Review Question - QID 107837

This patient is suffering from achalasia. You would expect to see absent peristaltic waves in the lower esophagus and failure of the lower esophageal sphincter (LES) to relax with swallowing.

This patient is suffering from achalasia, an esophageal motility disorder characterized by absent peristaltic activity in the distal esophagus and failure of relaxation of the LES. The underlying cause of achalasia is thought to be either autoimmune, viral, or neurodegenerative with the end result being loss of innervation of the lower esophageal smooth muscle. Patients present with dysphagia to liquids (85%) and solids (91%), heartburn, chest pain, regurgitation of food, and difficulty belching. Workup includes a barium swallow, esophageal manometry, and endoscopic examination.

The American College of Gastroenterology released a consensus statement on the diagnosis of achalasia. While barium swallow is often the first diagnostic test ordered in the workup of dysphagia, and EGD is often used to rule out a stricture (i.e. one caused by cancer), these tests are not sufficient to make the diagnosis. In order to make a formal diagnosis of achalasia, esophageal manometry must be performed. Classic findings on manometry included increased LES tone, failure of LES relaxation on deglutition, and aperistalsis at the distal esophagus.

Sandler et al. evaluates the incidence of esophageal cancer in patients who suffer from achalasia. Findings of their cohort study indicate that patients with achalasia have a 16 fold increase in their risk of developing esophageal cancer when compared to the general population. Despite this increase in risk, there is no evidence that routine screening in patients with achalasia is beneficial due to the low overall incidence of esophageal cancer in achalasia patients.

Figure A shows the classic "bird's-beak" deformity characteristic of patients with achalasia, but only present in 2/3 of patients. Illustration A shows a Zenker's diverticulum. Illustration B shows a barium swallow of a patient with an obstructive mass. Illustration C shows a barium swallow of a patient with diffuse esophageal spasm. Illustration D shows the barium swallow of a patient with systemic sclerosis (scleroderma).

Incorrect Answers:
Answer 1: These manometric findings are consistent with a diagnosis of Zenker's diverticulum causing dysphagia.
Answer 2: These manometric findings are consistent with a stricture or mechanical obstruction VS hypertensive lower esophageal sphincter, such as obstruction by a lower esophageal cancer.
Answer 3: These manometric findings are consistent with a diagnosis of scleroderma in which the esophagus becomes infiltrated with scar tissue.
Answer 4: These manometric findings are consistent with a diagnosis of diffuse esophageal spasm or "nutcracker esophagus" in which there is no progression of esophageal peristalsis, but rather the contractions occur at the same time.