Chapter 26

In 1914, Ernest Heller first described the double-cardiomyotomy technique, one anterior and one posterior along the gastroesophageal junction (GEJ), for the surgical treatment of achalasia.1 This operation was modified subsequently such that only an anterior myotomy is currently performed. This operation has yielded excellent results, with 90–95% of patients receiving durable relief of dysphagia.2–6 At the University of Washington, we converted from a thoracoscopic approach to a laparoscopic Heller myotomy in 1994. Along with several major centers, we now consider laparoscopic esophageal myotomy to be an excellent first-choice therapy because of the low morbidity of minimally invasive techniques and the high levels of success after myotomy.3,6–8 Moreover, surgery provides the most dependable long-term relief of dysphagia, permitting accurate division of the muscle fibers of the lower esophageal sphincter (LES) rather than blind disruption, as with dilation.2

Achalasia is an esophageal motility disorder characterized by aperistalsis of the esophageal body combined with lack of LES relaxation. The most common primary esophageal motility disorder, achalasia, is still relatively uncommon, with a reported incidence of 0.5–1 per 100,000 people in North America. First described as cardiospasm over 300 years ago, the name achalasia was coined by Lendrum in 1937, literally meaning “failure to relax.”9,10

The disease manifests with progressive dysphagia, first to solids and then to liquids, in individuals between the ages of 20 and 50 years (but often both earlier and later in life). No gender predilection exists. Ineffective relaxation of the LES and loss of esophageal peristalsis lead to impaired emptying and gradual esophageal dilatation, resulting in severe dysphagia. Patients also may complain of regurgitation (commonly of undigested food), cough, aspiration, wheezing, and choking, often made worse by assuming the supine position for sleep, when esophageal contents flow back into the airway. Stress or cold liquids may exacerbate symptoms. Chest pain is reported commonly, although it is of unclear etiology because most patients have no peristalsis. Distention and uncoordinated peristalsis are often postulated as a cause. A subset of patients has simultaneous contractions of normal or near-normal amplitude on manometry, a variation often called vigorous achalasia. These simultaneous contractions are postulated as one cause of chest pain, similar to other hypercontractile esophageal motility disorders. Overdistention of the esophagus may also contribute to pain. Heartburn may occur but is usually the result of fermentation of unevacuated food in the esophagus and not gastroesophageal reflux.6 Delay in treatment, however, is usually a result of misdiagnosis, where achalasia is mistaken for gastroesophageal reflux disease (GERD). Recurrent aspiration pneumonia is another presenting symptom of achalasia that can sidetrack clinicians and requires exclusion of airway lesions or malignancy.

Mild weight loss is a late manifestation of the disease. Rapid or significant drop in weight of more than 10 lb (4 kg) should alert clinicians to the possibility of an esophageal malignancy that has caused obstruction. This entity is often referred to as pseudoachalasia. If ...

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