Achalasia

What is achalasia?

Achalasia is a disorder of the primary motility of the esophagus characterized by the loss of peristalsis and increased tonicity plus failure of the lower esophageal sphincter (LES) relaxation in response to swallowing.

Epidemiology

  • It has a worldwide prevalence of 6 to 10 per 100,000 inhabitants per year.
  • Average age of diagnosis is 49 years. Although it may occur in the first decade of life.
  • May have a genetic component with an autosomal recessive pattern.
  • It is associated with esophageal cancer and other esophageal complications, however longitudinal studies indicate that it does not affect life expectancy.

Etiology and Pathophysiology

  • Progressive inflammation and selective loss of inhibitory myelin neurons in the Auerba plexus, affecting the normal production of intestinal active peptide (VIP) and Nitric Oxide (ON).
  • Failure in the relaxation of the LES.
  • Obstruction at the level of gastroesophageal junction (CGU) and proximal esophageal dilatation.
  • 25% of patients with achalasia may have a hypertensive EEI.
  • The close similarity with Chagas disease indicates that there may be an infectious cause in achalasia. The association with HSV-1 and HSV-2, polio and papilloma virus is not yet concluded.
  • Certain class of CMH II antigen, expressed in autoimmune endocrinopathies and Rheumatoid Artirtis, such as HLA-DQw1, HLA-DQB1 and HLA-DRB1 have been associated with achalasia.

Differential Diagnostics

The main differential diagnosis of primary achalasia is the Chagas disease that is caused by Trypanosoma Cruzi. The main differences between Chagas disease and primary Acalasias are mentioned below.

  • In Chagas, there is denervation of the inhibitory and exudatory myenteric nerves in primary achalasia only of the inhibitory nerves.
  • In Chagas, there is hyposensitivity of IAS to gastrin and in primary achalasia, hypersensitivity.
  • In Chagas the pressure of the LES is low, in contrast to that of Achalasia (except in 25% of cases).

Symptom

  • Cardinal symptom: Dysphagia
  • Regurgitation: 75%
  • Heartburn: 50%
  • Thoracic Pain
  • Loss of weight 35%
  • Fear of Eating
  • Chronic bronchoaspiration (33%)

Diagnosis

  • Contrast esophagogram, narrowing of the distal third of the esophagus (bird’s beak), hidroaereo level, slow emptying of the barium and esophageal dilation.
  • Manometry, Non-propulsive esophageal contraindications IHT hypertensive (50%) (> 24mmHg), 25% IEM normotensive (14-24mmHg), 25% IAS Hypotensive (<14mmHg).
  • Endoscopy of upper digestive tract, Exclusion of cancer, narrowing, scleroderma, marked resistance of the CGU to the advance of the endoscope.

Secondary Achalasia

  • Cancer (Malignant Achalasia) 75% of cases due to infiltration of the median plexus
  • Post-vagotomy states
  • Amyloidosis, Sarcoidosis
  • Paraneoplastic neuropathy (Prostate cancer, Pancreas)

Complications

  • Esophagitis
  • Epiphrenic diverticulum (secondary diverticulum of pulsion) due to increased intra-esophageal pressure
  • Chronic microaspiration wing airway 33%
  • Squamous cell carcinoma of the esophagus

Treatment

  • Laparoscopic esophageal myotomy (Heller procedure) + antireflux procedure
  • 38-60% of patients undergoing Heller’s procedure without antireflux surgery develop GER
  • There is no clear documentation of relationship in patients undergoing the procedure of Heller and Barrett’s Esophagus or Adenocarcinoma.

Leave a Reply