Retroflexed Image of Narrowed Gastroesophageal Junction

A "tight" gastroesophageal junction retroflexed image Sir Thomas Willis is credited with the first report of a patient with achalasia in 1674. Von Mikulicz in 1882 and Einhorn in 1888 hypothesized that the disease was due to the absence of opening of the cardia or "cardiospasm." Over the past three centuries, achalasia has emerged as an important model by which to understand the pathophysiology and therapy of motility disorders emanating from a defect in the enteric nervous system. It is the most extensively studied and readily treatable gastrointestinal motor disorder. This review discusses current concepts in achalasia with an emphasis on the pathophysiology and etiology of the disease. Specific secondary etiologies of achalasia are discussed that provide insight into mechanisms responsible for the neurodegeneration that characterizes the disorder. Diffuse esophageal spasm is also discussed, although there is a paucity of data regarding this condition.