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    Achalasia – (lat. Achalasia cardiae) 

    Achalasia is a rare sickness of the esophagus that most often occurs in the ages 10 to 30 years [1]. It manifests itself as a disturbance of the motor ability both in the esophagus inner muscle layer, as well as in the lower esophagus mouth, also known as the upper stomach mouth or LES (Lower Esophageal Sphincter) [1]. Why people are afflicted by this sickness is unknown, but every year it affects approximately 100 new victims in Sweden of both sexes (1.2 – 1.5 people per 100,000 citizens per year)[1]. Because the sickness is unusual, it’s often difficult to to get a correct diagnosis straightaway. Averagely, a patient with achalasia will have an incorrect diagnosis for four years, before the correct conclusion is reached. It is common that symptoms are confused with those of hiatus hernia or, just as likely, the sickness can be incorrectly diagnosed as anorexia or bulimia nervosa. [1]

    For these reasons, we are going to highlight some important differentiators, and also give a short description of different treatments that are available for achalasia.
     


    The common symptoms of achalasia

    • It is always a problem to swallow food of above a certain bite size (bolus).
    • You would prefer to stand up during mealtimes and even try to ’jump’ the food down.
    • Food becomes stuck in the esophagus unchewed and unprocessed, which creates an urge to be sick. Food vomited in this way comes up whole, undigested and tastes as it did when you swallowed it. Amongst youths and teenagers this is easily mis-diagnosed as anorexia, or bulimia.
    • A feeling of cramp in the chest.
    • Repeated pneumonia is common.
    • Sudden weight loss.

    It is common that the symptoms for achalasia are confused with those of hiatus hernia. Read more about hiatus hernia here.

    If you suspect achalasia you should immediately ask for a referral to an ear, nose and throat doctor, a stomach surgeon, a child surgeon or a gastroenterologist.  Read more under ”Examinations -  if you suspect achalasia”. [1]

     

    Achalasia and hiatus hernia - the differences

    Achalasia is a rare type of esophageal dysphagia, but in some cases can be reminiscent of the symptoms of a hiatus hernia, which is much more common. If diagnosed, achalasia should be recorded in the medical records with the entire correct Latin description ”achalasia cardiae”, and nothing else.
     

    Misconceptions and wrong diagnoses

    Unfortunately, it’s not uncommon that achalasia and hiatus hernia are confused with each other, which can lead to an incorrect diagnosis when the doctor examines. Misunderstandings between the doctor and patient can also occur when, for example, the doctor describes a blockage in the upper stomach mouth - esophagal sphincter - using the Latin word ”achalasia” which means ”blockage” or bottleneck. Hiatus hernia can also be perceived as a feeling of a blockage in the chest, which the doctor can correctly report as ”achalasia in the rib cage”. This must not be confused with the diagnosis ”achalasia cardiae”.

    The difference here is that person with achalasia cardiae has consistent difficulty in swallowing food, has an unusually high pressure in the lower part of esophagus at the entrance to the stomach, loses weight rapidly, and often suffers a series of pneumonias. [1] The patient does not have acidic reflux as he would with a hiatus hernia: a patient with achalasia has a basic pH value (pH> 7) in the esophagus when examined. In the table below we illustrate the differences between these two types of esophagal dysphagia.

     

    Checklist – the differences between achalasia cardiae and hiatus hernia

    Achalasia cardiae

    Hiatus hernia

    Always a problem with swallowing food above a certain bite size.

    This symptom comes and goes.

    Never had acidic reflux or heartburn.

    Acidic reflux or heartburn.

    Rapid weight loss.

    No direct weight loss.

    Recurring pneumonias.

    Pneumonia unusual.

    Food that has been swallowed is regurgitated in an unprocessed form. The taste is the same as when it was swallowed.

    Food that has lodged in the esophagus can be regurgitated a while after a meal, with a frothy saliva.

    High-pressure at the lower esophagus mouth (upper stomach mouth).

    Discovered by measuring pressure through high-resolution colour manometry which uses colours to show the pressure in the various vessels: for example the colour red indicates high pressure, and blue low pressure.

    Low-pressure at the lower esophagus mouth (upper stomach mouth).

    Discovered by measuring pressure through high-resolution colour manometry which uses colours to show the pressure in the various vessels: for example the colour red indicates high pressure, and blue low pressure.

    A swallow x-ray will show that the lower part of the esophagus is constricted like a bird’s beak, whilst it may be widened in the section above this. As an example, a Novalucol® tablet, swallowed during an x-ray examination, can be seen to become stuck during its passage and remain in the esophagus for a while before it is passed to the stomach

    A swallow x-ray is performed with a barium meal being swallowed whilst the patient is lying on his side, with a pressure cuff around the stomach. In this way pressure is invoked on the rupture and part of the stomach glides up above the diaphragm. Note! It can still be difficult to discover the hernia, and for that reason it is very important to listen carefully to the symptoms.

    Measuring the pH value in the esophagus returns a value on the base side >7 (pH 7 = neutral; pH 4 – 7,5 = normal range)

     

    Measuring the pH value in the esophagus returns a value on the acidic side <4 (pH 7 = neutral; pH 4 – 7,5 = normal range)

     


     

    The underlying causes of the symptoms of achalasia

    Achalasia is characterised by a disturbance of the motor functions both in the inner longitudinal muscle layer of the esophagus, and in the lower esophagus mouth (also known as the upper stomach mouth). This means that the forcing muscle layer in the esophagus has stopped working properly, and therefore food travels less efficiently than normal down to the stomach.

    Read more on “The swallowing process -  when it works as it should

    The pressure is high at the lower esophagus mouth, and the esophagus does not empty itself completely. Typically, food remains lying in the lower part of the esophagus which, with time, becomes distended: see picture. To have food still sitting in the esophagus creates a feeling of pressure and of wanting to vomit. This is also an underlying reason why a patient afflicted with achalasia would rather eat standing up, or even try to ’bounce down’ their food by jumping. They will also have cramp in the chest, lose weight rapidly, and often be struck by repeated pneumonias.  A young person’s being sick in this way has nothing to do with bulimia nervosa or anorexia nervosa. [1]

     

    If you suspect achalasia - examination immediately!

    If achalasia cardiae is suspected you should immediately request a referral to an ear, nose and throat doctor, a stomach surgeon, a child surgeon or a gastroenterologist; those are the doctors who have most experience of this condition.

    To diagnose achalasia, a pH test, a pressure measurement (e.g. a colour manometry), and a swallow x-ray must be carried out. These are required to establish if: a base pH value (>7) is present, if there is an increased pressure in the region of the lower esophagus mouth, and to judge the condition of the forcing (peristaltic) muscles, and to look for any evidence of widening of the esophagus.

    A gastroscopy is performed to rule out other causes. A flexible tube is inserted down the esophagus so that pictures and samples can be taken on the way down the stomach. Here you can see if the esophagus is widened and if, despite a fasting period before the examination, pieces of food remain in the esophagus. In the early stages of the condition, and in inexperienced hands, a wrong diagnosis can easily be made. [1, 3]

     

    Present on examination: [1]

    • Base pH value (pH > 7) when tested in the esophagus.
    • High-pressure in the region of the lower esophagus mouth LES. No forcing - peristaltic - waves, during swallowing. High pressures show during the pressure measuring procedure using colour manometry.
    • During the swallowing x-ray, if the condition has existed for more than 1 to 2 years, a widening of the esophagus will be seen where it has been stretched by food that has become stuck on its way down to the stomach. The lower part of the esophagus looks constricted like a bird’s beak, immediately before the entrance to the stomach.

       

    Treating achalasia

    What is perhaps most important is that one is correctly diagnosed, is taken seriously, and does not worry needlessly about some other dangerous, imagined, condition. If one loses weight, or doesn’t put weight on as one should, then surgery should always be considered as an option. [1] If, however, there is no acute risk for the patient’s health then a neuromuscular treatment with IQoro® should be considered. IQoro® is a new and unique neuromuscular treatment method that requires just 30 seconds’ exercise, three times per day and can be tried as an alternative before a decision to operate is made. Read more below.
     

    There are three proven surgical methods: [1]

    • The mouth of the esophagus (upper stomach mouth) is widened from within with a balloon. This method often has to be repeated and can be unnecessarily painful for the patient. There is also a risk that the esophagus will split, requiring emergency surgery. [3]
       
    • The upper abdominal cavity, or the lower chest cavity is opened and the lower part of the esophagus musculature is cut through as far as the mucous membrane. This is done so that the lower esophagus can be widened, and food thus pass more easily (Heller’s myotomy).
       
    • The same as above, but a so-called Fundoplication is performed as well, in which a cuff is fitted around the lower esophagus mouth so that food cannot run back into the esophagus through the now-widened esophagus mouth. This procedure has been used successfully in Sweden since the 1990s.
       
    • A new method is the Peroral Endoscopic Myotomy (POEM). In this procedure, the cutting of the lower part of the esophagus musculature is carried out through an endoscopic technique. The method should mean that it is easier to operate to the individual patient’s requirement, and that the patient can be dismissed from hospital after just a few hours. However the method requires careful evaluation. Causing a rupture in the esophagus during the operation would be the most serious complication, and this could lead to infection and the need for further operations. [3]
       

    An operation causes the symptoms to cease in 80 to 90% of cases, but the operation does not treat the fundamental cause: the sickness in the musculature remains. Even after the treatment, the patient’s swallowing problems can be present for the rest of his or her life to a greater or lesser extent. [2, 3] In instances where the operation is not successful there can be serious consequences for the patient’s future quality of life.
     

    IQoro® – to be considered before the decision to operate.

    There has been not yet been a clinical study of IQoro® on patients with achalasia cardiae. Upon diagnosis of the condition, if there is no suspected risk of a ruptured esophagus, IQoro® could be tested as a treatment alternative - under the careful supervision of a doctor – before a decision to operate is made.

    Research[4] shows that IQoro® acts on, and regenerates, the entire swallowing process from the face, lips, mouth, tongue, pharynx, esophagus and via the diaphragm to the stomach. It stimulates the sensory nerves in the oral cavity, and by doing this reaches the brain’s control system for the swallowing process. It is therefore probable, even if un-researched at present, that IQoro® should be able to act on the impairment in the esophagus’ inner forcing muscle layer and the lower esophagus mouth.

    Read more about treatment with IQoro® here.

    Read more about the research behind IQoro®

    Achalasia – (lat. Achalasia cardiae) 
    Achalasia – (lat. Achalasia cardiae) 

     


    Text by:

    Dr. Mary Hägg, Doctor of Medicine, Post-doctoral researcher at Uppsala University specialising in orofacial medicine, Head of Department at the Speech and Swallowing Faculty: Ear, Nose and Throat Clinic at Hudiksvall Hospital, Sweden. Registered Dentist. In cooperation with MYoroface.

    October 2016.


    (Translation from the original Swedish text by T.W.Morris, MYoroface)

    1. Tibbling-Grahn L. (2004), Broschyr 2 Svenska dysfagiförbundet, Stoppar maten upp i bröstet när du äter? Achalasia cardiae, En matstrupssjukdom som kan starta i tidiga tonåren, (pdf).
      (English translation: ”Does food get stuck in your chest when you eat? Achalasia cardiae: an esophagus sickness that can start in the early teens”).
       

    2. Läkartidningen.se, (2014), Ny metod att behandla akalasi provas nu, hämtad 2016-09-09,
      http://www.lakartidningen.se/Klinik-och-vetenskap/Klinisk-oversikt/2014/11/Ny-metod-att-behandla-akalasi-provas-nu/
      (English translation: ”New method of treating Achalasia cardiae is now being tested”).
       

    3. Överläkare Larsson LG., (2004), Universitetsjukhuset Örebro, Kirurgiska kliniken, Patientinformation till dig som ska opereras för Akalasi, (pdf) hämtad 2016-09-09,
      http://www.usorebro.se/Files-sv/Sjukhusstab/Dokument/Patientinfo/alakais.pdf
      (English translation: ”Patient information to those about to be operated on for Achalasia cardiae”).
       

    4. IQoro® - List of scientific articles

     

     

        

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