Idiopathic achalasia is a rare esophageal motility disorder characterized by loss of esophageal peristalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition.
The disease can occur at any age, (similar in men and women), but is usually diagnosed between 25 and 60 years.
It is characterized predominantly by dysphagia to solids and liquids (difficulty to swallow), bland regurgitation, and chest pain.
Weight loss (usually between 5 to 10 kg) is present in most but not in all patients. Heartburn occurs in 27%–42% of achalasia patients. Etiology is unknown. Some familial cases have been reported
The diagnosis is based on history of the disease, radiography (barium esophagogram), and esophageal motility testing (esophageal manometry).
Endoscopic examination is important to rule out cancer as the cause of achalasia.
Treatment is strictly palliative. Current medical and surgical therapeutic options (pneumatic dilation, surgical myotomy, and pharmacologic agents) aimed at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Excellent palliation is available in over 90% of patients.
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