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08-SEPTEMBER-2008 07:42:01 - Achalasia Achalasia Classification and external resources ICD-10 K22.0 ICD-9 530.0 OMIM 200400 DiseasesDB 72 MedlinePlus 000267 eMedicine radio/6 med/16 MeSH 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 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.1 Achalasia is characterized by difficulty swallowing, regurgitation, and sometimes chest pain. Diagnosis is reached with esophageal manometry and barium swallow X-ray studies. Various treatments are available, although none cure the condition completely. Certain medications or 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.2 Achalasia affects about one person in 100,000 per year.32 Contents 1 Signs and symptoms 2 Diagnosis 2.1 Barium swallow 2.2 Esophageal manometry 2.3 Biopsy 3 Treatment 3.1 Medication 3.2 Pneumatic dilatation 3.3 Surgery 3.4 Alternative medicine 3.5 Lifestyle changes 3.6 Follow-up 4 See also 5 References 6 External links Signs and symptoms Dysphagia difficulty in swallowing which becomes worse over time, generally involving both liquids and solids. Regurgitation of undigested food. Coughing, especially when lying in a horizontal position. Food and liquid, including saliva, are retained in the esophagus and may be inhaled into the lungs aspiration,2 potentially leading to aspiration pneumonia. Some patients experience chest pains resembling heartburn or pressure on the sternum. Most patients, but not all, 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. Bird's beak appearance, typical in achalasia. Bird's beak appearance, typical in achalasia. Specific tests for achalasia are barium swallow and esophageal manometry. In addition, a 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.2 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 Schematic of manometry in achalasia showing aperistaltic contractions, increased intraesophageal pressure and failure of relaxation of the lower esophageal sphincter. Schematic of manometry in achalasia showing aperistaltic contractions, increased intraesophageal pressure and failure of relaxation of the lower esophageal sphincter. 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 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.2 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 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 hypertrophied musculature and absence of certain nerve cells of the myenteric plexus, a network of nerve fibers that controls esophageal peristalsis.4 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. However, 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.2 Pneumatic dilatation In 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 must be fixed by surgery right away. This treatment causes some scarring which may increase the difficulty of Heller myotomy if this surgery is needed later. Gastroesophageal reflux GERD occurs after pneumatic dilation in some patients. 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.3 Surgery Heller myotomy helps 90% of achalasia patients. It can usually be performed by a keyhole approach, or laparoscopically.5 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 fundoplication or wrap is generally added in order to prevent excessive 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. Alternative medicine Temporary improvement of achalasia symptoms in some cases has been reported with acupuncture, traditional Chinese herbal medicine, and relaxation techniques. Lifestyle changes Both before and after successful treatment, achalasia patients may need to eat slowly, chew very well, drink plenty of water with meals, and avoid eating near bedtime. Raising the head of the bed or sleeping with a wedge pillow promotes emptying of the esophagus by gravity. After surgery or pneumatic dilatation, proton pump inhibitors can help prevent reflux damage by inhibiting gastric acid secretion; and foods that can aggravate reflux, including ketchup, citrus, chocolate, mint, alcohol, and caffeine, may need to be avoided. Follow-up Follow-up monitoring: Even after successful treatment of achalasia, swallowing may still deteriorate over time. The esophagus should be checked every year or two with a timed barium swallow because some may need pneumatic dilations, a repeat myotomy, or even esophagectomy after many years. In addition, some physicians recommend pH testing and endoscopy to check for reflux damage, which may lead to a premalignant condition known as Barrett's esophagus or a stricture if untreated. See also Heller myotomy References ^ Park W, Vaezi M 2005. Etiology and pathogenesis of achalasia: the current understanding. Am J Gastroenterol 100 6: 1404-14. doi:10.1111/j.1572-0241.2005.41775.x. PMID 15929777. ^ a b c d e f Spiess AE, Kahrilas PJ August 1998. Treating achalasia: from whalebone to laparoscope. JAMA 280 7: 638-42. doi:10.1001/jama.280.7.638. PMID 9718057. ^ a b Lake JM, Wong RK September 2006. Review article: the management of achalasia - a comparison of different treatment modalities. Aliment. Pharmacol. Ther. 24 6: 909-18. doi:10.1111/j.1365-2036.2006.03079.x. PMID 16948803. ^ Emanuel Rubin, Fred Gorstein, Raphael Rubin, Roland Schwarting, David Strayer 2001. Rubin's Pathology - clinicopathological foundations of medicine. Maryland: Lippincott Williams Wilkins, page 665. ISBN 0-7817-4733-3. ^ Deb S, Deschamps C, Cassivi SD, et al. 2005. Laparoscopic esophageal myotomy for achalasia: factors affecting functional results. Annals of Thoracic Surgery 80 4: 1191-1195. doi:10.1016/j.athoracsur.2005.04.008. PMID 16181839. External links and Diseases/Digestive Disorders/Esophagus/ Achalasia at the Open Directory Project U.S. Society for Surgery of the Alimentary Tract - Achalasia treatment guidelines. Patient.co.uk - Achalasia patient information. Achalasia Mailing List A mailing list where achalasia patients share experiences, advice, questions, and support. achalasia.us Resources for achalasia patients. v d e Digestive system - Digestive disease - Gastroenterology primarily K20-K93, 530-579 Upper GI tract Esophagus Esophagitis Candidal - Boerhaave syndrome - UES Zenker's diverticulum - LES Barrett's esophagus, Mallory-Weiss syndrome - Esophageal motility disorder Nutcracker esophagus, Achalasia, Diffuse esophageal spasm, GERD - Esophageal stricture - Megaesophagus Stomach Gastritis Atrophic, Ménétrier's disease, Gastroenteritis - Peptic gastric ulcer/Dieulafoy's lesion - Dyspepsia - Pyloric stenosis - Achlorhydria - Gastroparesis - Gastroptosis - Portal hypertensive gastropathy - Gastric antral vascular ectasia - Gastric dumping syndrome - Gastric volvulus Intestinal/ enteropathy Small intestine/ duodenum/jejunum/ileum Enteritis Duodenitis, Jejunitis, Ileitis Peptic duodenal ulcer Malabsorption: Coeliac - Tropical sprue - Blind loop syndrome - Whipple's - Short bowel syndrome - Steatorrhea Large intestine appendix/colon Appendicitis - Colitis Pseudomembranous, Ulcerative, Ischemic Functional colonic disease IBS, Intestinal pseudoobstruction/Ogilvie syndrome Megacolon/Toxic megacolon - Diverticulitis/Diverticulosis Large and/or small Enterocolitis Necrotizing - IBD Crohn's disease vascular: Abdominal angina - Mesenteric ischemia - Angiodysplasia Bowel obstruction: Ileus - Intussusception - Volvulus - Fecal impaction Constipation - Diarrhea Rectum/anus Proctitis Radiation proctitis - Proctalgia fugax - Rectal prolapse - Anal fissure/Anal fistula - Anal abscess Accessory Liver Hepatitis Viral hepatitis, Autoimmune hepatitis, Alcoholic hepatitis - Cirrhosis PBC - Fatty liver NASH - vascular Hepatic veno-occlusive disease, Portal hypertension, Nutmeg liver - Alcoholic liver disease - Liver failure Hepatic encephalopathy, Acute liver failure - Liver abscess - Hepatorenal syndrome - Peliosis hepatis Gallbladder Cholecystitis - Gallstones/Cholecystolithiasis - Cholesterolosis - Rokitansky-Aschoff sinuses - Postcholecystectomy syndrome Bile duct/ other biliary tree Cholangitis PSC, Ascending - Cholestasis/Mirizzi's syndrome - Biliary fistula - Haemobilia - Gallstones/Cholelithiasis common bile duct Choledocholithiasis, Biliary dyskinesia Pancreatic Pancreatitis Acute, Chronic, Herary - Pancreatic pseudocyst - Exocrine pancreatic insufficiency - Pancreatic fistula Hernia Diaphragmatic: Congenital diaphragmatic - Hiatus Abdominal hernia: Inguinal Indirect, Direct - Umbilical - Incisional - Femoral Obturator hernia - Spigelian hernia Peritoneal Peritonitis Spontaneous bacterial peritonitis - Hemoperitoneum - Pneumoperitoneum GI bleeding Upper Hematemesis, Melena - Lower Hematochezia See also congenital, neoplasia Retrieved from http://en..org/wiki/Achalasia Categories: Gastroenterology Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Français Italiano Bahasa Melayu Nederlands 日本語 Polski Português РуÑ?Ñ?кий Svenska Tagalog اردو This page was last modified on 25 August 2008, at 23:23
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