Isolated Achalasia and Achalasia associated with syndromes
What is it?
It is a disease that affects the innervation of the esophagus and associated neurotransmitters and is characterized by two aspects:
- absence of peristaltic waves of the body of the esophagus or the absence of propagation of waves that allow food to pass through the esophagus and reach the stomach.
- little or no relaxation of the lower esophageal sphincter, which is the "valve" between the stomach and the esophagus
To date, the true cause of this disease has not been clarified.
Causes and risk factors
It affects men and women of almost all ages equally, especially between the ages of 30 and 60, with a peak around 40. The incidence is about 8 cases per 100,000 inhabitants per year.
Which are the symptoms?
The characteristic symptoms are:
- dysphagia (sensation of obstructed passage of food in the esophagus) which arises first in episodes, then constantly. It is often defined as a 'paradox' because it is more pronounced for liquids than for solids
- retrosternal pain
- regurgitation (return to the mouth, without retching, of undigested material, typically not acidic as it never reached the stomach)
- significant weight loss, closely linked to the course and duration of the disease
How is it diagnosed?
It is possible to diagnose with the radiological study of the esophageal transit and, above all, with the esophageal manometry better if at high resolution.
The gastroscopy can direct the diagnosis but it is not decisive.
Suggested exams
How is it treated?
It is impossible to restore esophageal motility; Therefore, the therapeutic options are based on an attempt to reduce the obstruction opposite to the passage of food into the stomach, due to the inability to relax the lower esophageal sphincter (cardia).
- Pharmacological therapy: drugs are prescribed that reduce the tone of the lower esophageal sphincter, in particular calcium antagonists or nitro derivatives; however, this therapy is not definitive and is usually used as a temporary measure to improve symptoms and to allow the patient to eat.
- Mechanical expansion: the balloon is inserted until it reaches the cardia (the "valve" that does not open correctly); the balloon is inflated to sever the muscle fibers of the lower esophageal sphincter and allow subsequent passage of food into the stomach. This procedure is simple and immediate; however, it is impossible to predict the duration of the benefit. In addition, there is a risk of cracking or perforation of the esophagus of about 4-6%.
- Botulinum toxin treatment: endoscopic injection of botulinum toxin into the lower esophageal sphincter; it is an effective treatment that is simple to implement and does not pose a risk to the patient. However, treatment usually does not guarantee long-term effectiveness.
- Surgical treatment: Heller extra-mucous myotomy in combination with antireflux plasty guarantees the best result. This technique is usually performed laparoscopically, without incising the abdominal wall. To correct the inability of the "valve" to relax, the muscle fibers of the cardia are surgically cut, which thus remain open, and an antireflux procedure (called antireflux plasty) is performed to limit the outflow of gastric material into the esophagus.
If the operation is performed in specialized centers, it guarantees an excellent and lasting result in terms of symptoms; in addition, thanks to modern antireflux plasty, it provides effective protection against the reflux of material from the stomach into the esophagus.
- Endoscopic myotomy (P.O.E.M.). This endoscopic technique, introduced several years ago, makes it possible to cut the muscle fibers of the cardia endoscopically. This procedure has been shown to be very effective in treating symptoms and to allow fast food resumption. The main problem with this technique is that the "valve" remains open and therefore there is a very high risk of material being thrown from the stomach into the esophagus. Studies conducted so far have identified pathological reflux in 50% of patients undergoing this procedure.
The choice of a therapeutic approach must take into account various factors and must be made jointly by the doctor and patient, who must be properly informed about the risks and benefits of each procedure.
Where do we treat it?
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