The 2018 ISDE achalasia guidelines.

Achalasia is a relatively rare primary motor esophageal disorder, characterized by absence of relaxations of the lower esophageal sphincter and of peristalsis along the esophageal body. As a result, patients typically present with dysphagia, regurgitation and occasionally chest pain, pulmonary complication and malnutrition. New diagnostic methodologies and therapeutic techniques have been recently added to the armamentarium for treating achalasia. With the aim to offer clinicians and patients an up-to-date framework for making informed decisions on the management of this disease, the International Society for Diseases of the Esophagus Guidelines proposed and endorsed the Esophageal Achalasia Guidelines (I-GOAL). The guidelines were prepared according the Appraisal of Guidelines for Research and Evaluation (AGREE-REX) tool, accredited for guideline production by NICE UK. A systematic literature search was performed and the quality of evidence and the strength of recommendations were graded according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE). Given the relative rarity of this disease and the paucity of high-level evidence in the literature, this process was integrated with a three-step process of anonymous voting on each statement (DELPHI). Only statements with an approval rate >80% were accepted in the guidelines. Fifty-one experts from 11 countries and 3 representatives from patient support associations participated to the preparations of the guidelines. These guidelines deal specifically with the following achalasia issues: Diagnostic workup, Definition of the disease, Severity of presentation, Medical treatment, Botulinum Toxin injection, Pneumatic dilatation, POEM, Other endoscopic treatments, Laparoscopic myotomy, Definition of recurrence, Follow up and risk of cancer, Management of end stage achalasia, Treatment options for failure, Achalasia in children, Achalasia secondary to Chagas' disease.

Esophageal hypermotility: cause or effect?

Nutcracker esophagus (NE), Jackhammer esophagus (JHE), distal esophageal spasm (DES), and hypertensive lower esophageal sphincter (HTLES) are defined by esophageal manometric findings. Some patients with these esophageal motility disorders also have abnormal gastroesophageal reflux. It is unclear to what extent these patients' symptoms are caused by the motility disorder, the acid reflux, or both. The aim of this study was to determine the effectiveness of laparoscopic Nissen fundoplication (LNF) on esophageal motility disorders, gastroesophageal reflux, and patient symptoms. Between 2007 and 2013, we performed high-resolution esophageal manometry on 3400 patients, and 221 patients were found to have a spastic esophageal motility disorder. The medical records of these patients were reviewed to determine the manometric abnormality, presence of gastroesophageal symptoms, and amount of esophageal acid exposure. In those patients that underwent LNF, we compared pre- and postoperative esophageal motility, gastroesophageal symptom severity, and esophageal acid exposure. Of the 221 patients with spastic motility disorders, 77 had NE, 2 had JHE, 30 had DES, and 112 had HTLES. The most frequently reported primary and secondary symptoms among all patients were: heartburn and/or regurgitation, 69.2%; respiratory, 39.8%; dysphagia, 35.7%; and chest pain, 22.6%. Of the 221 patients, 192 underwent 24-hour pH monitoring, and 103 demonstrated abnormal distal esophageal acid exposure. Abnormal 24-hour pH monitoring was detected in 62% of patients with heartburn and regurgitation, 49% of patients with respiratory symptoms, 36.8 % of patients with dysphagia, and 32.6% of patients with chest pain. Sixty-six of the 103 patients with abnormal 24-hour pH monitoring underwent LNF. Thirty-eight (13NE, 2JHE, 6 DES, and 17 HTLES) of these 66 patients had a minimum of 6-month postoperative follow-up that included clinical evaluation, esophageal manometry, and 24-hour pH monitoring. Postoperatively, all 38 patients had normal distal esophageal acid exposure. Of these 38 patients, symptoms resolved in 28 and improved in 10. Of six patients (one with NE, two JHE, and three with HTLES) that underwent postoperative esophageal manometry, five exhibited normal motility. Typical reflux symptoms are common among patients with esophageal hypermotility disorders. Abnormal 24-hour pH monitoring is present in the majority of patients with who report typical reflux symptoms and almost half of patients who report respiratory symptoms. Conversely, the majority of patients who report dysphagia or chest pain have normal distal esophageal acid exposure. Based on a small number of patients in this study, it also appears that motility disorders often improve after LNF. LNF is associated with resolution or improvement in reflux related symptoms and esophageal motility parameters in patients exhibiting abnormal esophageal acid exposure. This suggests that patient symptoms are due to abnormal acid exposure and not the motility disorder.

Prevalence of respiratory symptoms in patients with achalasia.

Achalasia is a primary esophageal motor disorder that results in poor clearance of the esophagus. Although an esophagus filled with debris and undigested food should put these patients at risk for aspiration, the frequency with which the latter occurs has never been documented. In this study, we sought to determine the incidence of respiratory symptoms and complaints in patients with achalasia. A comprehensive symptom questionnaire was administered to 110 patients with achalasia presenting to the Swallowing Center at the University of Washington between 1994 and 2008 as part of their preoperative work-up. Questionnaires were analyzed for the frequency of respiratory complaints in addition to the more typical symptoms of dysphagia, regurgitation, and chest pain. Twenty-two achalasia patients with respiratory symptoms who had also undergone Heller myotomy and completed a post-op follow-up questionnaire were analyzed as a subset. Ninety-five patients (86%) complained of at least daily dysphagia. Fifty-one patients (40%) reported the occurrence of at least one respiratory symptom daily, including cough in 41 patients (37%), aspiration (the sensation of inhaling regurgitated esophagogastric material) in 34 patients (31%), hoarseness in 23 patients (21%), wheezing in 17 patients (15%), shortness of breath in 11 patients (10%), and sore throat in 13 patients (12%). Neither age nor gender differed between those with and those without respiratory symptoms. In the subset of patients with respiratory symptoms who had undergone Heller myotomy, respiratory symptoms improved in the majority after the procedure. Patients with achalasia experience respiratory symptoms with much greater frequency than the approximately 10% that was previously believed. Awareness of this association may be important in the workup and ultimate treatment of patients with this uncommon esophageal disorder.

Does combined multichannel intraluminal esophageal impedance and manometry predict postoperative dysphagia after laparoscopic Nissen fundoplication?

Laparoscopic Nissen fundoplication (LNF) is an effective treatment for gastroesophageal reflux disease; however, some patients develop dysphagia postoperatively. Manometry is used to evaluate disorders of peristalsis, but has not been proven useful to identify which patients may be at risk for postoperative dysphagia. Multichannel intraluminal impedance (MII) evaluates the effective clearance of a swallowed bolus through the esophagus. We hypothesized that MII combined with manometry may detect those patients most at risk of developing dysphagia after LNF. Between March 2003 and January 2007, 74 patients who agreed to participate in this study were prospectively enrolled. All patients completed a preoperative symptom questionnaire, MII/manometry, and 24-h pH monitoring. All patients underwent LNF. Symptom questionnaires were administered postoperatively at a median of 18 months (range: 6-46 months), and we defined dysphagia (both preoperatively and postoperatively) as occurring more than once a month with a severity >or=4 (0-10 Symptom Severity Index). Thirty-two patients (43%) reported preoperative dysphagia, but there was no significant difference in pH monitoring, lower esophageal sphincter pressure/relaxation, peristalsis, liquid or viscous bolus transit (MII), or bolus transit time (MII) between patients with and without preoperative dysphagia. In those patients reporting preoperative dysphagia, the severity of dysphagia improved significantly from 6.8 +/- 2 to 2.6 +/- 3.4 (P < 0.001) after LNF. Thirteen (17%) patients reported dysphagia postoperatively, 10 of whom (75%) reported some degree of preoperative dysphagia. The presence of postoperative dysphagia was significantly more common in patients with preoperative dysphagia (P= 0.01). Patients with postoperative dysphagia had similar lower esophageal sphincter pressure and relaxation, peristalsis, and esophageal clearance to those without dysphagia. Neither MII nor manometry predicts dysphagia in patients with gastroesophageal reflux disease or its occurrence after LNF. The presence of dysphagia preoperatively is the only predictor of dysphagia after LNF.

Operative manometry and endoscopy during laparoscopic Heller myotomy. An initial experience.

BACKGROUND: We report our initial experience using operative esophageal manometry as an adjunct to endoscopy to determine the completeness of esophagogastric high-pressure zone (HPZ) obliteration during laparoscopic Heller myotomy. METHODS: Between July 1997 and October 1998, we performed laparoscopic Heller myotomies in 20 patients (eight male, 12 female; median age, 41 years). Mean duration of symptoms was 3.2 +/- 2.6 years (r = 0.5-11), and 45% of the patients had received prior dilation or toxin injection. A 16-channel esophageal manometry catheter was placed prior to anesthesia, with sites crossing the lower esophageal sphincter (LES). An endoscope was passed intraoperatively to localize the squamocolumnar junction, and the myotomy was performed. While the translucency was imaged in the area of the incision, we determined the adequacy of myotomy by visual assessment of LES and gastric cardia opening in response to endoscopic air insufflation. Manometry was then performed to detect any potential residual high pressure at the myotomized esophagogastric junction (EGJ). If it was found, the locus of persistent pressure was identified by probing along the myotomy, and residual muscle fibers were cut to yield a minimum pressure at the EGJ. RESULTS: A persistent HPZ was identified after the initial myotomy in 10 of 20 patients (50%). A Dor fundoplasty completed the operation. The mean operating time was 2.6 +/- 0.5 h (median, 2.5; r = 2-3.5 h), and the mean hospital stay was 1.6 +/- 1 days (median, 1, r = 1-5 days). The mean LES pressure was 2 +/- 3 mmHg immediately postmyotomy (p < 0.001 compared with preoperative value). Of 20 patients, only two have reported recurrence of dysphagia (10%). One had a recurrent HPZ on manometry, and one developed esophagitis, which resolved with omeprazole. CONCLUSIONS: Our initial experience suggests that operative esophageal manometry is a useful adjunct to upper endoscopy during laparoscopic Heller myotomy, quantitatively assuring obliteration of the nonrelaxing LES and HPZ.

Esophageal sensitivity and symptom perception in gastroesophageal reflux disease.

Patients with gastroesophageal reflux disease (GERD) experience a wide spectrum of symptoms, varying both in quality and severity. This review summarizes clinical observations of esophageal sensitivity and symptom perception in GERD patients. The Bernstein test, although lacking standardization, remains a useful tool in determining esophageal sensitivity to acid stimuli. Ambulatory 24-hour pH monitoring with symptom event marking and subsequent symptom-reflux correlation between acid reflux events and esophageal symptomatology now provides an alternative method for establishing esophageal acid sensitivity. The intraesophageal balloon distention test (IEBD) was developed to assess esophageal sensitivity to mechanical stimuli. Variants of each of these tests have been applied to the evaluation of uncomplicated GERD patients and patients with esophagitis and Barrett's metaplasia, who generally demonstrate less esophageal sensitivity than the former group. Studies using these methods have demonstrated increased esophageal sensitivity in patients with esophageal chest pain and have also identified a subset of patients with esophageal symptoms yet normal esophageal acid exposure, a condition referred to as "hypersensitive esophagus." The Bernstein test, 24-hour pH monitoring with symptom assessment, and IEBD have each contributed to our understanding of esophageal pain syndromes; it is hoped that future work in this area will lead to improved and more specific therapy for these patients.

Esophageal solid bolus transit: studies using concurrent videofluoroscopy and manometry.

OBJECTIVE: Our aim was to assess the efficacy and mechanism of solid bolus transit through the esophagus. METHODS: Eight healthy volunteers were studied with concurrent manometry and videofluoroscopy while swallowing 5 ml liquid barium, a 5-6 mm diameter bread ball, and 4 g chewed bread in both a supine and upright posture. As many as four successive swallows were performed until clearance was achieved. RESULTS: The esophageal clearance of liquid barium was 100% with the first swallow. Clearance of the unchewed bread ball occurred with the first swallow in only 6.7% of trials in the upright posture and 5.9% in the supine posture. After four swallows, clearance was 100% and 52.9% in the upright and supine postures, respectively. Chewed bread was more readily cleared than unchewed bread, with 100% clearance after two swallows in the upright posture and 91% clearance after four swallows in the supine posture. The most common locus of bread stasis was at the aortic arch and carina. The bread boluses were noted to move more effectively when localized in the head as opposed to the tail of the bolus composite. Nonocclusive contractions often occurred at the bolus tail despite the increased peristaltic amplitude seen with the chewed bread. Failed peristalsis, a frequent cause for solid clearance failure, was observed during 30% of all bread swallows. This usually occurred distal to the stopping point of the bolus, suggesting it to be the result rather than the cause of impaired transit. CONCLUSIONS: Although infrequently perceived by these normal subjects and in contradistinction to liquid clearance, bread is rarely cleared from the esophagus with a single swallow. Mastication and an upright posture facilitate the esophageal transport of solids. Bolus composition and impaired bolus transit alter the amplitude and conductance of peristalsis. Manometric data pertaining to liquid clearance through the esophagus do not readily apply to bread.

Bolus transit assessed by an esophageal stress test in postfundoplication dysphagia.

INTRODUCTION: Dysphagia is common after Nissen fundoplication but the relationship between dysphagia and bolus transit is poorly defined. This study compared bolus transit of fundoplication patients to normal individuals. METHODS: Twelve fundoplication patients and 20 healthy volunteers rated their ability to swallow eight bolus consistencies from no difficulty (0) to extreme difficulty (3) to compute a dysphagia score (range = 0-24). A 16-lumen manometric assembly was positioned across the esophagogastric junction (EGJ) and subjects were imaged fluoroscopically in a supine posture while swallowing 5 cc liquid barium and a 5-cc marshmallow-like viscoelastic barium bolus. Videofluoroscopic images were analyzed for total esophageal transit time and the fraction of time required to cross the EGJ. Manometric tracings were analyzed for the intrabolus pressure proximal to the EGJ, intragastric pressure, and distal peristaltic amplitude for each bolus. RESULTS: Dysphagia scores for fundoplication patients were significantly higher (7.3 +/- 5.1, range = 1-17) than for normals (0.5 +/- 0.6, range = 0-2). This correlated with longer total transit times for liquids and solids (r = 0.60, P < 0.01) and a greater percentage of transit time attributable to the EGJ transit. Retrograde flow at the EGJ (escape of bolus proximally up the esophagus) and peristaltic dysfunction were more frequent in fundoplication patients. However, no differences existed in manometric parameters between groups. CONCLUSIONS: Fundoplication impairs both liquid and solid esophageal bolus transit. Dysphagia perceived by fundoplication patients correlated with increased transit time, particularly across the EGJ. Combined quantitative evaluation with manometry and fluoroscopy reveals functional defects in fundoplication subjects, which are not evident by either modality alone.

Manometric heterogeneity in patients with idiopathic achalasia.

BACKGROUND & AIMS: In certain cases of achalasia, particularly those in early stages with minimal endoscopic or radiographic abnormalities, the diagnosis may rely on manometry, which is the most sensitive test for the disease. The aim of this study was to critically evaluate the manometric criteria in a population of patients with idiopathic achalasia. METHODS: Clinical histories and manometric recordings of 58 patients with idiopathic achalasia and 43 control subjects were analyzed with regard to esophageal body contraction amplitude, peristaltic effectiveness in terms of both completeness and propagation velocity, lower esophageal sphincter (LES) resting pressure, LES relaxation pressure, and intraesophageal-intragastric pressure gradient. Variants of achalasia were defined by finding manometric features that significantly differed from the remainder of achalasia patients, such that the diagnosis might be questioned. RESULTS: Four manometrically distinct variants were identified. These variants were characterized by (1) the presence of high amplitude esophageal body contractions, (2) a short segment of esophageal body aperistalsis, (3) retained complete deglutitive LES relaxation, and (4) intact transient LES relaxation. In each instance, the most extreme variant is discussed and compared with the remainder of the achalasia population and with controls. CONCLUSIONS: The significance in defining these variants of achalasia lies in the recognition that these sometimes confusing manometric findings are consistent with achalasia when combined with additional clinical data supportive of the diagnosis. Furthermore, such variants provide important clues into the pathophysiology of this rare disorder.

Reduced tLESR elicitation in response to gastric distension in fundoplication patients.

Transient lower esophageal sphincter relaxations (tLESRs) are vagally mediated in response to gastric cardiac distension. Nine volunteers, eight gastroesophageal reflux disease (GERD) patients, and eight fundoplication patients were studied. Manometry with an assembly that included a barostat bag was done for 1 h with and 1 h without barostat distension to 8 mmHg. Recordings were scored for tLESRs and barostat bag volume. Fundoplication patients had fewer tLESRs (0.4 +/- 0.3/h) than either normal subjects (2.4 +/- 0.5/h) or GERD patients (2.0 +/- 0.3/h). The tLESRs rate increased significantly in normal subjects (5.8 +/- 0.9/h) and GERD patients (5.4 +/- 0.8/h) during distension but not in the fundoplication group. All groups exhibited similar gastric accommodation (change in volume/change in pressure) in response to distension. Fundoplication patients exhibit a lower tLESR rate at rest and a marked attenuation of the response to gastric distension compared with either controls or GERD patients. Gastric accommodation was not impaired with fundoplication. This suggests that the receptive field for triggering tLESRs is contained within a wider field for elicitation of gastric receptive relaxation and that only the first is affected by fundoplication.