In 1914, Ernest Heller described the first cardiomyotomy for the treatment of achalasia.1 He described an anterior and posterior myotomy along the gastroesophageal junction (GEJ) using a thoracoabdominal incision. The operation was subsequently modified by Groenveldt and Zaaijer to include only the anterior myotomy. Owing to the morbidity of the open approach, this operation was performed primarily in patients who failed medical 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.2-5 At the University of Washington, we converted from a thoracoscopic approach to a laparoscopic Heller myotomy in 1994, and along with most centers, we now consider laparoscopic esophageal myotomy to be an excellent first-line therapy because of the low morbidity, durability, and the high levels of success after minimally invasive esophagocardiomyotomy.3,6–9
Clinical Characteristics of Achalasia
Achalasia is characterized by aperistalsis of the mid-to-distal esophageal body combined with lack of LES relaxation. Although it is the most common primary esophageal motility disorder, it is rare. The reported incidence in North America is 0.5 to 1 per 100,000 people. Achalasia was first described as cardiospasm in the 17th century by Sir Thomas Willis. The name achalasia, meaning “failure to relax,” was coined by Lendrum in 1937.10
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 lead 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 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. A subset of patients with simultaneous contractions of normal or near-normal amplitude on manometry, are considered to have a variation of Achalasia termed vigorous achalasia.11 In reality, achalasia may be a heterogeneous collection of disorders or subtypes as has been recently described by Pandolfino et al.12 As with other hypercontractile esophageal motility disorders, simultaneous contractions are postulated as one cause of chest pain, but this seems unlikely for most patients with achalasia who demonstrate little even simultaneous contractile ...