Abstract
Idiopathic achalasia is a primary esophageal motor disorder characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition. It is a rare disease with an annual incidence of approximately 1/100,000 and a prevalence rate of 1/10,000. The disease can occur at any age, with a similar rate in men and women, but is usually diagnosed between 25 and 60 years. It is characterized predominantly by dysphagia to solids and liquids, 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, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor. Association of achalasia with viral infections and auto-antibodies against myenteric plexus has been reported, but the causal relationship remains unclear. 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 malignancy 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. Although it cannot be permanently cured, excellent palliation is available in over 90% of patients.
Highlights
Definition and epidemiology Idiopathic achalasia is a primary esophageal motor disorder of unknown etiology characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition [14]
Our study, which was one of the largest studies comparing the outcome of two therapies, suggested that both therapies are effective at 1 month, but pneumatic dilation (PD) results in significantly better symptom improvement at 12 months compared with Botulinum toxin (BT) (70% vs. 32% respectively) [2]
Studies suggest that laparoscopic myotomy is not a cost-effective therapy as the initial cost is too high [73]. It is an effective treatment modality in patients with achalasia who have failed to respond to PD, as the 10-year remission rate in these patients following myotomy is shown to be 77% comparing to 72% and 45% in patients "successfully" treated with a single PD and patients undergoing several dilations respectively [74]
Summary
Definition and epidemiology Idiopathic (primary) achalasia is a primary esophageal motor disorder of unknown etiology characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition [14]. Current medical and surgical therapeutic options proposed for this well-recognized motor disorder of esophagus are aimed at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids [8,9,10,11,12] These therapeutic options include pneumatic dilation, surgical myotomy, and less effectively pharmacologic agents [11,13]. Timed barium esophagogram Barium swallow was initially used by Vantrappen et al [49] in achalasia patients to determine the cause of persistent symptoms after treatment with pneumatic dilation This led to suggestion that barium esophagogram with fluoroscopy is the single best diagnostic study for achalasia.
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