MKSAP Quiz: progressive dysphagia for solids and liquids
MKSAP Quiz: progressive dysphagia for solids and liquids
A 56-year-old woman is evaluated for a 3-year history of progressive dysphagia for solids and liquids; she has had a 6.8-kg (15-lb) weight loss during this time. The dysphagia was initially intermittent, but recently swallowing almost all food or drink causes a feeling of chest tightness and discomfort with increasingly frequent regurgitation of undigested food. The dysphagia is sometimes alleviated by standing upright. Her medical history is significant only for hypertension, and her medications include lisinopril and a multivitamin.
On physical examination, the patient appears uncomfortable and restless; she is thin but does not have thenar wasting. She is afebrile; the blood pressure is 142/92 mm Hg, the pulse rate is 96/min, and the respiration rate is 22/min. The BMI is 23.
Barium esophagography shows a dilated esophagus with an air/fluid level and tapered narrowing of the distal esophagus. Esophagogastroduodenoscopy shows a dilated esophagus with retained food and a tight lower esophageal sphincter, which allowed passage of the endoscope.
Which of the following is the most likely diagnosis?
A. Achalasia
B. Diffuse esophageal spasm
C. Peptic stricture
D. Scleroderma esophagus
MKSAP Answer and Critique
The correct answer is A) Achalasia. This item is available to MKSAP 15 subscribers as item 15 in the Gastroenterology and Hepatology section.
The patient's history is typical for achalasia, an uncommon but important primary motility disorder of the esophagus. The barium study and endoscopic appearance described are typical for achalasia, but the diagnosis is confirmed manometrically with esophageal motility studies. The manometric diagnosis of achalasia usually includes an elevated lower esophageal sphincter pressure, failure of the lower esophageal sphincter to relax with swallowing, and diminished or absent peristalsis of the esophageal body.
Diffuse esophageal spasm typically presents with noncardiac chest pain. The diagnosis of diffuse esophageal spasm is made manometrically by the finding of more than 20% of swallows having simultaneous contractions in the distal esophagus. Peptic stricture would present with dysphagia, but would typically show a longer, non-tapered stricture on barium esophagography. Furthermore, peptic strictures seldom present with megaesophagus, as seen in this patient. Scleroderma esophagus leads to loss of esophageal motility and often severe reflux or distal esophageal strictures, not a dilated esophagus.
Treatment of achalasia is usually pneumatic dilatation of the esophagus or surgical myomectomy, the latter of which can be done laparoscopically. Pneumatic dilatation, even in experienced hands, is associated with a 5% to 10% risk of esophageal perforation. Botulinum toxin injection can afford relief of achalasia in patients who are not considered candidates for endoscopic or surgical interventions.
Key Point
- Achalasia is a primary motility disorder of the esophagus and requires manometric diagnosis.