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

  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]

  It is important to contact a physician if you have difficulty swallowing, keeping food down or if you are experiencing unintended weight loss. [uofmhealth.org]

  She's had multiple episodes of pneumonia and has experienced an unintentional weight loss of 13.6 kg (30 lb) over the last 22 months. Mrs. [journals.lww.com]

• Cough

  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]

• Hoarseness

  Symptoms of achalasia include difficulty swallowing (dysphagia), regurgitation of undigested food, chest pain that may be perceived as heartburn, and occasionally respiratory complications such as hoarseness or pneumonia from aspirating retained food [columbiasurgery.org]

  Indigestion – stomach bloating, excessive belching Throat irritation Projectile vomiting due to obstruction in the lower part of the esophagus, stomach or duodenum Chronic reflux may lead to a host of symptoms in the upper airway, including coughing, hoarse [healthhype.com]

  Some people with GERD have only a sore throat, chronic cough, or hoarseness. Others have no symptoms at all. Plus and Minus Icon Several lifestyle and dietary habits can make gastroesophageal reflux disease continue or worsen. [mskcc.org]

  This relaxation allows the esophagus to empty contents into the stomach. 12 Without LES relaxation, food, fluids, and air remain trapped in the esophagus, contributing to dysphagia, regurgitation, aspiration, cough, chest pain, hoarseness, and over time [journals.lww.com]

• Pharyngitis

  The esophageal stage of swallowing is the last part and follows the pharyngeal stage which is preceded by the voluntary stage. [healthhype.com]

  Jones B, Donner MW, Rubesin SE, Ravich WJ, Hendrix TR: Pharyngeal findings in 21 patients with achalasia of the esophagus. Dysphagia 2 :87–92, 1987 Google Scholar 8. Carlsson-Nordlander S: Acute upper airway obstruction in a patient with achalasia. [link.springer.com]

  Pharyngoesophageal high-resolution manometry This is a quantitative evaluation of the pressure and timing of pharyngeal contraction and upper esophageal relaxation. [worldgastroenterology.org]

  View Article PubMed Google Scholar Inoue H: Endoscopic diagnosis of tissue atypism (EA) in the pharyngeal and esophageal squamous epithelium; IPCL pattern classification and ECA classification]. Kyobu Geka. 2007, 60 (8 Suppl): 768-775. [bmcgastroenterol.biomedcentral.com]

• 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]

  Oesophageal hypercontraction NUTCRACKER OESOPHAGUS “Nutcracker oesophagus” is a term coined by Castell and colleagues for the condition in which patients with non-cardiac chest pain and/or dysphagia exhibit peristaltic waves in the distal oesophagus with [dx.doi.org]

• Throat Irritation

  irritation Projectile vomiting due to obstruction in the lower part of the esophagus, stomach or duodenum Chronic reflux may lead to a host of symptoms in the upper airway, including coughing, hoarse voice and a lump in the throat feeling. [healthhype.com]

• Dysphagia

  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]

  Nevertheless, since one of the most important postoperative outcomes is dysphagia resolution, a longer myotomy may be more appropriate. [sages.org]

• Dysphagia

  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]

  Nevertheless, since one of the most important postoperative outcomes is dysphagia resolution, a longer myotomy may be more appropriate. [sages.org]

• Regurgitation

  O's pneumonia has been related to regurgitation of retained food and fluids, and her weight loss is related to dysphagia and regurgitation of both solid food and liquids secondary to esophageal achalasia. 1 She can no longer eat or drink without extreme [journals.lww.com]

  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]

  There may be regurgitation of food, inhalation of swallowed material, with pneumonia and severe loss of weight. The condition is sometimes complicated by cancer of the oesophagus. Also known as cardiospasm. [medical-dictionary.thefreedictionary.com]

  Achalasia can dramatically affect a person’s quality of life by making it difficult to eat, disrupting sleep due to regurgitation, and may cause substantial weight loss. [uofmhealth.org]

• Choking

  As the esophagus becomes wider and weaker, symptoms can include: Difficulty swallowing food, a condition called dysphagia Food or liquid flowing back up into the throat, or regurgitation Waking up at night from coughing or choking because of regurgitation [choc.org]

  Achalasia causes symptoms of coughing, choking, regurgitation, dysphagia and food retention in the esophagus. [hopkinsmedicine.org]

  Symptoms of achalasia include: Difficulty swallowing Pressure in the chest after eating Backing up of undigested food and fluid (regurgitation) sometimes hours after eating Coughing or choking on food Bad breath The symptoms can worsen with certain foods [ctsurgerypatients.org]

• Regurgitation of Undigested Food

  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]

  Patients gradually develop dysphagia for both solids and liquids, and about one third regurgitate undigested food at night. [merckmanuals.com]

  Symptoms of achalasia include difficulty swallowing (dysphagia), regurgitation of undigested food, chest pain that may be perceived as heartburn, and occasionally respiratory complications such as hoarseness or pneumonia from aspirating retained food [columbiasurgery.org]

• Halitosis

  Halitosis is a very nonspecific sign that may suggest advanced achalasia or longterm obstruction, with accumulation of slowly decomposing residues in the esophageal lumen. [worldgastroenterology.org]

• 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]

  Clinical findings Dysphagia, chest pain, vomiting, heartburn. Imaging Plain films, air-fluid level in enlarged oesophagus, barium swallow, bird’s beak tapering and, if prolonged, sigmoid oesophagus. DiffDx Chaga’s disease, T-cruzi amastigotes. [medical-dictionary.thefreedictionary.com]

  pain, allergic reactions, and rarely, mediastinitis. [journals.lww.com]

  In addition, they may have severe chest pain due to the elevated pressure in the esophageal body. The evaluation and treatment of these patients is the same as patients with achalasia. [memorialhermann.org]

• 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]

  Subsequent weight loss is common, as is retrosternal chest pain, reflux, and regurgitation of undigested food. 1 Contrast esophagram, endoscopy, and esophageal manometry are the tests of choice for diagnosing achalasia. [laparoscopy.blogs.com]

• Petechiae

  […] lax, elongated, tortuous − Uncoordinated, nonpropulsive, or absent contractions − Diverticulumlike pouch above the LES − Increased resistance to cardial intubation − Mucosal changes due to food retention: padlike thickening of the mucosa, erythema, petechiae [gastrointestinalatlas.com]

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

  Other less common dysmotility disorders include spastic esophageal motility disorder (nutcracker esophagus and diffuse esophageal spasm), inefficient esophageal motility disorder, and secondary esophageal motility disorders, which are related to other [ctsurgerypatients.org]

  Achalasia is a rare esophageal motility disorder that is usually idiopathic in origin. [ncbi.nlm.nih.gov]

• Multiple Ulcerations

  Lower Third of the Esophagus, there are multiple ulcerations and a small pseudodiverticula. Incompetent lower esophageal sphincter is evident. Video Endoscopic Sequence 22 of 31. More images and video clips of the lower third of the esophagus. [gastrointestinalatlas.com]

Non-surgical treatments include: Botox injections Balloon Dilation Surgical Treatment The most effective and durable achalasia treatment is obtained by a myotomy: cutting the muscle of the lower esophageal sphincter (the valve between the esophagus and [memorialhermann.org]

Clinicians usually use a low-compliance balloon such as Rigiflex dilator to perform endoscope-guided PD for the treatment of esophageal achalasia. [ncbi.nlm.nih.gov]

Intraoperative Esophageal Perforation Rates of Patients with and without Prior Treatment Type of Endoscopic Treatment Perforations Reference Prior Endoscopic Treatment No Prior Endoscopic Treatment Reference Prior Endoscopic Treatment No Prior Endoscopic [sages.org]

Endoscopic ultrasound (EUS) is considered a useful adjunct for grading and establishing the prognosis of these patients. [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]

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]

Although the precise etiology is unknown, it is often thought to be either autoimmune, viral immune, or neurodegenerative. The diagnosis is based on history of the disease, barium esophagogram, and esophageal motility testing. [regione.lazio.it]

Epidemiological data are controversial due to different methodologies applied, the regional differences and the number of patients involved. [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. [jama.ama-assn.org]

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]

— To review the pathophysiology and management of achalasia. [jama.ama-assn.org]

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]

Achalasia Treatment The goal of therapy is to obtain relief of difficulty swallowing by opening the lower esophageal sphincter (the valve between the esophagus and the stomach) while preventing GERD. [memorialhermann.org]

Endoscopic injection of the esophageal muscle with botulinum toxin (called “Botox”), can paralyze the lower esophageal sphincter and prevent spasms. The relief, however, is only temporary, and symptoms return quickly in most patients. [surgery4children.com]

To prevent reflux after the procedure, surgeons typically perform a partial wrap of the stomach around the esophagus. [nyp.org]

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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.