In 1914, Ernest Heller described the first cardiomyotomy for the treatment of achalasia.1 Initially performed open, this morbid operation was performed primarily in patients who failed medical and endoscopic management. It was not until the early 1990s with the advent of minimally invasive techniques that minimally invasive esophagocardiomyotomy became a viable first-line therapy. This operation has yielded excellent results, with 90% to 95% of patients receiving durable relief of dysphagia for patients with achalasia.2–4 This success led to use of myotomy for other esophageal motility disorders with variable, but inferior, success. With the ability to better classify the esophageal motility disorders with high-resolution manometry, we are now able to apply and form new opinions in the management of these rare disorders.
Achalasia is characterized by aperistalsis of the mid-to-distal esophageal body combined with lack of lower esophageal sphincter (LES) relaxation. Although it is the most common primary esophageal motility disorder, it is rare, occurring in 1 person in 100,000.5 Achalasia manifests most commonly with progressive dysphagia, starting with solids and progressing to liquids. The diagnosis usually is made between the ages of 20 and 50 years without a gender predilection. Ineffective relaxation of the LES and loss of esophageal peristalsis leads to impaired emptying and gradual esophageal dilatation, resulting in severe dysphagia. Patients may use various maneuvers to attempt to clear the esophagus, including drinking liquids, standing after swallowing, walking around during meals, raising hands over the head, and extending or flexing the neck. Patients also may complain of regurgitation of undigested food, cough, aspiration, wheezing, and choking. These symptoms often are made worse in the recumbent position, when esophageal contents flow back into the airway, and in some can lead to recurrent aspiration pneumonia. Chest pain is commonly reported, and it is postulated to be a result of esophageal overdistention and uncoordinated peristalsis. Stress or cold liquids can also exacerbate these symptoms in some. Heartburn may occur but is usually the result of fermentation of unevacuated food in the esophagus and not gastroesophageal reflux disease (GERD). Initial misdiagnosis as GERD or the presence of aspiration pneumonia can lead to a delay in treatment. Mild weight loss is occasionally a manifestation of the disease. Rapid or significant drop in weight of more than 10 lb (4 kg) along with advanced age (>60 years) or rapid onset of symptoms (<6 months) should alert clinicians to the possibility of an esophageal malignancy that has caused obstruction (and with it all the other manifestations of achalasia). This entity is often referred to as pseudoachalasia. If any diagnostic doubt exists, CT or endoscopic ultrasound may be used to confirm the presence of obstruction secondary to tumor.
Esophageal manometry is the standard for diagnosing achalasia (Fig. 34-1...