Achalasia 189093 224936450 2008-07-11T02:18:07Z Jfdwolff 46555 rm reference to isolated case {{Infobox_Disease | Name = Achalasia | Image = | Caption = | DiseasesDB = 72 | ICD10 = {{ICD10|K|22|0|k|20}} | ICD9 = {{ICD9|530.0}} | ICDO = | OMIM = 200400 | MedlinePlus = 000267 | eMedicineSubj = radio | eMedicineTopic = 6 | eMedicine_mult = {{eMedicine2|med|16}} | MeshName = Esophageal+Achalasia | MeshNumber = C06.405.117.119.500.432 | }} '''Achalasia''', also known as '''esophageal achalasia''', '''achalasia cardiae''', '''cardiospasm''', '''dyssynergia esophagus''', and '''esophageal aperistalsis''', is an [[esophageal motility disorder]]: The [[smooth muscle cell|smooth muscle]] layer of the [[esophagus]] loses normal [[peristalsis]] (muscular ability to move food down the esophagus), and the [[lower esophageal sphincter]] (LES) fails to relax properly in response to [[swallowing]].<ref name=Park_2005>{{cite journal |author=Park W, Vaezi M |title=Etiology and pathogenesis of achalasia: the current understanding |journal=Am J Gastroenterol |volume=100 |issue=6 |pages=1404–14 |year=2005 |pmid=15929777 |doi=10.1111/j.1572-0241.2005.41775.x}}</ref> Achalasia is characterized by [[dysphagia|difficulty swallowing]], [[regurgitation (digestion)|regurgitation]], and sometimes [[chest pain]]. Diagnosis is reached with [[esophageal manometry]] and [[barium swallow]] X-ray studies. Various treatments are available, but none cure the condition completely. Certain medications or [[botulinum toxin|botox]] may be used in some cases, but more permanent relief is brought by [[esophageal dilatation]] and surgical cleaving of the muscle ([[Heller myotomy]]). The most common form is primary achalasia, which has no known underlying cause. However, a small proportion occurs as a secondary result of other conditions, such as [[esophageal cancer]] or [[Chagas disease]] (an infectious disease common in South America).<ref name=Spiess/> Achalasia affects about one person in 100,000 per year.<ref name=Lake/><ref name=Spiess/> ==Signs and symptoms== *[[Dysphagia]] (difficulty in swallowing) which becomes worse over time, generally involving both liquids and solids. *[[Regurgitation (digestion)|Regurgitation]] of undigested food. *[[Cough]]ing, especially when reclining or lying down. This is caused by food remaining in the esophagus, and can lead to [[pulmonary aspiration|aspiration]] of food matter into the [[lung]]s.<ref name=Spiess/> *Some patients experience [[chest pain]]s resembling [[heartburn]] or pressure on the sternum. *Most patients have [[weight loss]] due to inadequate nutrient intake. ==Diagnosis== Due to the similarity of symptoms, achalasia can be mistaken for more common disorders such as [[gastroesophageal reflux disease]] (GERD), [[hiatus hernia]], and even [[psychosomatic]] disorders. Specific tests for achalasia are [[barium swallow]] and [[esophageal manometry]]. A [[Computed tomography|CT scan]] of the chest and [[endoscopy]] of the esophagus, stomach and duodenum ([[esophagogastroduodenoscopy]] or EGD), with or without [[endoscopic ultrasound]], are typically performed to rule out the possibility of cancer.<ref name=Spiess/> The internal tissue of the esophagus generally appears normal in [[endoscopy]], although a "pop" may be observed as the scope is passed through the non-relaxing [[lower esophageal sphincter]] with some difficulty. ===Barium swallow=== The patient swallows a barium solution, with continuous [[fluoroscopy]] (X-ray recording) to observe the flow of the fluid through the esophagus. Normal peristaltic movement of the esophagus is not seen. There is acute tapering at the [[lower esophageal sphincter]] and narrowing at the [[cardia|gastro-esophageal junction]], producing a "bird's beak" or "rat's tail" appearance. The esophagus above the narrowing is often dilated (enlarged) to varying degrees as the esophagus is gradually stretched, and it may contain food debris.<ref name=Spiess/> An air-fluid margin is often seen over the barium column due to the lack of peristalsis. A five-minute timed barium swallow can provide a useful benchmark to measure the effectiveness of treatment. ===Esophageal manometry=== [[Image:Achalasia EMS.jpg|thumb|right|Schematic of manometry in achalasia showing [[peristalsis|aperistaltic]] contractions, increased intraesophageal pressure and failure of relaxation of the lower esophageal sphincter.]] Because of its sensitivity, manometry (esophageal motility study) is considered the key test for establishing the diagnosis. A thin tube is inserted through the nose, and the patient is instructed to swallow several times. The probe measures muscle contractions in different parts of the esophagus during the act of swallowing. ===Biopsy=== [[Biopsy]], the removal of a tissue sample during endoscopy, is not typically necessary in achalasia, but if performed shows [[hypertrophy|hypertrophied]] musculature and absence of certain nerve cells of the [[myenteric plexus]], a network of nerve fibers that controls esophageal peristalsis.<ref name=pathology>{{cite book | author= Emanuel Rubin, Fred Gorstein, Raphael Rubin, Roland Schwarting, David Strayer | title = Rubin's Pathology - clinicopathological foundations of medicine | publisher = Lippincott Williams & Wilkins | date = 2001 | location = Maryland | pages=page 665 | isbn=0-7817-4733-3 }}</ref> ==Treatment== ===Medication=== Drugs that reduce LES pressure may be useful, especially as a way to buy time while waiting for surgical treatment. These include [[calcium channel blockers]] such as [[nifedipine]], and [[nitrates]] such as [[isosorbide dinitrate]] and [[nitroglycerin]]. Unfortunately, many patients experience unpleasant side effects such as [[headache]] and swollen feet, and these drugs often stop helping after several months. [[Botulinum toxin]] (botox) may be injected into the lower esophageal sphincter to paralyze the muscles holding it shut. As in the case of cosmetic botox, the effect is only temporary, and symptoms return relatively quickly in most patients. Botox injections cause scarring in the sphincter which may increase the difficulty of later [[Heller myotomy]]. This therapy is only recommended for patients who cannot risk surgery, such as elderly persons in poor health.<ref name=Spiess>{{cite journal |author=Spiess AE, Kahrilas PJ |title=Treating achalasia: from whalebone to laparoscope |journal=JAMA |volume=280 |issue=7 |pages=638–42 |year=1998 |month=August |pmid=9718057 | url=http://jama.ama-assn.org/cgi/reprint/280/7/638 |doi=10.1001/jama.280.7.638}}</ref> ===Pneumatic dilatation=== *[[Esophageal dilatation|Balloon (pneumatic) dilation]], also called dilatation. The muscle fibers are stretched and slightly torn by forceful inflation of a balloon placed inside the lower esophageal sphincter. Gastroenterologists who specialize in achalasia and have performed many of these forceful balloon dilations achieve better results and fewer perforations. There is always a small risk of a perforation which would have to be fixed by surgery right away. [[Gastroesophageal reflux disease|Gastroesophageal reflux (GERD)]] occurs after pneumatic dilation in some patients. This treatment causes some scarring which may increase the difficulty of [[Heller myotomy]] if this surgery is needed later. Pneumatic dilation is most effective on the long term in patients over the age of 40; the benefits tend to be shorter-lived in younger patients. It may need to be repeated with larger balloons for maximum effectiveness.<ref name=Lake>{{cite journal |author=Lake JM, Wong RK |title=Review article: the management of achalasia - a comparison of different treatment modalities |journal=Aliment. Pharmacol. Ther. |volume=24 |issue=6 |pages=909–18 |year=2006 |month=September |pmid=16948803 |doi=10.1111/j.1365-2036.2006.03079.x |url=http://www.blackwell-synergy.com/doi/full/10.1111/j.1365-2036.2006.03079.x}}</ref> ===Surgery=== [[Heller myotomy]] helps 90% of achalasia patients. It can usually be performed by a [[Laparoscopic surgery|keyhole approach]], or laparoscopically.<ref name=Deb_2005>{{cite journal |author=Deb S, Deschamps C, Cassivi SD, et al. |title=Laparoscopic esophageal myotomy for achalasia: factors affecting functional results |journal=Annals of Thoracic Surgery |volume=80 |issue=4 |pages=1191–1195 |year=2005 |pmid=16181839 |doi=10.1016/j.athoracsur.2005.04.008}}</ref> The myotomy is a lengthwise cut along the esophagus, starting above the LES and extending down onto the stomach a little way. The esophagus is made of several layers, and the myotomy only cuts through the outside muscle layers which are squeezing it shut, leaving the inner muscosal layer intact. A partial [[Nissen fundoplication|fundoplication]] or "wrap" is added in order to prevent excessive [[Gastroesophageal reflux disease|reflux]], which can cause serious damage to the esophagus over time. After surgery, patients should keep to a [[soft diet]] for several weeks to a month, avoiding foods that can aggravate reflux. ===Follow-up=== Follow-up monitoring: Even after successful treatment of achalasia, swallowing may still deteriorate over time. It is important to check every year or two with a timed barium swallow because some may need pneumatic dilations, a repeat myotomy, or even [[esophagectomy]] after many years. Some physicians recommend pH testing and endoscopy to check for reflux damage, which may lead to a stricture or cancer of the esophagus if untreated. ==See also== * [[Heller myotomy]] ==References== <!-- --------------------------------------------------------------- See http://en.wikipedia.org/wiki/Wikipedia:Footnotes for a discussion of different citation methods and how to generate footnotes using the <ref> & </ref> tags and the {{Reflist}} template -------------------------------------------------------------------- --> {{Reflist|2}} ==External links== * {{dmoz|Health/Conditions and Diseases/Digestive Disorders/Esophagus}} * [http://www.guideline.gov/summary/summary.aspx?doc_id=5504&nbr=003747 U.S. Society for Surgery of the Alimentary Tract] - guideline about achalasia] * [http://www.patient.co.uk/showdoc/40000974 Patient.co.uk] - page on achalasia {{Gastroenterology}} [[Category:Gastroenterology]] [[de:Achalasie]] [[es:Acalasia]] [[fr:Achalasie]] [[it:Acalasia]] [[ms:Akalasia]] [[nl:Achalasie]] [[ja:アカラシア]] [[pl:Achalazja przełyku]] [[pt:Acalasia]] [[ru:Ахалазия кардии]] [[sv:Achalasia cardiae]] [[tl:Achalasia]] [[ur:فقدِ لیانی]]