Etiology and Clinical FeaturesTop
The etiology of distal esophageal spasm (DES) and hypercontractile (jackhammer) esophagus is unknown. They may occur at any age but usually develop in patients aged >40 years.
Clinical manifestations include noncardiac chest pain, usually retrosternal, and dysphagia to solid foods and liquids in the majority of patients. The pain may occur immediately after a meal but sometimes is independent of food intake. Dysphagia may be severe and may lead to malnutrition.
Diagnosis is based on high-resolution esophageal manometry after other esophageal disorders have been excluded on the basis of upper gastrointestinal endoscopy with biopsy or barium esophagram. DES is characterized by spastic or premature contractions in the distal esophagus. Hypercontractile esophagus is characterized by high pressure but normally sequential contractions in the smooth muscles of the esophagus.
The most effective treatment of DES and jackhammer esophagus is not well defined. Pharmacologic treatment includes calcium channel blockers (nifedipine and diltiazem) or nitrates (nitroglycerin, isosorbide dinitrate) if patients have no gastroesophageal reflux disease or if it is well controlled. Many patients require antisecretory therapy with proton pump inhibitors. Advantages of surgical over medical treatment have not been proven.