Introduction

Diseases of the esophagus can generally be categorized into four types of abnormality: those of inadequate lower esophageal relaxation; those of uncoordinated contraction; those of hypercontrac-tion; and those of hypocontraction.1 Classic achalasia falls into this first category. Directly translated, achalasia means 'failure to relax', and describes a rare disease in which the lower esophageal sphincter (LES) remains constricted during swallowing and there is an absence of esophageal peristalsis. The first case of achalasia was reported in 1674 by Thomas Willis.2 His patient was otherwise healthy, but suffered from frequent vomiting and regurgitation of food. Successful palliation was achieved through postprandial passage of a whalebone with a sponge attached to the tip, which accomplished both tamping of the food through the LES, as well as some degree of forceful dilatation. However, it was Sir Cooper Perry in 1913 who first used the term 'achalasia' to describe this disease.3-5

The exact etiology of achalasia is unknown, but certain factors remain consistent, namely inflammation of the myenteric plexuses with varying degrees of ganglion cell loss. When symptomatology suggests achalasia, the diagnosis is confirmed through radiographic and manometric studies. In treating children with achalasia, numerous modalities have been employed with varying degrees of success, including drug treatment, forceful dilatation and surgical esophagomy-otomy. The purpose of this chapter is to provide an overview of this disease process, the tools with which to diagnose it and the available treatment options.

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