Intestinal achalasia is a disease when cardia doesn’t open reflectively during swallowing. This condition is accompanied by low tonus of thoracic esophagus and disorders of intestinal motility. This disease was first described in 1672. According to statistics, it’s typical for 1 person out of 100.000. Most often, achalasia occurs in 40-50 years. Achalasia in children is a rare occasion, and it accounts for about 3,9% of all cases. As a rule, women are more prone to this disease than men.

Causes of achalasia development

Doctors don’t know the exact case of intestinal achalasia development. Most often cases include infection diseases, exterior pressure on the esophagus, inflammation proceses, malignant tumors, infiltrative disorders, etc. In children, achalasia of esophagus is often diagnosed after the age of five. As a rule, first symptoms of diseases are often ignored, which is why the disease is diagnosed lately. The most widespread symptoms of esophagus achalasia are dysphagy and vomiting right after meals.

Classification of esophagus achalasia (cardia)

  • I stage – functional spasm of esophagus sphincter. Narrowing of caria and widening of esophagus are absent.
  • II stage – stabile spasm of esophagus sphincter with moderate widening of lower esophagus sections.
  • III stage – scars and stenosis of sphincter with pronounced esophagus widening.
  • IV stage – pronounced stenosis of esophagus sphincter and severe widening and S-shaped deformation of esophagus that’s accompanied by chronic inflammation of esophagus mucosa.

Symptoms of cardia achalasia

Clinical symptoms of cardia achalasia are dysphagy, regurgitation and retrosternal pain. Dysphagy is characterized by problems swallowing. In some cases, the disorder of swallowing is momentary and it develops gradually; as a rule, flu and other virus diseases, or stress precede dysphagy. In some patients, the symptoms occur accidentally (for instance, after fast meals), then they become more regular and obstruct passage for both solid and liquid foods.

Dysphagy during cardia achalasia can also be elective and occur only when a person eats some certain type of foods. Adjusting to the hindered process of swallowing process, patients find new ways to regulate passage of foods – keep breathing, gulp air, drink foods with water, etc. Sometimes, paradoxical dysphagy develops during cardia achalasia – passage of liquid foods is obstructed more than passage of solid foods.

Regurgitation during cardia achalasia can develop as the result of reverse discharge of foods into mouth cavity after esophagus muscle contraction. Sometimes regurgitation can manifest into minor belching or full-mouth vomiting.

Regurgitation can be either periodic (for instance, during the process of eating together with dysphagy) and take place right after food intake, or 2-3 hours after meal. Less often, food discharge can happen in nighttime (so-called night regurgitation): foods can often get into breathing channels which causes night cough. Minor regurgitation is typical for stages I-II of cardia achalasia, and oesophageal vomiting is typical of stages III-IV, when esophagus is stretched.

Pains during achalasia can appear both with empty stomach, or when a person swallows. Pain is usually located behind the chest, and may also spread to the jaws, neck or between bladebones. While at stages I-II of cardia achalasia pains are conditioned by muscle spasms, at stages III-IV esophagitis starts developing. Cardia achalasia usually causes periodic colicky pains – esophagitis crisis that can develop because of worrying, physical activity, or during nighttime, and last for several minutes or an hour. Sometimes pain subsides after vomiting or when foods pass into stomach, in other cases it can be cured with spasmolytics.

Problems with passage of foods and constant belching lead to weight loss, loss of ability to work, lower social activity. Due to permanent symptoms, some patients with achalasia develop neurosis and affective states. Sometimes such people have to be treated by neurologist to eliminate various disorders. However, neural disorders usually disappear when cardia achalasia is treated.

Complications of achalasia esophagus

  • Esophagus bezoars
  • Diverticulum of distal segment of esophagus
  • Esophageal squamous cell cancer
  • Esophageal varicose veins dilatation
  • Esophageal periacardic fistula
  • Space-occupying lesion of neck
  • Pneumopericardium
  • Pulmonary involvement
  • Barrett esophagus
  • Stridor with obstruction of upper respiratory passages
  • Dissection of submucous membrane of esophagus
  • Suppurative pericarditis

Some of possible complications are resulted by collection of foods and secretion of esophageal glands in restrained esophagus.

In case of long-lasting achalasia, esophagus can widen considerably. Space-occupying lesions and stridor can appear as the result of pressure on upper respiratory passages.

Esophagus wall thinning can occur when it widens, and this is a factor promoting tears, dissection of submucous membrane of esophagus, suppurative pericarditis or fistulas.

Diagnosis of cardia achalasia

On different stages of achalasia, there can be cardia obstruction with minor dilation of proximal section. While the disease is progressing, a doctor can see the following changes on X-ray images: widening of esophagus, narrowing in the lower section with a small coronoid narrowing at the place of narrowed section.

Alhough the way the disease develops is pretty typical, it can be often mistaken with esophagus cancer, especially in patients after 50 and at early stages. Esophagoscopy has proved to be very useful for achalasia diagnosis. Research of esophagus movements helps to approve of clinical symptoms of achalasia. Doctors usually reveal low pressure in esophagus with dilation of its opening and loss of vermicular movement after swallowing.

After swallowing, pressure in the entire esophagus rises. During swallowing, esophagus sphincter doesn’t open, which gives doctors reason to suggest achalasia. In some patients, poor vermicular movement develops into diffuse spasm, and after swallowing a person has severe repeating spasms.

Treatment of achalasia

Achalasis treatment is rarely treated with medications. As a rule, medications only improve symptoms of disease. Patient is prescribed a spare diet, sedative drugs, vitamins and antispasmatics. As a rule, drugs give only temporary relief. Forced widening of cardia can be performed only with a mechanic, pneumatic or hydrostatic dilater.

Pneumatic dilators are most widely used, because they’re considered to be the safest. Doctors inject a catheter with a vessel in the stomach under X-ray examination. In stomach opening, the balloon is filled with air and pulled outside. It allows widening the clearance of esophagus. Esophagus walls can be torn in 1% of cases when an elastic dilator is used, while the risk increases to 6% when mechanic dilators are used. In 80% of cases dilation has positive effects and helps to suppress the symptoms of achalasia. However, if dilation does not give positive results, doctors can apply surgical treatment of achalasia.

The most widespread modern surgical way of esophagus treatment is bilateral esophagogastromyotomy. During this operation, muscle layers of distal segment of esophagus are cut along. Sometimes front esophagogastromyotomy is enough. About 90% of patients recover after this surgery. Unsatisfactory results are mostly connected with scars appearing down the road. This operation is preferable for treatment of advanced stages of achalasia in children.

Prognosis and prevention of cardia achalasia

Cardia achalasia progresses slowly. Untimely treatment of pathology can lead to bleeding, tearing of esophagus wall, development of mediastinitis, general weakness.Besides, cardia achalasia increases the risk of esophagus cancer.

After pneumocardiodilation, cardia achalasia can reoccur in 6-12 months. Prognosis is good, if there were no irreversible changes of esophagus motions, and surgical treatment was performed in time. Patients with cardia achalasia are recommended to be regularly examined by gastroenterologist, follow doctor’s instructions and go through regular diagnostics.

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Professor MD MD hc Guido Schumacher

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