This patient's presentation is consistent with diffuse esophageal spasm, which occurs due to uncoordinated contractions of the esophagus. Esophageal manometry (an esophageal motility study that measures pressure in the esophagus) would show long duration, large amplitude, periodic, non-peristaltic contractions.
Diffuse esophageal spasm (DES) is thought to arise from acid reflux and irritation acid reflux resulting in a spasm of the esophagus. This condition is characterized by substernal chest pain, dysphagia, and a manometric pattern of frequent simultaneous contractions with intermittently normal peristalsis. Upper GI barium swallow shows a classic "corkscrew" pattern as seen in image A. The condition can be treated with reflux medications (reduced acid triggers less esophageal spasm), nitrates, and calcium channel blockers (both inhibit smooth muscle contraction).
Fulp et al. reviews causes of esophageal chest pain and reports that GERD is the most common cause of esophageal chest pain. While not the most common, achalasia, diffuse esophageal spasm, nutcracker esophagus, hypertensive lower esophageal sphincter and nonspecific motility disorders should also be considered in the differential diagnosis.
Chen et al. reviews radiographic and manometric findings in diffuse esophageal spasm. Incomplete or absent primary peristalsis was observed on radiographs in 76% of patients and mild to severe tertiary activity was seen in 71% of patients. The authors conclude that most patients with DES show abnormal esophageal motility on radiographs, although the findings were nonspecific and required clinical and manometric correlation.
Answer 1: An abnormal ECG with chest pain is suspicious for a myocardial infarction.
Answer 2: An abnormal coronary angiogram with chest pain is suspicious for a myocardial infarction.
Answer 3: An abnormal esophageal biopsy with chest pain is suspicious for GERD with metaplasia or esophageal malignancy.
Answer 4: Abnormal pulmonary function tests would be seen in obstructive (low FEV1:FEV ratio) or restrictive (normal/high FEV1:FEV ratio) lung disease, not diffuse esophageal spasm.
Fulp SR, Richter JE. Esophageal chest pain. Am Fam Physician. 1989 Sep;40(3):101-16. Review.
PMID:2672745 (Link to Abstract)
Chen YM, Ott DJ, Hewson EG, Richter JE, Wu WC, Gelfand DW, Castell DO. Diffuse esophageal spasm: radiographic and manometric correlation. Radiology. 1989 Mar;170(3 Pt 1):807-10.
PMID:2916033 (Link to Abstract)
USMLE World Step 1 QBank Question #280. Copyright © USMLEWorld, LLC 2012