Etiology and Clinical FeaturesTop
The etiology of distal esophageal spasm (DES) and hypercontractile (jackhammer) esophagus is unknown. Hypercontractile esophagus may be a primary disorder of excessive excitation of the smooth muscle. Both disorders 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.
DiagnosisTop
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, while hypercontractile esophagus is characterized by high pressure but normally sequential contractions in the smooth muscles of the esophagus.
TreatmentTop
The most effective treatment of DES and jackhammer esophagus is not well defined. Pharmacotherapy includes calcium channel blockers (nifedipine and diltiazem) or nitrates (nitroglycerin, isosorbide dinitrate) if patients have no gastroesophageal reflux disease (GERD) or if it is well controlled. Esophageal dysmotility may be induced by acid reflux, which is why the treatment of GERD may help alleviate symptoms. Many patients require antisecretory therapy with proton pump inhibitors. For those who have either no GERD or a well-controlled GERD, treatment options include an antispasmodic agent (eg, hyoscyamine 0.25 mg tid as needed), a nitrate (eg, isosorbide dinitrate 5 to 10 mg bid to tid as needed), a calcium channel blocker (eg, nifedipine, extended release, 30 mg/d), or phosphodiesterase inhibitor (sildenafil 25 to 50 mg/d). Advantages of surgical over medical treatment have not been proven.