Achalasia, also known as esophageal achalasia, achalasia cardiae, cardiospasm, dyssynergia esophagus, and esophageal aperistalsis, is an esophageal motility disorder, a disease in which normal movement of the muscles in the esophagus is disturbed. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The International Statistical Classification of Diseases and Related Health Problems 10th Revision ( ICD -10) is a coding of diseases and signs symptoms abnormal findings K00-K93 - Diseases of the digestive system (K00-K14 Diseases of oral cavity salivary glands and jaws ( Disorders of Tooth development and Eruption The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify Diseases The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. The Mendelian Inheritance in Man project is a Database that catalogues all the known Diseases with a genetic component, and—when possible—links them The Diseases Database is a free Website that provides information about the relationships between medical conditions Symptoms, and Medications. MedlinePlus, with the MedlinePlus Medical Encyclopedia, is a website network containing Health information from the world's largest medical Library eMedicine is an online clinical medical knowledge base that was founded in 1996 by Scott Plantz and Richard Lavely two medical doctors Medical Subject Headings ( MeSH) is a huge Controlled vocabulary (or metadata system for the purpose of indexing journal articles and books An esophageal motility disorder is a medical disorder causing difficulty in Swallowing, Regurgitation of food and a Spasm -type pain which can The esophagus or oesophagus (see American and British English spelling differences) sometimes known as the gullet, is an organ in It is characterized by difficulty swallowing, chest pain and regurgitation. Dysphagia should not be confused with the similarly pronounced Dysphasia, a language disorder In Medicine, chest pain is a Symptom of a number of serious conditions and is generally considered a Medical emergency. Regurgitation is the controlled flow of Stomach contents back into the Esophagus and Mouth. Diagnosis is reached with esophageal manometry. An esophageal motility study (EMS or esophageal manometry is a study performed to evaluate the pressure of the Esophagus in various stages along its length Various treatments are available, but none cure the condition completely. Certain medications may be used, as well as injection of the esophageal muscles with botulinum toxin, but more permanent relief is brought by esophageal dilatation and surgical cleaving of the muscle (e. Botulinum toxin is a Neurotoxin Protein produced by the Bacterium Clostridium botulinum. Esophageal dilatation is a therapeutic endoscopic procedure that enlarges the lumen of the Esophagus. g. a procedure known as Heller myotomy). Heller myotomy is a surgical procedure in which the muscles of the Cardia ( Lower esophageal sphincter or LES are cut allowing food and liquids to pass to the ^[1][2]

In achalasia, 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. Smooth muscle is a type of non- Striated muscle, found within the Tunica media layer of large and small Arteries and Veins, the bladder The esophagus or oesophagus (see American and British English spelling differences) sometimes known as the gullet, is an organ in In the Esophagus After food is chewed into a bolus it is swallowed to move it into the esophagus The cardia (also known as Z-line or esophagogastric junction or gastroesophageal junction) is the anatomical term for the junction orifice of Swallowing, known scientifically as deglutition is the process in the human or animal body that makes something pass from the Mouth, to the Pharynx, into ^[3] 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). Esophageal cancer is malignancy of the Esophagus. There are various subtypes Chagas disease (doença de Chagas enfermedad de Chagas mal de Chagas in both languages also called American trypanosomiasis) is a tropical Parasitic ^[1] Achalasia affects one person in 100,000 every year. ^[2][1]

Signs and symptoms

Characteristic symptoms of achalasia are worsening difficulty in swallowing, uniquely involving both fluids and solids simultaneously, and burning chest pain that may resemble heartburn and gastroesophageal reflux. Dysphagia should not be confused with the similarly pronounced Dysphasia, a language disorder In Medicine, chest pain is a Symptom of a number of serious conditions and is generally considered a Medical emergency. Heartburn or pyrosis is a painful or burning sensation in the Esophagus, just below the breastbone usually associated with regurgitation of gastric acid Gastroesophageal reflux disease ( American English and Canadian English) or Gastro-oesophageal reflux disease ( British English, Hiberno-English As the esophagus gets more obstructed, undigested food may be regurgitated, especially when reclining or at night where it can cause cough and aspiration of food matter into the lungs. Regurgitation is the controlled flow of Stomach contents back into the Esophagus and Mouth. In Medicine, a cough ( Latin: tussis) is a sudden and often repetitively occurring defence Reflex which helps to clear the large breathing passages In Medicine, aspiration is the entry of secretions or foreign material into the trachea and Lungs The patient may either inhale the material lung is the essential Respiration organ in air-breathing Animals including most Tetrapods a few Fish and a few Snails The most primitive ^[1]

Diagnosis

Given the similarity of many of the symptoms with commoner digestive conditions such as gastroesophageal reflux disease (GERD) and hiatus hernia, diagnosis may be delayed. Gastroesophageal reflux disease ( American English and Canadian English) or Gastro-oesophageal reflux disease ( British English, Hiberno-English A hiatus hernia or hiatal hernia is the protrusion (or Herniation) of the upper part of the Stomach into the Thorax through a tear or weakness Only in very severe cases does a very distended part of the esophagus with a fluid level become visible on a standard chest X-ray. A chest X-ray, commonly abbreviated CXR, is a projection radiograph ( X-ray) taken by a Radiographer, of the Thorax which is used ^[1]

Specific tests for achalasia are barium swallow and esophageal manometry. A barium swallow is a Medical imaging procedure used to examine the upper GI (gastrointestinal tract which includes the esophagus and to a lesser extent the stomach An esophageal motility study (EMS or esophageal manometry is a study performed to evaluate the pressure of the Esophagus in various stages along its length A CT scan of the chest and esophagogastroduodenoscopy (endoscopy of the esophagus, stomach and duodenum), with or without endoscopic ultrasound, are typically performed to exclude the possibility of cancer. Computed tomography (CT is a Medical imaging method employing Tomography. Endoscopy means looking inside and typically refers to looking inside the body for medical reasons using an instrument called an endoscope. Endoscopic ultrasound is a medical procedure in which an endoscopically directed Ultrasound is used to obtain images of the internal organs in the ^[1] Endoscopy is typically normal, although difficulty negotiating the distal esophagus may be observed.

Barium swallow

A barium swallow investigation requires the patient to swallow a barium solution, with continuous fluoroscopy (X-ray recording) to observe the flow of the fluid through the esophagus. Fluoroscopy is an imaging technique commonly used by Physicians to obtain real-time moving images of the internal structures of a patient through the use of a fluoroscope In achalasia, normal peristaltic movement of the esophagus is lost; there is acute tapering at the level of the lower esophageal sphincter and narrowing is observed at the gastro-esophageal junction. The cardia (also known as Z-line or esophagogastric junction or gastroesophageal junction) is the anatomical term for the junction orifice of The cardia (also known as Z-line or esophagogastric junction or gastroesophageal junction) is the anatomical term for the junction orifice of The findings are classically described as "parrot's beak" or "rat's tail" appearance. In many cases there is also dilation of the area proximal to the narrowing, which may be very large and containing food debris. ^[1]

Esophageal manometry

Schematic of manometry in achalasia showing aperistaltic contractions, increased intraesophageal pressure and failure of relaxation of the lower esophageal sphincter. In the Esophagus After food is chewed into a bolus it is swallowed to move it into the esophagus

Esophageal motility studies are more sensitive than barium studies. This test involves swallowing a probe that measures muscle contraction in different parts of the esophagus during the act of swallowing. The test is not specific, in the sense that Chagasic achalasia and malignancy can give a similar appearance. ^[1]

Biopsy

Biopsy, the removal of a tissue sample during endoscopy, is not typically necessary in achalasia, but if performed shows hypertrophied of the musculature and absence of the nerve cells that together form the myenteric plexus, the network of nerve fibers that controls esophageal peristalsis. A biopsy (in Greek: βίος life and όψη look/appearance is a Medical test involving the removal of cells or tissues Hypertrophy is the increase of the size of an organ or in a select area of the tissue Part of the Enteric nervous system, Auerbach's Plexus (or myenteric plexus) exists between the longitudinal and circular layers of Muscularis externa In Chagas disease, a secondary cause of achalasia, the ganglion cells are destroyed by Trypanosoma cruzi, the causative parasite. Chagas disease (doença de Chagas enfermedad de Chagas mal de Chagas in both languages also called American trypanosomiasis) is a tropical Parasitic Trypanosoma cruzi is a species of parasitic Euglenoid Trypanosomes The species causes the Trypanosomiasis diseases ^[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. Calcium channel blockers are a class of drugs and natural substances with effects on many excitable cells of the body like the muscle of the Heart, smooth muscles Nifedipine (brand name Adalat, Nifedical, and Procardia) is a Dihydropyridine Calcium channel blocker. In Inorganic chemistry, a nitrate is a salt of Nitric acid with an Ion composed of one Nitrogen and three Oxygen atoms Isosorbide dinitrate (ISDN (also known as Dilatrate is a Nitrate used pharmacologically as a vasodilator, e Nitroglycerin ( NG) ( US spelling also known as nitroglycerine, ( UK Spelling trinitroglycerin, trinitroglycerine Unfortunately, many patients experience unpleasant side effects such as headache and swollen feet, and these drugs often stop helping after several months. A headache ( cephalalgia in medical terminology is a condition of pain in the Head; sometimes Neck or upper back pain may also be interpreted

Intra-sphincteric injection of botulinum toxin (or botox), to paralyze the lower esophageal sphincter and prevent spasms. A sphincter is a structure usually a circular Muscle, that normally maintains constriction of a natural body passage or orifice and which relaxes as required by normal physiological Botulinum toxin is a Neurotoxin Protein produced by the Bacterium Clostridium botulinum. A spasm is a sudden involuntary contraction of a Muscle, a group of muscles or a hollow organ, or a similarly sudden Contraction of an Orifice As in the case of botox injected for cosmetic reasons, the effect is only temporary, and symptoms return quickly in most patients. Botox injections cause scarring in the sphincter which may increase the difficulty of later Heller myotomy. Heller myotomy is a surgical procedure in which the muscles of the Cardia ( Lower esophageal sphincter or LES are cut allowing food and liquids to pass to the This therapy is only recommended for elderly patients who cannot risk surgery.

Pneumatic dilatation

Balloon (pneumatic) dilation, also called dilatation. Esophageal dilatation is a therapeutic endoscopic procedure that enlarges the lumen of the Esophagus. 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 done many of these forceful balloon dilations have better results and fewer perforations than inexperienced ones. There is always a small risk of a perforation which would have to be fixed by surgery right away. Gastroesophageal reflux (GERD) occurs after pneumatic dilation in some patients. Gastroesophageal reflux disease ( American English and Canadian English) or Gastro-oesophageal reflux disease ( British English, Hiberno-English Pneumatic dilation causes some scarring which may increase the difficulty of Heller myotomy if this surgery is needed later. Heller myotomy is a surgical procedure in which the muscles of the Cardia ( Lower esophageal sphincter or LES are cut allowing food and liquids to pass to the 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. This treatment may need to be repeated with larger balloons for maximum effectiveness.

Surgery

Heller myotomy helps 90% of achalasia patients. Heller myotomy is a surgical procedure in which the muscles of the Cardia ( Lower esophageal sphincter or LES are cut allowing food and liquids to pass to the It can usually be performed by a keyhole approach, or laparoscopically. Laparoscopic surgery, also called Minimally invasive surgery (MIS, bandaid surgery, Keyhole surgery, or pinhole surgery ^[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 added in order to prevent excessive reflux, which can cause serious damage to the esophagus over time. Nissen fundoplication is a Surgical procedure to treat Gastroesophageal reflux disease (GERD and Hiatus hernia. In a Dor (anterior) fundoplication, part of the stomach is laid over the esophagus and stitched in place so whenever the stomach contracts, it also closes off the esophagus instead of squeezing stomach acids into it. After surgery, patients should keep to a soft diet for several weeks to a month, avoiding foods that can aggravate reflux. A Soft diet is recommended in many situations including some types of Dysphagia (difficulty swallowing surgery involving the jaw mouth or Gastrointestinal tract

Follow-up

Follow-up monitoring: Even after successful treatment of achalasia, swallowing may still deteriorate over time. It's 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. Esophagectomy or Oesophagectomy (regional variation in spelling is the surgical removal of all or part of the Esophagus (also spelt 'oesophagus' Some doctors 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

External links

• and Diseases/Digestive Disorders/Esophagus/ Achalasia at the Open Directory Project
• U.S. Society for Surgery of the Alimentary Tract - guideline about achalasia]
• Patient.co.uk - page on achalasia