during consultations. AIM: The aim of this study was to develop a preliminary achalasia-specific QPL created by esophageal experts. METHODS: The QPL content was derived through a modified Delphi method consisting of 2 rounds. In round 1, experts provided 5 answers to the prompts "What general questions should patients ask when given a new diagnosis of achalasia" and "What questions do I not hear patients asking, but given my expertise, I believe they should be asking?" In round 2, experts rated questions on a 5-point Likert scale. Questions considered "essential" or "important" were accepted into the QPL. Feedback regarding the QPL was obtained in a pilot study wherein patients received the QPL before their consultation and completed surveys afterwards. RESULTS: Nineteen esophageal experts participated in both rounds. Of 148 questions from round 1, 124 (83.8%) were accepted into the QPL. These were further reduced to 56 questions to minimize redundancy. Questions were categorized into 6 themes: "What is achalasia," "Risks with achalasia," "Symptom management in achalasia," "Treatment of achalasia," "Risk of reflux after treatment," and "Follow-up after treatment." Nineteen patients participated in the pilot, most of whom agreed that the QPL was helpful (84.2%) and recommended its wider use (84.2%). CONCLUSIONS: This is the first QPL developed specifically for adults with achalasia. Although well-received in a small pilot, follow-up studies will incorporate additional patient feedback to further refine the QPL content and assess its usability, acceptability, and feasibility.

Update on the Diagnosis and Treatment of Achalasia.

Achalasia is a rare disease of the esophagus with impaired relaxation of the lower esophageal sphincter and aperistalsis. The etiology is unknown but speculations include a viral or autoimmune etiology. All specialists dealing with swallowing and esophageal diseases should recognize the classic symptoms of dysphagia for solids/liquids, regurgitation, and choking, especially at night. High-resolution manometry is critical for the diagnosis with endoscopy and barium esophagram having a supportive role. The disease cannot be cured but most can return to near normal swallowing and a regular diet with appropriate therapy. Treatment includes smooth muscle relaxants, botulinum toxin injections to the lower sphincter, pneumatic dilation, Heller myotomy, and peroral endoscopic myotomy. One treatment does not fit all and a tailored approach through a multidiscipline team will give the best long-term outcomes.