Achalasia is a rare disease (4 new cases / year per 100,000 inhabitants), affecting the esophagus. It is a pathology attributable to the destruction of the nerves of the esophagus. We talked about it with Prof. Sandro Mattioli, expert in esophageal surgery, specialist in General and Thoracic Surgery, in Bologna and Cotignola
What are the symptoms of achalasia?
Esophageal achalasia is caused by the destruction of the intrinsic innervation of the esophagus with an autoimmune mechanism , which results in the abolition of the bowel’s motor activity. Foods and liquids then pass through the esophagus by gravity. This condition causes symptoms that worsen over time, making feeding very difficult:
- Dysphagia , or difficulty in swallowing, initially only liquids, after also solids;
- Regurgitation of food, especially during the night;
- Night cough, caused by regurgitation that enters the respiratory tract;
- Excessive salivation or sialorrhea;
- Halitosis , caused by food that stagnates in the esophagus, which consequently ferments generating stasis esophagitis;
- Chest pain;
- Weight loss.
How is the diagnosis made?
In the presence of the symptoms described above, a radiological study of the first digestive tract, endoscopy and esophageal manometry is required when the first two tests suggest the diagnosis of achalasia.
What are the therapeutic possibilities, what are the results and complications?
Five different therapies are possible, pharmacological, endoscopic and surgical: all have the purpose of opening the cardias, the anatomical and functional valve located between the esophagus and the stomach, which in achalasia is permanently contracted and blocks the transit of food:
- Temporary relaxation of the cardias follows the administration of nifedipine, a calcium channel blocker, sub lingually; this drug is vasodilator, it can produce moderate hypotension, headache; this therapy is symptomatic, it can be implemented for short times;
- With the inoculation of botulinum toxin endoscopically, in the muscle fibers that form the cardias, the pressure of the lower esophageal sphincter is reduced. In general, this procedure gives good temporary results but involves frequent relapses and the onset of resistance to treatment in about 50% of patients;
- The pneumatic dilation, carried out endoscopically, achieves good, long-lasting results. If the endoscopic maneuver is performed by an expert doctor, the risks of perforation and bleeding are very limited. In 25% of cases it is necessary to repeat the procedure to obtain an adequate clinical result which is good but lower than the results obtained with surgery (Heller-Dor point 5) in reference centers;
- With the esophageal myotomy performed endoscopically with a flexible instrument, known by the acronym POEM, the section of the musculature of the cardias is obtained, therefore the abolition of the valve, but an anti-reflux mechanism is not rebuilt. POEM can be burdened by over 40% of pathological gastro-esophageal reflux and even severe esophagitis; this condition requires the continuous intake, indefinitely, of drugs that reduce gastric acid secretion;
- Heller’s extramucosal myotomy associated with anti-reflux plastic according to Dor, performed with laparoscopic technique, is now the “gold standard” treatment for the treatment of achalasia. This technique has been adopted in over 70% of the cases published in the international scientific literature. The laparoscopic technique drastically reduces post-operative pain, the patient is able to feed himself the day after the operation, he is generally discharged after 2 or 3 days. The resumption of normal activities is almost immediate. The reference esophageal surgery centers offer high rates of good results, in the face of minimal complications. The Heller-Dor was proposed by me in the 1980s to the international community on the basis of precise clinical and pathophysiological data. We perform it with the intraoperative manometric control, with which we take into account the characteristics of each patient.
How to choose therapy?
Medical therapy and botulinum toxin injection can be considered as transition solutions, for example during pregnancy, for work reasons, if it is not possible temporarily to access a reference center that ensures good results. POEM raises important questions in many specialists, for example about the risk of cancer, as I will say later. The choice of a valid and definitive therapy concerns between pneumatic dilation and surgery. In my opinion, the surgical solution must be considered when it is possible to access a reference center.
What are the risks of cancering the acalasic esophagus? Can this possibility influence the choice of therapy?
In the acalasic megaesophagus, as the terminal phase of the disease is defined, in which the esophagus is very dilated, sinuous, the food stagnates inside, ferments and causes intense inflammation which in the long run induces epidermoidal cancer. If after the abolition of the cardial valve, whether it is dilation, POEM or surgical myotomy not associated with an anti reflux plastic, the condition of chronic reflux is created, the formation of adenocarcinoma is possible. With my group we have been studying for a few decades a wide range of patients suffering from esophageal achalasia, operated over time with very different techniques and not operated. We recently presented and published internationally the data we collected with a dedicated research program. In the case of a decompensated megaesophagus, we can calculate the cancer risk index on the basis of a validated algorithm. If the risk is low, we recommend conservative surgery, performed by us in these cases with a modified laparoscopic Heller-Dor technique; if the risk is high we recommend esophagectomy. In a series of patients operated in the university surgical group to which I belong, between 1955 and the end of the 70s, in which severe gastro-oesophageal reflux disease developed, we ascertained that the formation time of Barrett’s esophagus , an anatomopathological condition in which adenocarcinoma is implanted, is much less, for 50%, of the time in which Barrett is formed in reflux patients, for example with hiatal hernia and an esophagus with unimpaired motility. This is one of the reasons why I raised serious concerns about the use of POEM.