ABSTRACT

Gastrointestinal motility disorders encompass a wide array of signs and symptoms that can occur anywhere throughout the luminal gastrointestinal tract (Table 33.1). Dysphagia for solids and liquids, regurgitation of undigested food, respiratory complications (nocturnal cough and aspiration), chest pain and weight loss are the main symptoms of achalasia, a rare motor disorder of the oesophagus and lower oesophageal sphincter (LES). Since the šrst description of achalasia by Sir Thomas Willis in 1674, several theories on the aetiology and pathophysiology have been reported (Boeckxstaens 2007; Farrokhi and Vaezi 2007; Park and Vaezi 2005). Idiopathic (primary) achalasia, most common anywhere but in South America, is a neuromuscular disorder characterized by degenerative changes of the myenteric plexus leading to a loss of peristaltic contractions and impaired LES relaxation in response to swallowing. Primary achalasia is a quite rare disease with an incidence of approximately 1/100,000 and a prevalence rate of 10/100,000. Secondary achalasia shares clinical features with primary achalasia, but there is an identišable cause. Worldwide, the most common cause of secondary achalasia is Trypanosoma cruzi infection, found in Central and South America. When features of achalasia are caused by malignancy or by other inšltrative

33.1 Introduction .......................................................................................................................... 391 33.2 Pathophysiology .................................................................................................................... 392 33.3 Clinical Picture and Diagnosis ............................................................................................. 393 33.4 Treatment Options ................................................................................................................ 394

33.4.1 Pharmacologic Treatments ....................................................................................... 394 33.4.2 Endoscopic Procedures ............................................................................................. 394 33.4.3 Surgical Treatment .................................................................................................... 395

33.5 Applications to Other Areas of Palliative Care .................................................................... 395 33.6 Guidelines and Practical Methods and Techniques .............................................................. 396 33.7 Nutritional Aspects of Achalasia .......................................................................................... 396 Ethical Issues ................................................................................................................................. 398 Summary Points ............................................................................................................................. 399 List of Abbreviations ...................................................................................................................... 399 References ...................................................................................................................................... 399

diseases such as amyloidosis, the term pseudoachalasia is used. Complications of surgery involving the gastroesophageal junction, such as fundoplication, gastric banding and vagal injury, may lead to pseudoachalasia.