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Surg Clin N Am 85 (2005) 483–493

Todd A. Woltman, MD, PhD, Carlos A. Pellegrini, MD,
Brant K. Oelschlager, MD*
Department of Surgery, University of Washington, 1959 NE Pacific Street, Box 356410, Seattle, WA 98195-6410, USA

Achalasia is a primary esophageal motor disorder of unknown etiology. It is an uncommon ailment, with a reported incidence of 0.5 to 1 per 100,000 in theUnited States. Achalasia affects both sexes equally, typically presenting between the ages of 20 and 50, though it can occur at all ages. Ineffective relaxation of the lower esophageal sphincter (LES) combined with loss of esophageal peristalsis leads to impaired emptying and gradual esophageal dilation. Dysphagia is the cardinal feature of achalasia, accompanied by varying degrees of aspiration,weight loss, and pain. The anatomic defect appears to be a decrease or loss of inhibitory nonadrenergic, noncholinergic ganglion cells in the esophageal myenteric plexus. Histological analysis of esophagi resected from patients who had end-stage achalasia demonstrates myenteric inflammation and progressive depletion of ganglion cells and subsequent neural fibrosis [1]. There is also a significantreduction in the synthesis of nitric oxide and Vasoactive Intestinal Polypeptide (VIP), the most important mediators of relaxation in the lower esophageal sphincter. Macroscopically, there may often be thickening of the circular layer of the distal esophagus. Achalasia also carries a slightly increased risk of cancer, squamous cell carcinoma in particular.

Patient presentation Most patients who haveachalasia present with progressive dysphagia to solids and liquids, although symptoms may be subtle and nonspecific early in its course. The mean duration of symptoms before presentation is 2 years, and the diagnosis often takes much longer because the symptoms are often attributed to gastroesopahageal reflux disease (GERD) or other disorders.
* Corresponding author. E-mail (B.K. Oelschlager). 0039-6109/05/$ - see front matter Ó 2005 Elsevier Inc. All rights reserved. doi:10.1016/j.suc.2005.01.002



Initially, the patient may complain of the sensation of a retrosternal ‘‘sticking’’ of foodstuffs. Stress or cold liquids may exacerbate dysphagia. Patients may regurgitate undigested food, especially after meals orwhen lying supine. Patients may stand after eating or raise their arms over their head to enlist gravity and to increase the intrathoracic pressure in an attempt to aid esophageal emptying. If unable to force food into the stomach by the ingestion of liquids or other means, spontaneous or forced regurgitation are often employed to evacuate the esophagus. Occasionally, physicians even confuse thedisease with an eating disorder. As a result of regurgitation, aspiration of esophageal contents may lead to pulmonary disease. In fact, up to 10% of patients who have achalasia experience significant bronchopulmonary complications [2]. Many patients express a sensation of heartburn, explaining why many are initially diagnosed with GERD. Though patients who have achalasia may experiencegastroesophageal (GE) reflux [3], more often heartburn is secondary to fermentation of retained undigested food in the esophagus. Chest pain, clearly distinguishable from heartburn, occurs in 30% to 50% of patients. The etiology of this pain is unclear, and is unpredictably relieved by esophageal myotomy and other treatments. Weight loss is variable and tends to be insidious. The magnitude of weight loss tendsto correlate with the severity of the underlying disease. Rapid onset of symptoms (6 months), advanced age ([50 years), or significant weight loss ([15 lbs) should raise suspicion for pseudoachalasia, usually secondary to malignancy or extaluminal obstruction. In these cases, a thorough work-up with a CT scan or endoscopic ultrasound must be performed before further therapy is considered....
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