Achalasia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Twinkle Singh, M.B.B.S. [2], Ahmed Younes M.B.B.CH [3]

Achalasia is a primary esophageal motility disorder of unknown etiology.^[1][2]In this disorder, the smooth muscle layer of the esophagus has impaired peristalsis (muscular ability to move food down the esophagus), and the lower esophageal sphincter (LES) fails to relax properly in response to swallowing due to absent enteric neurons.^[3] It should be differentiated from pseudoachalasia (caused by neoplastic infiltration of myenteric neurons) and secondary achalasia (caused by extrinsic procedures such as previous fundoplication and gastric banding). Trypanosoma cruzi infection causing Chagas disease can also result in achalasia. It is an incurable chronic condition.^[1]

Achalasia is Greek for failure to relax and has been known for more than 300 years BC. The first successful esophagomyotomy was done in 1913 while laparoscopic esophagomyotomy was described in 1991.

Achalasia can be classified according to the pattern of abnormal peristalsis into three types. Different types of achalasia are shown to have different responses to therapies with type II having the best prognosis.

Achalasia is caused by degeneration of myenteric neurons, resulting from immune system activation. Evidence for antigens responsible for such immune system activation still remain inconclusive, however, viral antigens such as HSV-1, HPV, measles have been shown to play a role in achalasia pathogenesis. Genetic factors such as HLA class II alleles also predispose to achalasia development.

Achalasia is chronic esophageal motility disorder. The most common form is primary achalasia, which has no known underlying cause. It is due to the failure of distal esophageal inhibitory neurons. However, a small proportion occurs secondary to other conditions, such as esophageal cancer or Chagas disease.

Achalasia must be differentiated from other causes of dysphagia, odynophagia and food regurgitation such as GERD, esophageal adenocarcinoma, esophageal stricture, esophageal webs, motor disorders such as myasthenia gravis, stroke, Zenker’s diverticulum, diffuse esophageal spasm, systemic sclerosis and Plummer Vinson syndrome.

The incidence of Aachalasia is approximately ~ 1 per 100,000. There is no predilection to any age and has the same prevalence in both whites and non-whites.

The most potent risk factor in the development of achalasia is Allgrove syndrome. Other risk factors include herpes infection, measles infection, autoimmune diseases, and HLA type 2.

According to the USPSTF, no screening measures are recommended for achlasia.

If left untreated, the disease can progress causing complications such as candida esophagitis and esophageal perforation. However, achalasia does not alter the lifespan of the patients. Common complications include GERD, Barrett’s esophagus, and aspiration pneumonia. The prognosis is good with cure rate of 60-90% after surgical interventions.

The main symptoms of achalasia are dysphagia, regurgitation of undigested food, retrosternal chest pain and weight loss. Dysphagia involves both fluids and solids and progressively worsens over time. The chest pain experienced, also known as cardiospasm and non-cardiac chest pain can often be mistaken for a heart attack. Food and liquid, including saliva, can be retained in the esophagus and may be aspirated into the lungs. Some people may also experience coughing when lying in a horizontal position.

Physical examination is usually non significant as the diagnosis is dependent on the symptoms and the radiological tests. Patients with achalasia usually appear calm and in no acute distress. Physical examination of patients with achalasia is usually remarkable for weight loss and oral cavity ulcers.

A Laboratory work-up is usually non significant as the diagnosis is dependent on the symptoms and the radiological tests. Laboratory findings in patients with the diagnosis of achalasia may include microcytic hypochromic anemia and vitamin deficiencies.

Achalasia is caused by insufficient lower esophageal sphincter (LES) relaxation causing obstruction at gastro-esophageal junction. It leads to absent peristalsis and stasis of food in esophagus. To perform an X ray with barium swallow, the patient swallows a barium solution, which fails to pass smoothly through the lower esophageal sphincter. An air-fluid margin is seen over the barium column due to the lack of peristalsis. Narrowing is observed at the level of the gastroesophageal junction (“bird’s beak” or “rat tail” appearance of the lower esophagus). Esophageal dilation is present in varying degrees as the esophagus is gradually stretched by retained food. A five-minute timed barium swallow is useful to measure the effectiveness of treatment.

CT scan may show dilatation of the esophagus with air fluid levels in long-standing cases. CT scan may be used to exclude pseudoachalasia, or achalasia symptoms resulting from a different cause, usually esophageal cancer.

MRI can show the same findings found in CT scan such as esophageal dilation and air fluid levels. MRI can also reveal the underlying cause of achalasia such as esophageal adenocarcinoma.

Endoscopic ultrasound is required in cases where malignancy is suspected.

Esophagogastroduodenoscopy is complementary to manometry in diagnosing achalasia. It is indicated primarily to rule out any mechanical obstruction or pseudoachalasia (neoplastic iniltration).

Manometry is the key diagnostic test for achalasia. Barium esophagram and esophagogastroduodenoscopy are complimentry to manometry in diagnosing achalasia. Manometric findings such as absent peristalsis or incomplete LES relaxation without any mechanical obstruction characterize achalasia. Other supportive manometric findings in achalasia include raised basal LES pressure, increased intraoesophageal pressure and simultaneous non-propagating contractions.

Botulinum toxin, calcium channel blockers and nitrates are the most commonly used medical therapies for achalasia. However, they are not very effective and used only when pneumatic dilation and surgical procedures cannot be performed in high risk patients.

Most effective treatment options for achalasia are pneumatic dilation and laparoscopic myotomy. Pneumatic dilation works by flattening the waist of insufficiently relaxed LES by placing a balloon at LES. Laparoscopic myotomy relaxes LES by dissecting outer muscular layers of the esophagus and sparing the inner mucosal layer.

There are no primary preventive measures available for achalasia.

Many of the causes of achalasia are not preventable. However, treatment of the disorder may help to prevent complications.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Twinkle Singh, M.B.B.S. [2], Ahmed Younes M.B.B.CH [3]

Achalasia is Greek for failure to relax and has been known for more than 300 years BC. The first successful esophagomyotomy was done in 1913 while laparoscopic esophagomyotomy was described in 1991.

• Achalasia is Greek for failure to relax and has been known for more than 300 years BC.
• In 1674, Sir Thomas Willis described achalasia for the first time as functional obstruction of the esophagus at the cardiac sphincter and therefore, named it as cardiospasm.
• The first recorded successful treatment of achalasia was a sponge attached to a carved whalebone which was passed through esophagus, resulting in its dilation.
• In 1913, Ernest Heller performed the first successful esophagomyotomy to treat this disorder.
• In 1927, Sir Arthur Hurst coined the term achalasia for the first time.^[1][2]
• In 1991, Shimi et al described laproscopic approach for esophagomyotomy for the first time.^[3]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-In-Chief:Rim Halaby, Twinkle Singh, M.B.B.S. [2], Ahmed Younes M.B.B.CH [3]

Achalasia can be classified according to the pattern of abnormal peristalsis into three types. Different types of achalasia are shown to have different responses to therapies with type II having the best prognosis.

• Type I achalasia is associated with no evidence of esophageal pressurization.
• Integrated relaxation pressure (IRP)>10 mmHg distinguishes type 1 achalasia from absent peristalsis.^[1]

• Type II achalasia is associated with esophageal compression (panesophageal pressurization).
• This type of achalasia is most likely to respond to therapy.

• Type III achalasia is associated with 2 or more spastic contractions (spastic achalasia).
• IRP>17 mmHg distinguishes type III achalasia from difuse esophageal spasm.^[1]
• It carries a negative predictive response to therapy. ^[2][3]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Twinkle Singh, M.B.B.S. [2], Ahmed Younes M.B.B.CH [3]

Achalasia is caused by degeneration of myenteric neurons, resulting from immune system activation. Evidence for antigens responsible for such immune system activation still remain inconclusive, however, viral antigens such as HSV-1, HPV, measles have been shown to play a role in achalasia pathogenesis. Genetic factors such as HLA class II alleles also predispose to achalasia development.

Achalasia is a motility disorder characterized by insufficiently relaxed lower esophageal sphincter and absent peristalsis. Esophageal motility is coordinated by enteric neurons, hence their degeneration results in the above mentioned esophageal motility abnormalities. Mostly, the inhibitory neurons which cause LES relaxation by producing nitric oxide are degenerated. Relative sparing of cholinergic neurons results in increased LES tone.^[1] The cause of enteric neuron degeneration is still unknown, however, the following theories have been suggested:

• Immune ganglionitis is one of the most popular theories

  • In a study done by Goldblum et al, resected specimens of the esophagus in achalasia showed partial to complete loss of myenteric neurons. An Inflammation consisting of lymphocytes, eosinophils, plasma cells and mast cells was present in all of the cases.^[2] Clark et al found that the above mentioned lymphocytic infiltrate consisted of activated cytotoxic T cells, further strengthening the immune nature of the disease.^[3]
  • Complement activation has also been proved to be involved in the pathogenesis of achalasia.^[4]
  • Antibodies to myenteric neurons have been found in the serum of patients with achalasia.^[5] The presence of HLA class II genes such as HLA DQA1*0103 and DQB1*0603 alleles has been shown to predispose the patients to develop anti-neuronal antibodies.^[6] However, Moses et al showed that the development of these antibodies could be secondary to an injury resulting from achalasia and may not be the primary causative factor.^[7]

• Antigens responsible for the above mentioned immune response are still not known, however, viral antigens such as HSV-1, HPV and measles viruses have been suggested to be involved. These are some proofs in favor of HSV-1 antigen involvement in achalasia pathogenesis:

  • It has been shown that immune cells involved in neuronal degeneration in LES are reactive to HSV-1.^[8][9]
  • In one study, HSV-1 DNA was found in all the patients with achalasia and also in the control population without achalasia. It was then suggested that genetically predisposed individuals having a latent HSV-1 infection develop an aberrant immune response to the degenerating neurons in LES and causing achalasia.^[10]

Proposed hypothesis for achalasia development:^[11]

Initial viral infection with HSV1 or HPV

Genetic predisposition with HLA DQA1*0103 and DQB1*0603 alleles

No

Yes

No achalasia

Aberrant immune response

Achalsia development

The above algorithm was adapted from a study done by Boeckxstaens GE.^[10][11]

• Few studies found no evidence of HSV-1, HPV or measles viral infections in patients with achalasia, hence, The involvement of above mention viral antigens in the development of achalasia remain inconclusive.^[12][13]

• The following genetic factors have also been suggested to be involved with achalasia development:^[11]

  • HLA class II molecules
  • Vasoactive intestinal peptide^[14]
  • KIT^[15]
  • Interleukin 23 receptor^[16]

• Several diseases have been associated with motor abnormalities similar or identical to those of achalasia and have been called pseudoachalasia.

  • Malignancy causing achalasia by direct infiltration of the esophageal neuronal plexus.
  • Chagas disease: Trypanosoma cruzi directly infects the esophagus.
  • Amyloidosis, sarcoidosis, eosinophilic gastroenteritis, neurofibromatosis, juvenile Sjögren’s, Ogilvie’s syndrome and Anderson-Fabry’s disease have also been associated with pseudoachalasia.

• Pathological examination reveals a defect in the nerves that control the motility of the esophagus (the myenteric plexus).
• The esophagus is dilated and hypertrophied.
• In Chagas disease, the ganglion cells are destroyed by Trypanosoma cruzi, the causative parasite.^[17]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Twinkle Singh, M.B.B.S. [2], Ahmed Younes M.B.B.CH [3]

Achalasia is chronic esophageal motility disorder. The most common form is primary achalasia, which has no known underlying cause. It is due to the failure of distal esophageal inhibitory neurons. However, a small proportion occurs secondary to other conditions, such as esophageal cancer or Chagas disease.

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated. Achalasia does not have life-threatening causes.

• Idiopathic^[1]
• Chagas disease

• Gastric carcinoma^[2]
• Herpes zoster
• HSV-1
• Measles virus^[3]
• Paraneoplastic syndrome
• Sarcoidosis

Cardiovascular	No underlying causes
Chemical/Poisoning	No underlying causes
Dental	No underlying causes
Dermatologic	No underlying causes
Drug Side Effect	No underlying causes
Ear Nose Throat	No underlying causes
Endocrine	Multiple endocrine neoplasia type 2
Environmental	No underlying causes
Gastroenterologic	Eosinophilic gastroenteritis, esophageal cancer, gastric carcinoma, idiopathic intestinal pseudo-obstruction, myenteric plexus degeneration, pancreatic cancer
Genetic	No underlying causes
Hematologic	No underlying causes
Iatrogenic	No underlying causes
Infectious Disease	Chagas disease, HSV-1, herpes zoster, measles
Musculoskeletal/Orthopedic	No underlying causes
Neurologic	Neurofibromatosis
Nutritional/Metabolic	No underlying causes
Obstetric/Gynecologic	No underlying causes
Oncologic	Esophageal cancer, lung carcinoma, lymphoma, pancreatic cancer, paraneoplastic syndrome
Ophthalmologic	No underlying causes
Overdose/Toxicity	No underlying causes
Psychiatric	No underlying causes
Pulmonary	No underlying causes
Renal/Electrolyte	No underlying causes
Rheumatology/Immunology/Allergy	Sarcoidosis, Juvenile Sjögren’s syndrome
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Miscellaneous	Anderson-Fabry disease, Amyloidosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2]

Achalasia must be differentiated from other causes of dysphagia, odynophagia and food regurgitation such as GERD, esophageal adenocarcinoma, esophageal stricture, esophageal webs, motor disorders such as myasthenia gravis, stroke, Zenker’s diverticulum, diffuse esophageal spasm, systemic sclerosis and Plummer Vinson syndrome.

Achalasia must be differentiated from other causes of dysphagia, odynophagia and food regurgitation such as GERD, esophageal adenocarcinoma and esophageal stricture.

Disease	Signs and Symptoms								Barium esophagogram	Endoscopy	Other imaging and laboratory findings	Gold Standard
	Onset	Dysphagia			Weight loss	Heartburn	Other findings	Mental status
		Solids	Liquids	Type
Plummer-Vinson syndrome	Gradual	+	–	Non progressive	+/-	–	Glossitis Koilonychia	Normal	Thin projections on the anterior esophageal wall Multiple upper esophageal constrictions	Direct visualization of esophageal webs Superior to esophagogram {{#ev:youtube|HFfsTgsB6Pg}}	Videofluoroscopy shows mucosal and submucosal foldings	Triad of Iron deficiency anemia Esophageal webs Glossitis
Esophageal stricture	Gradual Sudden onset	+	–	Progressive	+/-	+/-	Odynophagia Cough Chest pain	Normal	Sacculations Fixed transverse folds Esophageal intramural pseudodiverticula	Mucosal edema Circumferential thickening in GERD Pale mucosa with white exudate in lymphocytic esophagitis Swelling and hemorrhagic congestion in caustic ingestion {{#ev:youtube|vax5E-jMnQ}}	Manometry may show dysmotility CT scan for staging malignant strictures	Barium esophagogram
Diffuse esophageal spasm	Sudden	+	+	Non progressive	+	+	Chest pain	Normal	Nonperistaltic and nonpropulsive contractions Corkscrew or rosary bead esophagus	Inconclusive {{#ev:youtube|2ipA34iMA3c}}	Manometry shows high-amplitude esophageal contractions CT scan may show hypertrophy of esophageal muscles	Manometry
Achalasia	Gradual	+	+	Non progressive	+/-	–	Regurgitation of undigested food Chest pain	Normal	“Bird’s beak” or “rat tail” appearance Dilated esophageal body Air fluid level (absent peristalsis) Absence of an intragastric air bubble	Dilated esophagus Residual food fragments Normal mucosa {{#ev:youtube|ydLcskQzEjM}}	Residual pressure of LES > 10 mmHg Incomplete relaxation of the LES Increased resting tone of LES Aperistalsis	History of dysphagia with positive endoscopy and manometry
Systemic sclerosis	Gradual	+	+	Progressive	+/-	+	Muscle and joint pain Raynaud’s phenomenon Skin changes	Normal	Dysmotility Patulous esophagus	Mucosal damage Peptic stricture (advanced cases)	Positive serology for Antinuclear antibodies Rheumatoid factor Creatine kinase ESR	Skin biopsy
Zenker’s diverticulum	Gradual	+	–		+/-	–	Food regurgitation Halitosis Cough Hoarseness	Normal	Thin projections on esophageal wall over Killian’s triangle	Outpouching of posterior pharyngeal wall Exclude the presence of SCC {{#ev:youtube|FdEruFsNdVA}}	CT & MRI shows out-pouching over the posterior esophagus in the Killian’s triangle	Barium esophagography
Esophageal carcinoma	Gradual	+	+	Progressive	+	+/-	Lymphadenopathy Cachexia	Normal	Irregular stricture Pre-stricture dilatation	Esophageal obstruction Staging of disease {{#ev:youtube|5ucSlgqGAno}}	CT and PET scan is an optional test for staging of the disease	Biopsy
Stroke (Cerebral hemorrhage)	Sudden	+	+	Progressive	+	+/-	Dysarthria Limb weakness Fatigue	Impaired	Pooling of contrast in the pharynx Aspiration of barium contrast into the airway	Reduced opening of upper esophageal sphincter Reduced larynx elevation	CT without contrast shows acute hemorrhage as a hyperattenuating clot	CT without contrast
Motor disorders (Myasthenia gravis)	Gradual	+	+	Progressive	+/-		Ptosis Diplopia Fatigue	Normal	Stasis in pharynx and pooling in pharyngeal recesses	Velopharyngeal insufficiency Delayed swallowing function	CT may show anterior mediastinal mass (thymoma) Positive tensilon test	Anti–acetylcholine receptor antibody test
GERD	Gradual Sudden onset	+	–	Progressive	+/-	+	Cough Hoarseness	Normal	Free acid reflux Esophagitis with scarring Strictures Barrett’s oesophagus	Erythema, erosions and ulceration Barrett’s esophagus	Esophageal manometry may show decreased tone of LES	24 hour esophageal pH monitoring
Esophageal web	Gradual	+	+/-	Progressive	–	+/-	Findings of the underlying cause such as iron deficiency anemia or bullous pemphigoid	Normal	Symmetrical narrowing of the esophagus	Smooth membrane not encircling the whole lumen	Videofluoroscopy shows mucosal and submucosal foldings	Barium esophagogram

	Manifestations	Diagnostic tools
Achalasia	Dyspnea[1] Dysphagia for solids and liquids is the most common feature, being seen in 91 % and 85% of patients respectively[2] Regurgitation of undigested food occurs in 76-91% of patients[2] Cough mainly when lying down in 30%[2]	Esophagogastroduodenoscopy findings include a dilated esophagus with residual food fragments, normal mucosa and occasionally candidiasis (due to the prolonged stasis). Barium swallow shows the characteristic bird’s beak appearance.
GERD	Retrosternal burning chest pain. Cough and hoarseness of voice. May present with complications such as strictures and dysphagia.[3]	Upper GI endoscopy shows the complications such as esophagitis and Barrett’s esophagus. Esophageal manometry may show decreased tone of the lower esophageal sphincter. 24-hour esophageal pH monitoring may be done to confirm the diagnosis.
Esophageal carcinoma	Dysphagia Odynophagia– fluids and soft foods are usually tolerated, while hard or bulky substances (such as bread or meat) cause much more difficulty[4] Weight loss Pain, often of a burning nature, may be severe and worsened by swallowing, and can be spasmodic in character Nausea and vomiting[4]	Upper GI endoscopy and esophageal biopsy the gold standard for the diagnosis of esophageal
Corckscrew esophagus	Retrosternal chest pain that presents with or without food intake.[5] The condition is not progressive and not causing complications.[6]	Barium swallow shows the characteristic corckscrew appearance of the esophagus.
Esophageal stricture	Patient may present with the symptoms of the underlying GERD. Dysphagia and odynophagia.[7]	Barium esophagography provides information about the site and the diameter of the stricture before the endoscopic intervention.[8]
Plummer-Vinson syndrome	Common symptoms of Plummer-Vinson syndrome include[9][10][11] Difficulty swallowing (more for solids) Weakness Pain Burning sensation in mouth Dry tongue Painful cracks in the angles of a dry mouth Pale color of the skin Less cmmon symptoms Cold intolerance Reduced resistance to infection Altered behavior Craving for for unusual items (such as ice or cold vegetables)	Lab tests are consistent with the diagnosis of iron deficiency anemia. Findings on an x-ray (barium esophagogram) suggestive of esophageal web/strictures associated with Plummer-Vinson syndrome appear as either: Thin projections on the anterior esophageal wall. Multiple upper (cervical) esophageal constrictions consistent with esophageal webs.

References

1. ↑ Ferri, Fred (2015). Ferri’s clinical advisor 2015 : 5 books in 1. Philadelphia, PA: Elsevier/Mosby. ISBN 978-0323083751.
2. ↑ ^{2.0 2.1 2.2} Boeckxstaens GE, Zaninotto G, Richter JE (2013). “Achalasia”. Lancet. doi:10.1016/S0140-6736(13)60651-0. PMID 23871090.CS1 maint: Multiple names: authors list (link)
3. ↑ Badillo R, Francis D (2014). “Diagnosis and treatment of gastroesophageal reflux disease”. World J Gastrointest Pharmacol Ther. 5 (3): 105–12. doi:10.4292/wjgpt.v5.i3.105. PMC 4133436. PMID 25133039.
4. ↑ ^{4.0 4.1} Napier KJ, Scheerer M, Misra S (2014). “Esophageal cancer: A Review of epidemiology, pathogenesis, staging workup and treatment modalities”. World J Gastrointest Oncol. 6 (5): 112–20. doi:10.4251/wjgo.v6.i5.112. PMC 4021327. PMID 24834141.
5. ↑ Matsuura H (2017). “Diffuse Esophageal Spasm: Corkscrew Esophagus”. Am. J. Med. doi:10.1016/j.amjmed.2017.08.041. PMID 28943381.
6. ↑ Lassen JF, Jensen TM (1992). “[Corkscrew esophagus]”. Ugeskr. Laeg. (in Danish). 154 (5): 277–80. PMID 1736462.CS1 maint: Unrecognized language (link)
7. ↑ Ruigómez A, García Rodríguez LA, Wallander MA, Johansson S, Eklund S (2006). “Esophageal stricture: incidence, treatment patterns, and recurrence rate”. Am. J. Gastroenterol. 101 (12): 2685–92. doi:10.1111/j.1572-0241.2006.00828.x. PMID 17227515.
8. ↑ Shami VM (2014). “Endoscopic management of esophageal strictures”. Gastroenterol Hepatol (N Y). 10 (6): 389–91. PMC 4080876. PMID 25013392.
9. ↑ López Rodríguez MJ, Robledo Andrés P, Amarilla Jiménez A, Roncero Maíllo M, López Lafuente A, Arroyo Carrera I (2002). “Sideropenic dysphagia in an adolescent”. J. Pediatr. Gastroenterol. Nutr. 34 (1): 87–90. PMID 11753173.
10. ↑ Chisholm M (1974). “The association between webs, iron and post-cricoid carcinoma”. Postgrad Med J. 50 (582): 215–9. PMC 2495558. PMID 4449772.
11. ↑ Larsson LG, Sandström A, Westling P (1975). “Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden”. Cancer Res. 35 (11 Pt. 2): 3308–16. PMID 1192404.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2]

The incidence of Achalasia is approximately ~ 1 per 100,000. There is no predilection to any age and has the same prevalence in both whites and non-whites.

• Achalasia is a rare disease, with an incidence of ~ 1 case per 100,000 per year in adults and a prevalence of 8-10 cases per 100,000.^[1][2][3]

• Most commonly, achalasia is diagnosed between 25 and 60 years old, mean age of diagnosis being > 50 years.^[4]

• Achalasia has no predilection to any age.^[4][3]

• Achalasia has the same prevalence in both whites and non-whites.^[5]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Assistant Editor-In-Chief:Rim Halaby, Ahmed Younes M.B.B.CH [2]

The most potent risk factor in the development of achalasia is Allgrove syndrome. Other risk factors include herpes infection, measles infection, autoimmune diseases, and HLA type 2.

• Allgrove syndrome, also known as triple-A syndrome (AAA), or as Achalasia-Addisonianism-Alacrimia syndrome^[1][2][3][4]
• Herpes infection^[1]
• Measles infection^[2]
• Autoimmune diseases^[3]
• HLA type 2^[4]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: , Ahmed Younes M.B.B.CH [2]

According to the USPSTF, no screening measures are recommended for achlasia.

According to the USPSTF, no screening measures are recommended for achlasia.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1], Associate Editor(s)-in-Chief: Ahmed Younes M.B.B.CH [2]

If left untreated, the disease can progress causing complications such as candida esophagitis and esophageal perforation. However, achalasia does not alter the lifespan of the patients. Common complications include GERD, Barrett’s esophagus, and aspiration pneumonia. The prognosis is good with cure rate of 60-90% after surgical interventions.

• The disease has a slow onset of symptoms, therefore the condition is usually advanced at the time of presentation.^[1]
• If left untreated, the disease can progress causing complications such as candida esophagitis, esophageal perforation and aspiration pneumonia. However, achalasia does not alter the lifespan of the patients.^[1]

Achalasia may be complicated by

• Gastroesophageal reflux disease (GERD) or heartburn.
• Achalasia patients have an increased risk of developing Barrett’s esophagus or Barrett’s mucosa, a premalignant condition which may lead to esophageal cancer over a period of years.^[2]
• Aspiration pneumonia: Food and liquid, including saliva, are retained in the esophagus and may be inhaled into the lungs, especially while sleeping in a horizontal position.
• Tearing (perforation) of the esophagus^[3]
• The incidence of esophageal cancer is controversial in patients with achalasia. Some Swedish studies report an increased incidence and suggest routine surveillance esophagogastroduodenoscopy (EGD). This has not been shown to be the case in the U.S.A., and current recommendations do not include routine EGD.^[4]

• With treatment, the outcome for achalasia is usually good.
• The cure rate ranges from 60 to 90% after surgical interventions.^[5]

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