An 83-year-old man with a history of chronic obstructive pulmonary disease, congestive heart failure, and hypertension presents with a 6-week history of choking while eating. He reports difficulty swallowing both solids and liquids. The patient mentions that he has a chronic productive cough, but he is unsure if it contributes to his difficulty with swallowing. He denies having experienced any fever, chills, sore throat, pleuritic chest pain, or acute worsening of his baseline dyspnea.
On physical examination, the patient appears well and in no general distress. His blood pressure is noted as 135/89 mm Hg, with a heart rate of 65 beats/min. His oral temperature is 98.6°F and his oxygen saturation while breathing room air is 94% (baseline, given his pulmonary condition). He has normal breath sounds, his cardiac examination is normal, and he has normal bowel sounds in the setting of a soft, nontender abdomen.
A barium swallow study is performed (see Images 1-2) on suspicion of an esophageal pathology for his condition.
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What is the diagnosis?
The barium collection in the esophagus resembles a common kitchen tool.
Authors:
Brian Morse, MD,
Radiology Resident,
UT Southwestern/Dallas
VA Medical Center
Pramod Gupta, MD
Staff Radiologist
Dallas VA Medical Center,
Clinical Assistant Professor
University of Texas Southwestern,
Dallas, TX
eMedicine Editors:
Brady Pregerson, MD,
Department of Emergency Medicine,
Cedars-Sinai Medical Center,
Los Angeles, CA
Dept. of Emergency Medicine,
Tri-City Medical Center,
Oceanside, CA
Rick G. Kulkarni, MD
Assistant Professor,
Yale School of Medicine,
Section of Emergency Medicine,
Department of Surgery,
Attending Physician,
Medical Director,
Department of Emergency Services,
Yale-New Haven Hospital, Conn
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Diffuse esophageal spasm (DES): The barium swallow studies (see Images 1-2) show alternating areas of constricted and unconstricted areas in the lumen of the esophagus. This pattern, referred to as a corkscrew esophagus, is seen in DES and results from abnormal and uncoordinated tertiary contractions of the esophageal musculature. DES is an uncommon disorder of esophageal motility, and its exact etiology is unknown. The incidence of DES increases with age.
Affected individuals may present with recurrent chest pain, dysphagia with or without regurgitation, or both. They may also have globus, or a sensation of a foreign body in the throat. The pain associated with DES is generally retrosternal and may radiate to the back. It may be more severe than that associated with coronary artery disease, but mild pain is also reported. The pain can last minutes to hours. Although swallowing is not always impaired during episodes of DES, 30-60% of patients have dysphagia with esophageal spasticity. The dysphagia is often at its worst when the chest pain is at its most severe. Swallowing difficulties associated with DES are intermittent and may vary in timing and severity, but they are usually neither progressive enough nor severe enough to result in weight loss. Physical findings are usually normal.
The differential diagnosis for the chest pain associated with esophageal spasm is broad and includes acute coronary syndrome, gastroesophageal reflux disease, esophageal masses, achalasia, and nutcracker esophagus (ie, organized esophageal contractions that increase in amplitude by at least 2 standard deviations), among others.
Testing for DES may include electrocardiography, chest radiography, and a blood workup (including an analysis of cardiac enzymes) when other conditions must be ruled out. Further outpatient evaluation, especially of cardiac chest pain, may need to occur before definitive tests for DES (barium esophagography and/or esophageal manometry) are done.
On barium studies, DES is characterized by intermittently absent or weakened primary esophageal peristalsis, with nonperistaltic tertiary contractions that compartmentalize the esophagus and produce a classic corkscrew or rosary-bead appearance. DES and other esophageal motility disorders can further be characterized with manometry. The criterion standard, manometry shows periods of normal peristalsis interspersed with abnormal, simultaneous contractions after at least 10-30% of wet swallows.
The initial treatments of choice for DES are calcium channel blockers, which reduce the intensity of esophageal contractions, and nitrates, which reduce the intensity of any associated spasms. Antacids and other treatments for reflux esophagitis should be considered as well; however, responses to these modalities vary. Second-line treatments include botulinum toxin, which temporarily decreases the release of acetylcholine, and a tricyclic antidepressant (specifically imipramine), which seems to reduce the intensity of associated chest pain. If pharmacologic therapy is unsuccessful, aggressive interventions may be attempted. These may include esophageal dilation, which may temporarily help with symptoms, or a myotomy. Esophagectomy is reserved for the most refractory cases.
References
- Castell D: Diffuse Esophageal Spasm and Nutcracker Esophagus. UptoDate. August 15, 2005. Available at: www.UpToDate.com. Date Accessed: December 12, 2006.
- Gaumnitz E, Fayyad A: Esophageal Motility Disorders. eMedicine Journal [serial online]. 2006. Available at: www.emedicine.com/med/topic740.htm. Date Accessed: December 12, 2006.
- Goyal R: Diffuse esophageal spasm and related motor disorders. In: Kasper DL, Braunwald E, Fauci A, et al, eds. Harrison’s Principles of Internal Medicine. 16th ed. New York, NY: McGraw-Hill Professional; 2004. Available at: University of Texas Southwestern Medical Center campus library, http://www4.utsouthwestern.edu/library/ebooks/ebooksResults.cfm?alpha=H. Accessed: December 12, 2006.
- Thomson ABR, Birkedal C: Esophageal Spasm. eMedicine Journal [serial online]. 2006. Available at: www.emedicine.com/med/topic743.htm. Date Accessed: December 12, 2006.
