Esophageal motility disorders: Current concepts of pathogenesis and treatment

Current concepts of esophageal motility disorders are summarized. Primary d ata sources were located via MEDLINE or cross-citation. No attempt was made to be comprehensive or inclusive of the literature because fewer than 10% of citations are discussed. Instead, emphasis was placed on new development s in diagnosis, therapeutics and practice patterns. Controlled therapeutic trials and pathophysiological observations are emphasized. Achalasia is a r are disease of failed lower sphincter relaxation and aperistalsis. Diffuse esophageal spasm (DES), an equally rare disease, is defined by nonpropagate d esophageal contractions. Nonspecific motility disorders, including nutcra cker esophagus and hypertensive lower esophageal sphincter, are identified only by manometry and are 10 times as prevalent. Neuromuscular pathology is evident only with achalasia (myenteric plexus neuron destruction). Pharmac ological therapies have limited efficacy with achalasia, more limited effic acy with DES and no efficacy with the nonspecific motility disorders. More efficacious therapies for the nonspecific disorders are directed at associa ted reflux disease or psychiatric disorders. Pneumatic dilation is effectiv e therapy for achalasia in 72% of instances but frequently requires repeat dilation and is complicated by a 3% perforation rate. Surgical myotomy is e ffective in 88% of patients with achalasia; morbidity from thoracotomy has been the major limitation but has been sharply reduced with a laparoscopic approach. In conclusion, although it has been suggested that esophageal mot ility disorders are distinct clinical entities, critical review of the lite rature supports this only in the case of achalasia, a disease of well defin ed pathophysiology, functional disturbance and therapies. This clarity dimi nishes progressively for DES and nonspecific esophageal motility disorders.