Abstract
Idiopathic achalasia is a primary esophageal motor disorder characterized by loss of esophageal peristalsis and insufficient lower esophageal sphincter relaxation in response to deglutition. Patients with achalasia commonly complain of dysphagia to solids and liquids, bland regurgitation often unresponsive to an adequate trial of proton pump inhibitor, and chest pain. Weight loss is present in many, but not all patients. Although the precise etiology is unknown, it is often thought to be either autoimmune, viral immune, or neurodegenerative. The diagnosis is based on history of the disease, barium esophagogram, and esophageal motility testing. Endoscopic assessment of the gastroesophageal junction and gastric cardia is necessary to rule out malignancy. Newer diagnostic modalities such as high resolution manometry help in predicting treatment response in achalasia based on esophageal pressure topography patterns identifying three phenotypes of achalasia (I-III) and outcome studies suggest better treatment response with types I and II compared to type III. Although achalasia cannot be permanently cured, excellent outcomes are achieved in over 90 % of patients. Current medical and surgical therapeutic options (pneumatic dilation, endoscopic and surgical myotomy, and pharmacologic agents) aim at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Either graded pneumatic dilatation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy guided by patient age, gender, preference, and local institutional expertise. The prognosis in achalasia patients is excellent. Most patients who are appropriately treated have a normal life expectancy but the disease does recur and the patient may need intermittent treatment.
Highlights
Idiopathic achalasia is a primary esophageal motor disorder characterized by loss of esophageal peristalsis and insufficient lower esophageal sphincter relaxation in response to deglutition
Dysphagia can occur in patients with other esophageal motility disorders, this symptom is most characteristic of achalasia and strongly suggests the diagnosis
Regurgitation not responding to adequate proton pump inhibitor (PPI) therapy and weight loss can be seen in 30 % to 90 % of patients
Summary
Number of patients % LES pressure decreased Remission rate Remission rate Remission rate enrolled post treatment at 1 months at 6 months at 12 months. Surgical myotomy was originally performed via thoracotomy with good to excellent results in 60-94 % of patients followed for 1–36 years [11] This intervention evolved to be performed with a laparotomy approach, a thoracoscopic approach, and via laparoscopy that has fallen in favor due to superior visualization of the gastroesophageal junction, the ability to add an antireflux procedure, decreased morbidity, shorter hospital stay, and faster recovery [103]. Multiple recent randomized controlled trials comparing Heller myotomy in conjunction with a Dor versus Toupet fundoplication showed significant improvement in both dysphagia and regurgitation symptoms regardless of the type of partial fundoplication with dramatic improvements in Eckardt scores [108, 109]. Patients undergoing Toupet fundoplication did have significantly better relative improvements in the EORTC QLQ-OES18 (functional scale), but otherwise
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