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Achalasia Cardia

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.

How does it present?
  • 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.

How is it diagnosed (Identified) ?
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 and heart attack. Diagnosis of achalasia begins with a careful medical history. The history should focus on the timing of symptoms and on eliminating other medical conditions that may cause similar symptoms. Specific tests for achalasia are barium swallow, endoscopy of the esophagus, stomach, and duodenum (Upper G I Endoscopy) and esophageal manometry.

Is Esophageal Manometry a must before any treatment?
Yes manometry is a must before treatment for Achalasia as it is the only confirmative test to diagnose achalasia. Because of its sensitivity, esophageal manometry (esophageal motility study) is considered the key test for establishing the diagnosis of achalasia.We frequently see patients coming with recurrence of symptoms after surgery or endoscopic dilatation and with incomplete workup before treatment. In many such patients there is a doubt about the diagnosis of achalasia. Manometry after surgery or dilatation is difficult to interpret and may not give adequate information required. A reverse scenario, there are many patients with difficulty in swallowing but normal endoscopy and barium study and are counselled to have no disease. Such scenario can arise in early achalasia and is always picked up on manometry. So all patients with difficulty in swallowing and normal endoscopy should certainly get an esophageal manometry done.

What is Manometry?
It is an investigation of motility function of esophagus and the LES (Muscular valve at the lower end of esophagus). A thin tube called catheter containing the transducer is inserted through the nose down your foodpipe. The catheter is attached to the manometry machine with sophisticated software. The catheter measures muscle contractions in different parts of the esophagus during the act of swallowing. Manometry reveals failure of the LES to relax with swallowing and lack of functional peristalsis in the smooth muscle of esophagus. Dr Chirag Tahkkar has very large experience in esophageal manometry and he is the first person to do these investigation in Gujarat.

What are the treatment options?
Treatments for achalasia include oral medications, stretching of the lower esophageal sphincter under endoscopic guidance (endoscopic dilatation), surgery to cut the sphincter (Heller’s Cardiomyotomy), and the injection of botulinum toxin (Botox) into the sphincter. All four treatments reduce the pressure within the lower esophageal sphincter to allow easier passage of food from the esophagus into the stomach. I would stress that no treatment improves the contraction of the foodpipe. This means only one problem, the lower end not opening on swallowing is addressed in each treatment modality. We still don’t have an answer to the non functioning of the foodpipe muscle. This explains some recurrence that we see with each modality. Patient needs some diet modification after treatment. A newer method of cutting the muscle of LES by endoscopic method called POEM (Per Oral Endoscopic Myotomy) has been developed. This method hold promise for future but is still not fully standardized and accepted.

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.

Is Surgery better than Endoscopic Dilatation?
In my opinion surgery has an advantage over endoscopic dilatation. In balloon (pneumatic) dilatation, the muscle fibers are stretched and torn by forceful inflation of a balloon placed inside the lower esophageal sphincter. Gastroenterologists who specialize in achalasia have performed many of these forceful balloon dilatations and achieve better results and fewer perforations. There is always a small risk of a perforation which may require immediate surgical repair. Those patients who have recurrence after 2 dilatation will require laparoscopic surgery. Pneumatic dilatation causes some scarring which may increase the difficulty of Heller cardiomyotomy if the surgery is needed later. Gastroesophageal reflux (GERD) occurs after pneumatic dilatation in some patients. Pneumatic dilatation is most effective in the long term on 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.

What should I expect after surgery?
Recovery after surgery is very fast as it is done by laparoscopic method. Patient is up and about in few hours of surgery. Liquids can be allowed in few hours of surgery and gradually progressed to soft diet. We advise soft diet for a couple of weeks after surgery. There are no stitches to be removed and just a dressing is required after a couple of days. Patient can be discharged in a couple of days. Routine household and office work can be started in a couple of days. There are no specific restrictions after surgery. We regularly do such surgeries and our patients are highly satisfied in long term.

What are the options if there is recurrence of dysphagia (inability to swallow food) ?
If you have a recurrence after two endoscopic dilatations a surgical cardiomyotomy is ideal for you. If you have recurrence after a surgical cardiomyotomy you may require a repeat surgery or may benefit from a dilatation. This depends on many factors and your surgeon should do a thorough assessment to decide this. Recurrence after a long timeof treatment may show a hugely dilated esophagus what we call a “Sigmoid Esopahgus “. Surgery or dilatation to open the LES alone is not likely to give any result in such a situation and a major surgery in form of removal of esophgus and replacing it with a tube made from stomach (Esophgectomy with gastric pullup) may be required.



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