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Esophageal Achalasia

Esophageal achalasia is a neurogenic motor abnormality with the loss of normal peristalsis and insufficient relaxation of the lower esophageal sphincter, leading to dysphagia and chest pain, with no response to proton pump inhibitor therapy.


Presentation

Esophageal achalasia usually sets in between the second and the sixth decade of life. Onset is insidious and symptoms gradually become more severe.

Patients present with dysphagia [1] for both solids and liquids [2], which differentiates it from dysphagias that only involve solids and are characteristic of tumors of the gastroesophageal junction. Regurgitation of food and saliva is another trait of this disease. Affected individuals also describe nocturnal regurgitation that may lead to aspiration. Substernal chest pain associated with meals leads to progressively diminished amounts of ingested food and weight loss [3]. Additional symptoms include heartburn [4] and nocturnal cough caused by acid reflux or retention of food in the esophagus [5].

Affected individuals may also seek medical attention for symptoms of candida esophagitis, esophageal carcinoma or aspiration pneumonia.

The clinical examination in uncomplicated esophageal achalasia does not detect pathologic elements [6].

Weight Loss
  • We describe a case of third-trimester weight loss due to the nausea and vomiting of achalasia. Pneumatic dilation of the esophagus is often necessary to improve oral intake.[ncbi.nlm.nih.gov]
  • Abstract A 12-year-old girl presented with cough, emesis, and weight loss of 18-month duration. She was diagnosed as having achalasia of the esophagogastric junction.[ncbi.nlm.nih.gov]
  • The patient we are reporting had peritoneal mesothelioma which presented with dysphagia and weight loss, in addition to the radiological and manometric picture of achalasia.[ncbi.nlm.nih.gov]
  • Abstract We report the case of a 65-year-old woman with a 10-year history of dysphagia, regurgitation, cough, and 10-kg weight loss caused by an epiphrenic diverticulum associated with esophageal achalasia managed with a laparoscopic approach.[ncbi.nlm.nih.gov]
  • It is characterized by dysphagia, and patients often have chest pain, regurgitation, weight loss, and an abnormal barium radiograph showing esophageal dilation with narrowing at the gastroesophageal junction.[ncbi.nlm.nih.gov]
Cough
  • Abstract A 12-year-old girl presented with cough, emesis, and weight loss of 18-month duration. She was diagnosed as having achalasia of the esophagogastric junction.[ncbi.nlm.nih.gov]
  • He had complained of cough for one year and a half, and pulmonary function studies showed severe airway obstruction. The chest radiogram showed the trachea compressed by the dilated esophagus.[ncbi.nlm.nih.gov]
  • Abstract A 55-year-old man was admitted to the Department of Internal Medicine of our hospital with chief complaints of fever, cough, and right-sided chest pain.[ncbi.nlm.nih.gov]
  • Abstract We report the case of a 65-year-old woman with a 10-year history of dysphagia, regurgitation, cough, and 10-kg weight loss caused by an epiphrenic diverticulum associated with esophageal achalasia managed with a laparoscopic approach.[ncbi.nlm.nih.gov]
  • An 11-year-old boy presented with a 9-month history of frequent pneumonia, productive cough, and a 1-year history of chest discomfort and odynophagia. Chest radiograph showed changes compatible with aspiration.[ncbi.nlm.nih.gov]
Non-Cardiac Chest Pain
  • The chest pain experienced, also known as cardiospasm and non-cardiac chest pain can often be mistaken for a heart attack. It can be extremely painful in some sufferers.[en.wikipedia.org]
Dysphagia
  • EA must be considered in any DS patient presenting with dysphagia.[ncbi.nlm.nih.gov]
  • Abstract Revisional surgery for persistent or recurrent dysphagia following Heller myotomy is rare and should become even more rare if an extended myotomy has been carried out.[ncbi.nlm.nih.gov]
  • RESULTS: The operating time, intraoperative blood loss, postoperative hospital stay, and improvement of dysphagia were no significant differences between these groups.[ncbi.nlm.nih.gov]
  • Patients present with dysphagia for both solids and liquids, which differentiates it from dysphagias that only involve solids and are characteristic of tumors of the gastroesophageal junction.[symptoma.com]
  • Pneumatic dilation overcome the dysphagia in 75% of patients whose surgery was unsuccessful. Considering both the primary surgery and this ancillary treatment, the operation was effective in 97% of achalasia patients.[ncbi.nlm.nih.gov]
Progressive Dysphagia
  • Most patients have progressive dysphagia for both liquids and solids. This article describes the symptoms of achalasia, its diagnosis, and treatment. The emphasis is on primary achalasia.[ncbi.nlm.nih.gov]
  • Abstract We describe a patient who developed an ataxic sensory syndrome associated with xerophthalmia and progressive dysphagia with regurgitation.[ncbi.nlm.nih.gov]
  • Symptoms are slowly progressive dysphagia , usually to both liquids and solids, and regurgitation of undigested food. Evaluation typically includes manometry, barium swallow, and endoscopy.[msdmanuals.com]
  • Left: Retained content and air-fluid level in the dilated esophagus of a 54 year-old woman with several years of progressive dysphagia. Right: View of lower esophageal sphincter zone, which never relaxed more than a few millimeters.[endoatlas.com]
  • This 33 year old female, 8 years previously underwent an open surgery by chest approach (Heller myotomy) due to esophageal achalasia, since 2 months complained of progressive dysphagia.[gastrointestinalatlas.com]
Choking
  • She denied heartburn, regurgitation, choking or coughing during eating, chest pain, vomiting, abdominal pain, or change in bowel habits. Her medications included levothyroxine, metoprolol, pantoprazole, and warfarin.[jnmjournal.org]
  • Effortless regurgitation of bland, undigested food or whitish foam (saliva) is common and may be associated with coughing and choking at night. Some people experience chest pain, and heartburn is common.[aboutgimotility.org]
  • This symptom can cause coughing and raise the risk of aspiration , or inhaling or choking on food.[healthline.com]
  • This symptom can cause coughing and raise the risk of aspiration, or inhaling or choking on food.[healthline.com]
Regurgitation of Undigested Food
  • Symptoms are slowly progressive dysphagia , usually to both liquids and solids, and regurgitation of undigested food. Evaluation typically includes manometry, barium swallow, and endoscopy.[msdmanuals.com]
  • The major symptoms of esophageal achalasia are dysphagia, chest pain, and regurgitation of undigested food. Currently, treatment options mainly focus on relief of the symptoms by reducing the LES pressure.[clinicaltrials.gov]
  • The main symptoms of achalasia are dysphagia (difficulty in swallowing), regurgitation of undigested food, chest pain behind the sternum, and weight loss. Dysphagia tends to become progressively worse over time and to involve both fluids and solids.[en.wikipedia.org]
  • Achalasia can also manifest alongside other diseases as a rare syndrome such as achalasia microcephaly. [8] Signs and symptoms [ edit ] The main symptoms of achalasia are dysphagia (difficulty in swallowing), regurgitation of undigested food, chest pain[en.wikipedia.org]
  • “Washing foods down” with liquids is often ineffective, and relief is only obtained by belching and regurgitation of undigested food. The process may become so embarrassing that patients avoid eating in public.[enotes.tripod.com]
Deglutition Disorder
  • Disorders Esophageal Diseases Gastrointestinal Diseases Digestive System Diseases[clinicaltrials.gov]
  • Key words Achalasia Esophagus Repetitive contractions Upper esophageal sphincter Deglutition Deglutition disorders This is a preview of subscription content, log in to check access. Preview Unable to display preview. Download preview PDF.[link.springer.com]
Chest Pain
  • The presence of chest pain and of sigmoid esophagus independently predicted the failure of the procedure. CONCLUSION: Laparoscopic myotomy can durably relieve dysphagia symptoms.[ncbi.nlm.nih.gov]
  • Abstract A 55-year-old man was admitted to the Department of Internal Medicine of our hospital with chief complaints of fever, cough, and right-sided chest pain.[ncbi.nlm.nih.gov]
  • Esophageal achalasia is a neurogenic motor abnormality with the loss of normal peristalsis and insufficient relaxation of the lower esophageal sphincter, leading to dysphagia and chest pain, with no response to proton pump inhibitor therapy.[symptoma.com]
  • The results of PD were recorded and evaluated by symptom scores based on the frequency of attack of 3 major symptoms: dysphagia, regurgitation, and chest pain, both before, and then 6 weeks, 6 months, 1 year after PD was performed.[ncbi.nlm.nih.gov]
Retrosternal Chest Pain
  • Features which present in the early stages of the disease may be similar to that of gastro-oesophageal reflux, including retrosternal chest pain typically after eating and heartburn[ 11 ].[doi.org]
  • The disease manifests as symptoms of dysphagia, regurgitation of food and retrosternal chest pain [ 8 ]. Even with advancements in diagnostic tools and accurate screening methods, the etiology remains unclear.[doi.org]

Workup

Tests which are valuable when trying to establish the diagnosis of esophageal achalasia: barium esophagogram, upper gastrointestinal endoscopy, and esophageal manometry.

Barium esophagogram establishes the morphology of the esophagus and the esophagogastric junction and evaluates the peristalsis. The esophagus can be dilated, angulated or tortuous and a narrow gastroesophageal junction with "bird neck" appearance has been described. Barium emptying is impaired and prolonged [7] [8].

Esophageal manometry shows the lack of normal peristalsis [9] with improper lower esophageal sphincter relaxation [10] leading to inadequate emptying in the absence of an obstruction. The esophagus may be atonic or non-contractile. Sphincter relaxation may be impaired completely, partially or not at all [11] [12]. On the other hand, esophageal pressure may be increased in all esophageal areas.

Upper gastrointestinal endoscopy is used in cases where a mechanical obstruction cannot be excluded [13], especially since achalasia symptoms may overlap with those of an infiltrative neoplasm. When trying to enter the stomach, a pressure described as a “pop” may be felt by the endoscopist while passing through the gastroesophageal junction. Finding food or saliva above the gastroesophageal junction is pathognomonic for achalasia. Endoscopic ultrasound is especially useful if a neoplasm is suspected. Furthermore, the mucosal examination is important in differentiating achalasia from acid reflux disease, peptic strictures and candida infection [5]. Endoscopy is especially useful in patients with systemic sclerosis, where manometry also shows abnormal peristalsis.

High-resolution manometry allows a more precise diagnosis of achalasia subtype. Chest X-ray may describe chronic pneumonia due to aspiration and a small or absent gastric air bubble. The trachea may be pushed anteriorly or may be bowed, due to compression by the dilated esophagus. Computer tomography is indicated if complications are suspected, as it identifies areas of mucosal thickening. Esophageal pH measurement is important in ruling out acid reflux disease [14].

Blood workup often shows no abnormal findings.

Esophageal Motility Disorder
  • A number of 41 patients had other esophageal motility disorders and 8 patients had normal esophageal manometry.[ncbi.nlm.nih.gov]
  • Abstract Esophageal achalasia is the best described primary esophageal motility disorder. Endoscopic ultrasound (EUS) is considered a useful adjunct for grading and establishing the prognosis of these patients.[ncbi.nlm.nih.gov]
  • Abstract Esophageal achalasia is a primary esophageal motility disorder characterized by the absence of esophageal peristalsis and failure of the lower esophageal sphincter to relax in response to swallowing.[ncbi.nlm.nih.gov]
  • Author information 1 Division of Gastroenterology, Stanford University School of Medicine, Stanford, CA 94305, USA. vagt@stanford.edu Abstract Esophageal achalasia is a primary esophageal motility disorder characterized by lack of peristalsis and a lower[ncbi.nlm.nih.gov]
  • Abstract Esophageal achalasia is a primary esophageal motility disorder of unknown origin, characterized by lack of peristalsis and by incomplete or absent relaxation of the lower esophageal sphincter in response to swallowing.[ncbi.nlm.nih.gov]
Mediastinal Mass
  • An important role of echocardiography in the diagnosis of mediastinal masses is emphasized.[ncbi.nlm.nih.gov]
Right Pleural Effusion
  • Plain radiography of the chest revealed widening of the mediastinum (attributed to esophageal achalasia), pneumonia, and right pleural effusion. According to the properties of the pleural fluid, empyema was diagnosed.[ncbi.nlm.nih.gov]
Posterior Mediastinal Mass
  • Abstract We present a patient with severe decompensated esophageal achalasia in whom transthoracic echocardiography revealed compression of the left heart chambers caused by the dilated esophagus representing a posterior mediastinal mass.[ncbi.nlm.nih.gov]

Treatment

  • The optimal treatment option is an ongoing matter of debate. In this review, we provide an update of the current progress in the treatment of esophageal achalasia.[ncbi.nlm.nih.gov]
  • At a median follow-up of 22 months, 128 patients (87%) did well and required no further treatment (ES 0.1).[ncbi.nlm.nih.gov]
  • This has brought a shift in the treatment algorithm of this disease, as today a laparoscopic Heller myotomy with partial fundoplication is considered the primary form of treatment in most Centers in North America.[ncbi.nlm.nih.gov]
  • CONCLUSION: Botox is still the least costly treatment, but the minimal difference in the longer term does not justify its use, given that surgery is a risk-free, definitive treatment.[ncbi.nlm.nih.gov]
  • INTRODUCTION: Laparoscopic Heller-Dor surgery is the current treatment of choice for patients with esophageal achalasia, but elderly patients are generally referred for less invasive treatments (pneumatic dilations or botulinum toxin injections).[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis of an "achalasia-carcinoma" is generally considered poor, although systematic studies assessing the incidence, prevalence, and prognosis of patients with "achalasia-carcinoma" are scant.[ncbi.nlm.nih.gov]
  • Endoscopic ultrasound (EUS) is considered a useful adjunct for grading and establishing the prognosis of these patients.[ncbi.nlm.nih.gov]
  • Abstract Extremely well differentiated primary gastric adenocarcinoma, which accounts for less than 0.2% of all gastric cancers, is associated with a better prognosis than other types of differentiated adenocarcinoma.[ncbi.nlm.nih.gov]
  • Despite the risk of mucosal injury, adequate Heller myotomy should be achieved to obtain a good prognosis. It is, therefore, of utmost importance to obtain mastery over the surgical technique to repair any mucosal injury that might occur.[ncbi.nlm.nih.gov]
  • […] without abnormal pressure) having an intermediate prognosis (81%) that is inversely associated with the degree of esophageal dilatation.[jama.jamanetwork.com]

Etiology

  • Achalasia is a rare disease of the esophagus that has an unknown etiology. Genetic, infectious, and autoimmune mechanisms have each been proposed.[ncbi.nlm.nih.gov]
  • The etiology of achalasia is poorly understood. Achalasia occurs across the lifespan, but is uncommon in children. Most patients have progressive dysphagia for both liquids and solids.[ncbi.nlm.nih.gov]
  • Because of the rarity of both diseases, their association in the same patient is unlikely to be casual even if speculation on their common etiology is impossible at present.[ncbi.nlm.nih.gov]
  • Etiology of the denervation is unknown, but viral and autoimmune causes are suspected, and certain tumors may cause achalasia either by direct obstruction or as a paraneoplastic process.[msdmanuals.com]
  • Etiology is unknown. Some familial cases have been reported, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor.[ncbi.nlm.nih.gov]

Epidemiology

  • Epidemiological data are controversial due to different methodologies applied, the regional differences and the number of patients involved.[ncbi.nlm.nih.gov]
  • DISCUSSION: The epidemiology of achalasia in the Veneto Region is in line with the situation reported elsewhere and did not change between 2001 and 2005. Achalasia patients are mostly seen at University Hospitals.[ncbi.nlm.nih.gov]
  • We determined the general population prevalence of autoimmune diseases from published epidemiological studies. The achalasia sample was, on average, 10-15 years older and had slightly more men than the control populations.[ncbi.nlm.nih.gov]
  • Esophageal achalasia in the Veneto region: epidemiology and treatment. Epidemiology and treatment of achalasia. J Gastrointest Surg. 2011; 15 :423–428. [ PubMed ] 7. Howard PJ, Maher L, Pryde A, Cameron EW, Heading RC.[ncbi.nlm.nih.gov]
  • .— Peer-reviewed publications located via MEDLINE using the search term esophageal achalasia (subheadings: complications, drug therapy, epidemiology, etiology, physiopathology, surgery, and therapy ) published in English from 1966 to December 1997.[dx.doi.org]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiology of this phenomenon has not been fully defined. A fully documented case of coexistent esophageal achalasia and upper esophageal sphincter relaxation abnormality presenting with airway obstruction is reported.[ncbi.nlm.nih.gov]
  • From the following article Pathophysiology of achalasia and diffuse esophageal spasm Ikuo Hirano GI Motility online (2006) doi :10.1038/gimo22 BACK TO ARTICLE Barium swallow illustrating spiral or "corkscrew" deformity of the tubular esophagus caused[nature.com]
  • The optimal treatment for achalasia remains an area of controversy given our lack of complete understanding about the pathophysiology of the disease as well as the high numbers of clinical relapse after treatment.[ncbi.nlm.nih.gov]
  • .— To review the pathophysiology and management of achalasia.[dx.doi.org]

Prevention

  • Retained contents in the esophagus were evacuated just before anesthesia induction to prevent regurgitation into the trachea. The POEM procedure was performed using an orally inserted flexible fiberscope.[ncbi.nlm.nih.gov]
  • However, advances in nutritional supplementation may prevent malnutrition and allow treatment and its complications to be delayed until after delivery.[ncbi.nlm.nih.gov]
  • The benefit of magnification and three dimensional imaging helps prevent esophageal perforation and identify residual circular muscle fibers.[ncbi.nlm.nih.gov]
  • The following review describes the data present in the literature in order to identify the best procedure that can achieve prevention or control of gastroesophageal reflux after a myotomy without impairing esophageal emptying.[ncbi.nlm.nih.gov]
  • The Toupet fundoplication may be the most effective in prevention of postoperative antireflux, but many medical institutions have selected the Dor fundoplication which covers the mucosal surface exposed by myotomy.[ncbi.nlm.nih.gov]

References

Article

  1. Francis DL, Katzka DA. Achalasia: update on the disease and its treatment. Gastroenterology. 2010;139:369–7474.
  2. Eckardt AJ, Eckardt VF. Treatment and surveillance strategies in achalasia: an update. Nat Rev Gastroenterol Hepatol. 2011;8:311–319.
  3. Richter JE. The diagnosis and misdiagnosis of Achalasia: it does not have to be so difficult. Clin Gastroenterol Hepatol. 2011;9:1010–1011.
  4. Kessing BF, Bredenoord AJ, Smout AJ. Erroneous diagnosis of gastroesophageal reflux disease in achalasia. Clin Gastroenterol Hepatol. 2011;9:1020–1024.
  5. Vaezi MF, Richter JE. Diagnosis and management of achalasia. American College of Gastroenterology Practice Parameter Committee. Am J Gastroenterol. 1999;94:3406-3412
  6. Ferri LE, Cools-Lartigue J, Cao J, et al. Clinical predictors of achalasia. Dis Esophagus. 2010;23(1):76-81.
  7. de Oliveira JM, Birgisson S, Doinoff C et al. Timed barium swallow: a simple technique for evaluating esophageal emptying in patients with achalasia. AJR Am J Roentgenol 1997;169:473–479.
  8. Andersson M, Lundell L, Kostic S et al. Evaluation of the response to treatment in patients with idiopathic achalasia by the timed barium esophagogram: results from a randomized clinical trial. Dis Esophagus 2009;22:264–273.
  9. Pandolfino JE, Kwiatek MA, Nealis T et al. Achalasia: a new clinically relevant classification by high-resolution manometry. Gastroenterology 2008;135:1526–1533.
  10. Pandolfino JE, Kahrilas PJ. AGA technical review on the clinical use of esophageal manometry. Gastroenterology. 2005;128:209–224.
  11. Morera C, Nurko S. Heterogeneity of lower esophageal sphincter function in children with achalasia. J Pediatr Gastroenterol Nutr. 2012;54:34–40.
  12. Galey KM, Wilshire CL, Niebisch S et al. Atypical variants of classic achalasia are common and currently under-recognized: a study of prevalence and clinical features. J Am Coll Surg. 2011;213:155–161.
  13. Dodds WJ, Stewart ET, Kishk SM et al. Radiologic amyl nitrite test for distinguishing pseudoachalasia from idiopathic achalasia. AJR Am J Roentgenol. 1986;146:21–23.
  14. Patti MG, Arcerito M, Tong J, et al. Importance of preoperative and postoperative pH monitoring in patients with esophageal achalasia. J Gastrointest Surg. 1997 Nov-Dec; 1(6):505-10.

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Last updated: 2018-06-22 03:40