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Query: UMLS:C0014848 (achalasia)
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Noncardiac chest pain is a heterogeneous condition for which diagnosis and treatment are challenging. Research is needed to streamline evaluation to minimize unnecessary invasive testing and costs. Chest pain clinics to assess chest pain patients are popular in the United States and may be of value in reassuring patients and reducing presentation to hospital; however, recently this has been contended [111]. Options for the effective treatment of NCCP are dependent on the risk of an adverse outcome and the cost-effectiveness of the management algorithm that is followed. Most (64%) of those presenting to the emergency department with chest pain are classified as having NCCP [112,113]. GERD is probably the most important cause and application of a test of acid suppression with a high-dose PPI for 1 to 2 weeks seems to be a useful diagnostic tool. In those patients with GERD-related NCCP, short-term and potentially long-term therapy with a PPI (commonly higher than standard dose) is required to alleviate symptoms. Esophageal dysmotility is relatively uncommon in patients with NCCP and evaluation by esophageal manometry might be limited to rule out achalasia. Chest wall syndromes are common but probably often missed. Many patients with NCCP have psychologic or psychiatric abnormalities, as either the cause or an effect of the chest pain, but diagnosis here depends on techniques not applied easily in the acute situation. Pain modulators seem to offer significant improvement in chest pain symptoms for non-GERD-related NCCP. Finally, trials of management strategies to deal with this problem are required urgently, because the earlier discharge of patients with NCCP may exacerbate the problem. Fig. 2 provides a flow chart for diagnosis and treatment of NCCP.
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PMID:Noncardiac chest pain: evaluation and treatment. 1285 5

Achalasia is a primary oesophageal motor disorder characterised by the abnormal relaxation of the lower oesophageal sphincter (LES) and absent oesophageal peristalsis. It is a rare disease, with an estimated incidence of approximately 1/100,000 and a prevalence close to 10/100,000 [1]. Its exact aetiology remains unknown. Autoimmune, infectious, degenerative and hereditary processes have all been proposed as factors that lead to a chronic inflammatory response in the myenteric plexus, thus resulting in selective loss of inhibitory neurons [2] and failure of the LES to relax and aperistalsis in the body of the oesophagus. The most common symptoms of achalasia are dysphagia for solids and liquids, regurgitation, chest pain, weight loss and heartburn in > 90 approximately 75, 40 - 50, approximately 60, approximately 40%, respectively [3,4]. The diagnosis is based on symptoms, barium swallow and manometry. A barium oesophagram typically shows a dilated oesophagus that tapers into a 'bird-beak' at the gastro-oesophageal junction with lack of normal peristalsis on fluoroscopic evaluation. The characteristic manometric features of achalasia are abnormal LES relaxation and aperistalsis; additionally, the LES pressure is frequently high, but can also be normal. Current practice of medicine is faced with rising healthcare costs and limited budgets [5]. We are therefore confronted with an increasing demand to justify the value of our therapeutic interventions, not only from the risk/benefit standpoint but also from the cost perspective [6,7].
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PMID:Cost-assessment of alternative management strategies for achalasia. 1459 55

Botulinum toxin A (BoTx), a potent inhibitor of acetylcholine release from nerve endings both within the myenteric plexus and at the nerve-muscle junction, has been shown to decrease the lower esophageal sphincter (LES) pressure in patients with achalasia. Because of this property, the esophageal injection of BoTx has been suggested as an alternative treatment in achalasia. The objective of this study was to determine the long-term efficacy and safety of intrasphincteric injection of BoTx in a group of achalasic patients. Nineteen patients (mean age 56.1 +/- 19.2 years) were enrolled in the study. All of them were injected endoscopically with 100 U of BoTx by sclerotherapy needle at different sites of the LES. Symptom score (dysphagia, regurgitation and chest pain, each on a 0-3 scale), esophageal manometer and esophageal radionuclide emptying were assessed before the treatment and at 4 weeks, 3 months and 1 year after BoTx injection. In case of failure or relapse (symptom score > 2), the treatment was repeated. All but five patients (74%) were in clinical remission at 1 month. Mean symptom score after 1 month of BoTx decreased from 7.1 +/- 0.9 to 2.2 +/- 2.5 (p < 0.05). LES pressure decreased from 38.4 +/- 13.7 to 27.4 +/- 13.5 mmHg (p < 0.05) and 10-min radionuclide retention decreased from 70.9 +/- 20.7% to 33.8 +/- 27.0% (p < 0.05). Side-effects (transient chest pain) were mild and infrequent. At 12 months, the clinical score was 0.9 +/- 0.5 (p < 0.05 vs. basal); mean LES pressure was 22.0 +/- 7.1 (p < 0.05 vs. basal) and 10-min radionuclide retention was 15.8 +/- 6.0% (p < 0.05 vs. basal). The efficacy of the first injection of BoTx lasted for a mean period of 9 months (range 2-14 months). At the time of writing (follow-up period mean 17.6 months, range 2-31), 14 patients (10 with one injection) were still in remission (74%). Our results showed that one or two intrasphincteric injections of BoTx resulted in clinical and objective improvement in about 74% of achalasic patients and are not associated with serious adverse effects; the efficacy of BoTx treatment was long lasting; this procedure could be considered an attractive treatment, especially in elderly patients who are poor candidates for more invasive procedures.
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PMID:Long-term follow-up of achalasic patients treated with botulinum toxin. 1460 98

Minimal invasive surgery using a laparoscopic or thoracoscopic approach for cardiomyotomy (Heller myotomy) has become a widely accepted procedure to treat achalasia of the esophagus. In this study, we evaluated the long-term results of Heller myotomy achieved by performing video-assisted thoracic surgery (VATS). We recruited patients with achalasia who had undergone VATS for Heller myotomy from 1991 to 2000 at the National Taiwan University Hospital. The myotomy was performed 6 cm above and 1 cm below the gastroesophageal junction. No fundoplication was performed during the procedure. The symptom score, which included dysphagia, regurgitation, and chest pain, was evaluated before and after surgery. Body weight was also recorded before and after surgery. The cases of 14 patients (4 men, 10 women) were studied. The mean patient age was 41.8 +/- 4.9 years. No postoperative mortality or morbidity was found in these patients. The follow-up duration was 56 +/- 7.17 months. The dysphasia score improved from 3.0 preoperatively to 0.79 +/- 0.30 postoperatively (p=0.001). The reflux score improved from 2.64 +/- 0.17 preoperatively to 0.50 +/- 0.20 postoperatively (p=0.001). All the improvements were still in place at the time of the most recent follow-up examinations. Heller myotomy to treat achalasia using a thoracoscopic approach can provide satisfactory long-term results.
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PMID:Enduring effects of thoracoscopic Heller myotomy for treating achalasia. 1464 47

Hypertensive lower esophageal sphincter (LES) is an uncommon manometric abnormality found in patients with dysphagia and chest pain, and is sometimes associated with gastroesophageal reflux disease (GERD). Preventing reflux by performing a fundoplication raises concerns about inducing or increasing dysphagia. The role of myotomy in isolated hypertensive LES is also unclear. The aim of this study was to determine the outcome of surgical therapy for isolated hypertensive LES and for hypertensive LES associated with GERD. Sixteen patients (5 males and 11 females), ranging in age from 39 to 89 years, with hypertensive LES (>26 mm Hg; i.e., >95th percentile of our control population) who had surgical therapy between 1996 and 1999 were reviewed. Patients with a diagnosis of achalasia and diffuse esophageal spasm were excluded. All patients had dysphagia or chest pain. Eight of 16 patients had symptoms of GERD, four had a type III hiatal hernia, and four had isolated hypertensive LES pain. Patients with hypertensive LES and GERD or type III hiatal hernia had a Nissen fundoplication, and those with isolated hypertensive LES had a myotomy of the LES with partial fundoplication. Outcome was assessed as follows: excellent if the patient was asymptomatic; good if symptoms were present but no treatment was required; fair if symptoms were present and required treatment; and poor if symptoms were unimproved or worsened. All patients were contacted by telephone for symptom assessment at a median of 3.6 years (range 3 to 6.1 years) after surgery. Patients with hypertensive LES and GERD or type III hiatal hernia had significantly lower LES pressure than those with isolated hypertensive LES (29.9 vs. 47.4 mm Hg; P=0.013). Dysphagia and chest pain were relieved in all patients at long-term follow up. Outcome was excellent in 10 of 16, good in 3 of 16, and fair in 3 of 16. All patients but one were satisfied with their outcome. Patients with hypertensive LES are a heterogeneous group in regard to symptoms and etiology. Treatment of patients with hypertensive LES should be individualized. A Nissen fundoplication for hypertensive LES with GERD or type III hiatal hernia relieves dysphagia and chest pain suggesting reflux as an etiology. A myotomy with partial fundoplication for isolated hypertensive LES relieves dysphagia and chest pain suggesting a primary sphincter dysfunction.
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PMID:Surgical management of hypertensive lower esophageal sphincter with dysphagia or chest pain. 1467 8

The primary aim of this study was to identify factors that influence outcome of the surgical treatment of achalasia. A secondary aim was to compare outcomes after laparoscopic Heller myotomy and partial fundoplication using either a Dor or Toupet hemifundoplication. Between 1994 and 2002, a total of 78 patients underwent laparoscopic Heller myotomy and partial fundoplication. Preoperative investigations included esophageal manometry, a videoesophogram, and upper gastrointestinal endoscopy with biopsy. In 64 patients (35 males and 29 females), telephone contact was possible at a median 24 months (IQR 14-34). A Dor fundoplication was performed in 41 patients and a Toupet fundoplication in 23. Symptoms were assessed prior to surgery and at follow-up by an independent physician using standardized definitions to grade the severity of dysphagia, regurgitation, and chest pain. To assess outcome, dysphagia was categorized as persistent or resolved. Persistent was defined as dysphagia that occurred on a weekly or daily basis. Resolved was defined as dysphagia that occurred occasionally or not at all. At follow-up, patients were asked to make a personal evaluation of their outcome as to whether (1) their swallowing was improved by the procedure, (2) they were satisfied with the outcome, and (3) they would undergo surgery again under the same circumstances. There was a significant improvement in dysphagia and regurgitation scores after surgery (P<0.05). The scores for chest pain/heartburn remained unchanged. By physician assessment, dysphagia was resolved in 49 patients (77%) and persisted in 15 (33%). By patient assessment, 62 patients (97%) reported an improvement in the symptom of dysphagia, and 60 (94%) stated that they were satisfied with their improvement and would undergo surgery if they had to make the choice again. On univariate analysis, patients who had resolution of their dysphagia had a significantly higher resting lower esophageal sphincter (LES) pressure prior to myotomy (P=0.01) and on multivariate analysis only a high resting LES pressure prior to surgery was a predictor of resolution of dysphagia (P=0.015). Outcome comparison of patients with Dor and Toupet fundoplications showed no significant differences in physician assessment of postoperative symptom scores and resolution of dysphagia, patient assessment of outcome, or postoperative use of proton pump inhibitors. Ninety-four percent of patients are satisfied with their surgical myotomy for achalasia. By physician assessment dysphagia was resolved in 77% of patients. A high LES resting pressure before surgery predicted resolution of dysphagia.
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PMID:Preoperative lower esophageal sphincter pressure affects outcome of laparoscopic esophageal myotomy for achalasia. 1501 30

Disorders of the upper digestive tract have a high impact on modern society, in terms of both direct and indirect health care costs and of social burden. The most common presenting symptom is either dysphagia or dyspepsia. Discriminating specific diagnoses within this wide group of diseases requires sound clinical judgment and application of procedures to distinguish organic from nonorganic disease and to further characterize the functional or motility disturbance of nonorganic diseases. Non-radionuclide-based diagnostic techniques include both noninvasive tests (upper gastrointestinal barium series, ultrasonography, and breath test for gastric emptying) and invasive procedures (fiberoptic endoscopy, esophagogastroduodenoscopy, pharyngeal manometry, stationary esophageal manometry, 24-h pH monitoring, esophageal biliary reflux monitoring, multichannel intraluminal impedance, and electrogastrography). Some of these techniques are not well tolerated by patients or not widely available. Radionuclide transit/emptying scintigraphy provides a means of characterizing exquisite functional abnormalities with a set of low-cost procedures that are easy to perform and widely available, entail a low radiation burden, closely reflect the physiology of the tract under evaluation, are well tolerated and require minimum cooperation by patients, and provide quantitative data for better intersubject comparison and for monitoring response to therapy. Despite the relatively low degree of standardization both in the scintigraphic technique per se and in image processing, these methods have shown excellent diagnostic performance in several function or motility disorders of the upper digestive tract. Dynamic scintigraphy with a radioactive liquid or semisolid bolus provides important information on both the oropharyngeal and the esophageal phases of swallowing, thus representing a useful complement or even a valid alternative to conventional invasive tests (such as stationary esophageal manometry) for evaluating abnormalities of oropharyngoesophageal transit. Clinical applications of esophageal transit scintigraphy include disorders such as nutcracker esophagus, esophageal spasm, noncardiac chest pain of presumed esophageal origin, achalasia, esophageal involvement of scleroderma, and gastroesophageal reflux and monitoring of response to therapy (either medical or surgical treatment of disease-for example, organic disease such as esophageal cancer). Scintigraphy with a radiolabeled test meal represents the gold standard for evaluating gastric emptying, whereas more recent radionuclide methods include dynamic antral scintigraphy and gastric SPECT for assessing gastric accommodation. Clinical applications of gastric-emptying scintigraphy include, among others, evaluation of patients with dyspepsia and evaluation of gastric function in various systemic diseases affecting gastric emptying. The present review includes the proposal of clinical algorithms for evaluating patients with the main disorders of the upper digestive tract. These algorithms, originally derived from available literature, have been developed on the basis of a vast clinical experience in conjunction with the specialists more deeply involved in the care of patients with such disorders (medical and surgical gastroenterologists and nuclear medicine physicians). The role of radionuclide gastroesophageal motor studies is clearly identified in the various steps of patients' management, from the initial diagnostic approach to functional characterization to postoperative follow-up or monitoring of medical therapy.
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PMID:Radionuclide gastroesophageal motor studies. 1518 Nov 37

The present study was carried out to evaluate the diagnostic usefulness of stationary esophageal manometry in 263 patients divided into three groups: 150 patients with reflux symptoms, 68 with dysphagia, and 45 with non-cardiac chest pain. Patients with endoscopic abnormalities were excluded. Standard manometry was performed following the station pull-through technique. In the group of patients with reflux symptoms 40.7% had a normal manometry and 57.3% had abnormalities, being the most frequent (43%) hypotensive lower esophageal sphincter. In the dysphagia group, 20.6% of manometries were normal and 79.4% were abnormal, of which achalasia was the most frequent disorder (53.7%). In the case of non-cardiac chest pain, 42.2% of patients had a normal manometry and 57.8% an abnormal one, of which hypotensive lower esophageal sphincter was the most frequent abnormality. A significant higher proportion of manometric alterations were found in the dysphagia group compared to reflux symptoms and non-cardiac chest pain (p < 0.05). No statistical differences were found between the reflux and the non-cardiac chest pain groups. Manometry yields a higher diagnostic value in patients with dysphagia, and therefore manometry should be performed routinely after the exclusion of any organic esophageal disease. Manometry is not a first-choice functional diagnostic test in the study of patirnts with gastroesophageal reflux or non-cardiac chest pain.
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PMID:Role of stationary esophageal manometry in clinical practice. Manometric results in patients with gastroesophageal reflux, dysphagia or non-cardiac chest pain. 1550 2

Achalasia has been described following fundoplication and is attributed to vagal nerve damage during surgery. Similarly, other traumatic events to the distal esophagus may be linked to the development of achalasia. Operative and nonoperative trauma as a possible factor in the development of achalasia was studied. A retrospective analysis of patients with achalasia (n = 64) at our institution was performed. Collected data included age, gender, symptoms, and history of operative and nonoperative traumatic events. Comparisons were made to a group of patients with similar symptoms but normal esophageal manometry (n = 73). Achalasia was diagnosed by manometry in 125 patients over a 6-year period. All patients with complete medical records (n = 64) were studied. A history of operative or nonoperative trauma to the upper gastrointestinal tract prior to the development of symptomatic achalasia was present in 16 of 64 (25%). Significantly fewer patients (9.5%) with symptoms of dysphagia, but normal manometry and upper endoscopy, had precedent trauma to the upper gastrointestinal tract (P < 0.05). All cases of nonoperative trauma occurred in motor vehicle accidents. Cases of operative trauma included coronary artery bypass surgery (n = 4), bariatric surgery (n = 2), fundoplication (n = 3), heart/lung transplantation (n = 1), and others (n = 5). Patients with proven achalasia and a history of trauma were more likely to have chest pain (RR, 4.5; P = 0.012) but less likely to have regurgitation (RR, 0.51; P = 0.01) or nausea/vomiting (RR, 0.0; P = 0.27) than those without a history of antecedent trauma. In this series, significantly more patients with achalasia had a history of preceding trauma than did patients with similar symptoms and normal esophageal manometry. Following trauma, patients may be at increased risk for developing achalasia, possibly from neuropathic dysfunction due to vagal nerve damage. Patients with posttraumatic achalasia may have symptoms which differ from those of other achalasia patients.
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PMID:Achalasia presenting after operative and nonoperative trauma. 1562 10

Catheter-based high-frequency intraluminal ultrasound imaging is a powerful tool to study esophageal sensory and motor function and dysfunction in vivo in humans. It can be combined with manometry, pH, and impedance measurement techniques to determine the relationships between different physiologic parameters. High-frequency intraluminal ultrasound imaging has provided a number of important insights regarding the longitudinal muscle function of the esophagus. On the basis of the ultrasound images and intraluminal pressure recordings, it seems that there is synchrony in the timing and the amplitude of contraction between the circular and longitudinal muscle layers. A sustained contraction of the longitudinal muscle layer is temporally related to esophageal chest pain and heartburn. The biomechanics of the esophageal wall and its relationship to sensory and motor function can be studied in humans in vivo by using high-frequency intraluminal ultrasound much more precisely than has previously been possible. Achalasia, diffuse esophageal spasm, and nutcracker esophagus are associated with hypertrophy of circular and longitudinal muscle layers. Finally, high-frequency intraluminal ultrasound imaging is the only technique that can detect reflux-related distention of the esophagus and its role in esophageal symptoms. Future approaches to display and quantify ultrasound image data are discussed. The principles of high-frequency intraluminal ultrasound described here are also applicable to study of the motor and sensory function of the other regions of the gastrointestinal tract.
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PMID:Sensory and motor function of the esophagus: lessons from ultrasound imaging. 1568 59


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