What is Achalasia Cardia?
Achalasia, also known as esophageal achalasia, achalasia cardia ,cardiospasm, and esophageal aperistalsis is a disorder of the esophagus (food pipe) in which the nerves and muscles do not work properly, causing swallowing difficulties, sometimes chest pain, regurgitation (food coming back in throat) and its consequent coughing and breathing problems (if food gets into the lungs). The cause of achalasia is unknown; however, there is degeneration of the esophageal muscles and, more importantly, the nerves that control the muscles.
Achalasia is a rare disease of the muscle of the esophageal body and the lower esophageal sphincter (LES). LES is a valve made of muscles at the lower end of food pipe (Esophagus) which opens when a person swallows foods and functions to prevent the food from coming back from stomach to esophagus after eating. In Achalsia this valve do not open on swallowing as it should do normally. Also the muscles of the food pipe which contract and send the food down normally do not work properly. Both the things together makes the food to retain in the food pipe for a longer time. Gradually with time if no treatment is done the food pipe enlarges and collects a large amount of food inside. A person can vomit (regurgitate this collected food hours after eating undigested).This food coming back may go to Trachea (airpipe) and Lungs in sleep causing cough and pneumonia. As muscles of foodpipe do not function as it should it is called an esophageal motility (activity of muscle) disorder.
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- The most common presenting feature is dysphagia (Inability to swallow food). This affects solids more than soft food or liquids.
- Regurgitation (Food or water coming back in throat or mouth) may occur in 80-90% and some patients learn to induce it to relieve pain.
- Chest pain occurs in 25-50%. It occurs after eating. It is more prevalent in early disease and may mimic pain of an heart attack.
- Heartburn is common feature because food retained in the esophagus (foodpipe) causes inflammation of esophagus Loss of weight may occur and cancer of esophagus should be ruled out.
- Nocturnal cough and even inhalation of refluxed contents is a feature of later disease. This may lead to pneumonia as well.
Oral medications Oral medications that help to relax the lower esophageal sphincter include groups of drugs called nitrates, for example, isosorbide dinitrate and calcium channel blockers, for example, nifedipine and verapamil . Although some patients with achalasia, particularly early in the disease, have improvement of symptoms with medications, most do not. By themselves, oral medications are likely to provide only short-term and not long-term relief of the symptoms of achalasia, and many patients experience side-effects from the medications.
Endoscopic Dilatation The lower esophageal sphincter also may be treated directly by forceful dilatation. Dilatation of the lower esophageal sphincter is done by having the patient swallow a tube with a balloon at the end. The balloon is placed across the lower sphincter with the help of X-rays, and the balloon is blown up suddenly. The goal is to stretch–actually to tear–the sphincter. The success of forceful dilatation has been reported to be between 60% and 90%. Patients in whom dilation is not successful can undergo further dilatations, but the rate of success decreases with each additional dilatation. If dilatation is not successful after two attempts, the sphincter may still be treated surgically. The main complication of forceful dilation is perforation (rupture) of the esophagus, which occurs 5% of the time. Half of the perforation heal without surgery, though patients with perforation who do not require surgery should be followed closely and treated with antibiotics. The other half of perforation require surgery. Although surgery carries additional risk for the patient, surgery can repair the rupture as well as permanently treat the achalasia with Heller’s cardiomyotomy. With endoscopic dilatation of LES the possibility of patient having GERD (Acid from stomach going up in foodpipe) increases and patient may require long term antacid medications for this. Death following forceful dilation is rare. Dilatation is a quick and inexpensive procedure compared with surgery, and requires only a short hospital stay. Laparoscopic Heller’s Cardiomyotomy With Fundoplication The sphincter also can be cut surgically, a procedure called Heller’s Cardiomyotomy. The surgery is done laparoscopically through small punctures in the abdomen. The esophagus is made of several layers, and the myotomy cuts only 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. It is my preference to add a fundoplication with cardiomyotomy in each patients. Thus surgery has an advantage of adding a antireflux procedure with cardiomyotomy and prevent GERD and antacid requirement. Cardiomyotomy is more successful than forceful dilation, probably because the pressure in the lower sphincter is reduced to a greater extent and more reliably. 80%-97% of patients have good results. With prolonged follow-up, however, some patients develop recurrent dysphagia. Patient requires about 2 days of hospitalization and death following such surgery is extremely rare. Dr Chirag Thakkar is having a high end experience in doing such surgeries and we see excellent results in operated patients in long term. Botulinum toxin Another treatment for achalasia is the endoscopic injection of botulinum toxin into the lower sphincter to weaken it. Injection is quick, nonsurgical, and requires no hospitalization. Treatment with botulinum toxin is safe, but the effects on the sphincter often last only for months, and additional injections with botulinum toxin may be necessary. Injection is a good option for patients who are at very very high risk for surgery, for example, patients with severe heart or lung disease. It also allows patients who have lost substantial weight to eat and improve their nutritional status prior to “permanent” treatment with surgery. This may reduce post-surgical complications.