What is Achalasia?
Achalasia is a rare disease of the muscle of the esophagus (swallowing tube). The term achalasia means "failure to relax" and refers to the
inability of the lower esophageal sphincter (a ring of muscle between the lower esophagus and the stomach) to open and let food pass into the
stomach. As a result, patients with achalasia have difficulty swallowing food.
The History of Achalasia.
1672-Sir Thomas Willis, First described Achalasia/cardiospasm and treated the problem with a dilation using a whale sponge attached to a whale
1881 von Mikulicz described the disease as cardiospasm, felt it was a functional problem not a mechanical one.
1913-Ernest Heller, Was the first person to successfully perform a esophagomyotomy
1929-Hurt and Rake figured out that the problem was due to the LES not relaxing. Named the disease Achalasia-failure to relax.
1937-F.C. Lendram changed the name from cardiospasm to Achalasia. (Hard to say who really changed the name between the last two entries
but that is the information that was found)
1962-Dor reports the first anterior partial fundoplication
1963-Toupet reports first posterior partial fundoplication
1991-Shimi and his colleagues perform the first Laproscopic Heller's in England
1994-Paricha et al introduces Botox as a method for reducing LES pressure.
Here is a link to read the history on achalasia. Sir Thomas Willis
How does the normal esophagus function?
The esophagus has three functional parts. The uppermost part is the upper esophageal sphincter, a specialized ring of muscle that forms the
upper end of the tubular esophagus and separates the esophagus from the throat. The upper sphincter remains closed most of the time to
prevent food in the main part of the esophagus from backing up into the throat. The main part of the esophagus is referred to as the body of the
esophagus, a long, muscular tube approximately 20 cm (8 in) in length. The third functional part of the esophagus is the lower esophageal
sphincter, a ring of specialized esophageal muscle at the junction of the esophagus with the stomach. Like the upper sphincter, the lower
sphincter remains closed most of the time to prevent food and acid from backing up into the body of the esophagus from the stomach.
The upper sphincter relaxes with swallowing to allow food and saliva to pass from the throat into the upper esophageal body. The muscle in the
upper esophagus just below the upper sphincter then contracts, squeezing food and saliva further down into the esophageal body. The ring-like
contraction of the muscle progresses down the body of the esophagus, propelling the food and saliva towards the stomach. (The progression of
the muscular contraction through the esophageal body is referred to as a peristaltic wave.). By the time the peristaltic wave reaches the lower
sphincter, the sphincter is open, and the food passes into the stomach.
The esophagus is a hollow muscular tube that connects the back of the throat to the top of the stomach. This tube in the adult ranges from 10 to
14 inches in length, and 1 inch in diameter. At rest, the esophagus is collapsed but opens readily to accept food and liquids. The upper portion
of this muscular tube is composed of muscle similar to that of the arms and legs (skeletal muscle) and is therefore under voluntary control. The
other two thirds of the esophagus are made of smooth muscle like the rest of the gut and is not under voluntary control. These muscles are
arranged with an inner circular layer and an outer longitudinal one.
Function and Control
To keep our food from coming back from the stomach to our mouth, the esophagus has two sphincter muscles; the one at the top is called the
upper esophageal sphincter (UES); the one at the bottom is the lower esophageal sphincter (LES). Functionally, the esophagus is then divided
into the UES, the body of the esophagus, and the LES.
The act of swallowing takes a great deal of coordination, most of which is unconscious. Within the brain is an area known as the "swallowing
center." This center acts to receive information from the esophagus and throat and to send out the message to activate swallowing. This
message center may be affected by strokes and other injuries. The smooth muscle has sensors within the wall that detect stretch from food or
liquids; also present are hormones and nerves which then make the smooth muscle contract to push the food or liquid toward the stomach. At
rest, then, the UES and LES are closed and the body of the esophagus is quiet. When we put food into our mouth, the nervous system detects
this and directs the UES to open. The food within the esophagus is moved downstream by movement of the smooth muscle (primary peristalsis),
and the LES relaxes for food to enter the stomach. This entire series of events takes about 8 seconds. After swallowing, the smooth muscle may
contract to sweep remaining material into the stomach (secondary peristalsis). This is also a protective mechanism to strip any acid that may
have washed back into the esophagus from the stomach.
How is esophageal function abnormal in achalasia?
In achalasia there is an inability of the lower sphincter to relax and open to let food pass into the stomach. In at least half of the patients, the
lower sphincter resting pressure (the pressure in the lower sphincter when the patient is not swallowing) also is abnormally high. In addition to the
abnormalities of the lower sphincter, the muscle of the lower half of the esophagus does not contract normally, that is, peristaltic waves do not
occur, and, therefore, food and saliva are not propelled down the esophagus and into the stomach. A few patients with achalasia have high-
pressure waves in the lower esophageal body following swallows, but these high-pressure waves are not effective in pushing food into the
stomach. These patients are referred to as having “vigorous” achalasia. These abnormalities of the lower sphincter and esophageal body are
responsible for food sticking in the esophagus.
What causes achalasia?
The cause of achalasia is unknown. Theories on causation invoke infection, heredity or an abnormality of the immune system that causes the
body itself to damage the esophagus (autoimmune disease).
The esophagus contains both muscle and nerves. The nerves coordinate the relaxation and opening of the sphincters as well as the peristaltic
waves in the body of the esophagus. Achalasia has effects on both the muscles and nerves of the esophagus; however, the effects on the
nerves are believed to be the most important. Early in achalasia, inflammation can be seen under the microscope in the muscle of the lower
esophagus, especially around the nerves. As the disease progresses, the nerves begin to degenerate and ultimately disappear, particularly the
nerves that cause the lower esophageal sphincter to relax. Still later in the progression of the disease, muscle cells begin to degenerate,
possibly because of the damage to the nerves. The result of these changes is a lower sphincter that cannot relax and muscle in the lower
esophageal body that cannot support peristaltic waves. With time, the body of the esophagus stretches and becomes very enlarged (dilated).
What are the symptoms of achalasia?
The most common symptom of achalasia is difficulty swallowing (dysphagia). Patients typically describe food sticking in the chest after it is
swallowed. Dysphagia occurs with both solid and liquid food. Moreover, the dysphagia is consistent, meaning that it occurs during virtually every
Sometimes, patients will describe only a heavy sensation in their chest after eating that may force them to stop eating. Occasionally, pain may be
severe and mimic heart pain. (Please read the Angina article on MedicineNet for more detail on different causes of chest pain).
Regurgitation of food that is trapped in the esophagus can occur, especially when the esophagus is dilated. If the regurgitation happens at night
while the patient is sleeping, food can enter the throat and cause coughing and choking. If the food enters the trachea (windpipe) and lung, it
can lead to pneumonia (aspiration pneumonia).
Because of the problem swallowing food, a large proportion of patients with achalasia lose weight.
What is an esophegeal spasm?
Esophageal spasms involve irregular contractions of the muscles in the esophagus, which is the tube that carries food from the mouth to the
stomach. These spasms do not propel food effectively to the stomach. The cause of esophageal spasm is unknown. Very hot or very cold foods
may trigger an episode in some people. The pain may be indistinguishable from angina and may radiate to the neck, jaw, arms, or back. Spasms
can last anywhere from moments to hours, and are usually very uncomfortable!! There are some medications that can help control these,
nitroglycerin may be effective in an acute episode. Long-acting nitroglycerin and calcium channel blockers are also used to treat esophageal
spasms. Chronic cases are sometimes treated with low-dose antidepressants such as nortryptiline, to reduce symptoms. Some home remedies
to try are: sipping hot or cold drinks, fizzy drinks like soda, some over the counter pain medications might help; it is mostly trial and error to see
what works best for each individual.
What are the complications of achalasia?
The complications of achalasia include weight loss and aspiration pneumonia. There often is inflammation of the esophagus, called esophagitis,
which is caused by the irritating effect of food and fluids that collect in the esophagus for prolonged periods of time. There may be esophageal
ulcerations as well.
Of potential concern is the possibility that there is an increased occurrence of cancer of the esophagus in patients with achalasia. However,
there is insufficient scientific evidence that achalasia increases a person’s risk of developing esophageal cancer, so authorities are currently not
recommending that patients with achalasia undergo regular upperfor cancer surveillance.
How is achalasia diagnosed?
Theoften is suspected on the basis of the history. Patients usually describe a progressive (worsening) of dysphagia for solid and liquid food over
a period of many months to years. They may note regurgitation of food, chest pain, or loss of weight. Rarely, the first symptom is aspiration
Because patients typically will learn to compensate for their dysphagia by taking smaller bites, chewing well, and eating slowly, the diagnosis of
achalasia often is delayed by months or even years. The delay in diagnosis of achalasia is unfortunate because it is believed that early
treatment--before marked dilation of the esophagus occurs—can prevent esophageal dilation and its complications.
The dysphagia in achalasia also is different from the dysphagia of esophageal stricture (narrowing of the esophagus due to scarring) and
esophageal cancer. In achalasia, dysphagia occurs with both solid and liquid food, whereas in esophageal stricture and cancer, the dysphagia
typically occurs only with solid food.
Stages of achalasia.
There are many different stages of achalasia. Here are some of the stages of achalasia:
2-3 cm is normal,
4-5 cm is stage two and bird beak looking,
5-7 cm is stage three,
8+ cm is signmoid or stage 4.