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Difficulty: Very hardGastroenterology and Hepatology

A 6565-year-old man presents to the clinic with a 33-month history of progressive difficulty swallowing both solids and liquids. He reports frequent regurgitation of undigested food, especially at night, and an unintended weight loss of 88 kg (17.617.6 lb) during this period. His medical history is significant for hypertension and a 4040-pack-year smoking history. Vital signs are within normal limits. Physical examination is unremarkable. A barium esophagram demonstrates esophageal dilation with symmetric, smooth tapering at the gastroesophageal junction. Upper gastrointestinal endoscopy shows retained food debris in the esophageal body but normal overlying mucosa; the endoscope passes easily into the stomach with gentle pressure. High-resolution esophageal manometry demonstrates absent peristalsis in the esophageal body and a high integrated relaxation pressure, indicating failure of the lower esophageal sphincter to relax upon swallowing. Which of the following is the most appropriate next step in management?

  1. Endoscopic ultrasound of the gastroesophageal junctionAnswer
  2. B
    Laparoscopic Heller myotomy
  3. C
    Initiation of intravenous piperacillin-tazobactam
  4. D
    24-hour ambulatory esophageal pH monitoring
  5. E
    Empiric trial of a high-dose proton pump inhibitor

Answer

Endoscopic ultrasound of the gastroesophageal junction
The patient's clinical and diagnostic findings (absent peristalsis, high integrated relaxation pressure, and a 'bird's beak' appearance) are highly indicative of an achalasia motility pattern. However, primary achalasia typically has a prolonged, indolent course in younger patients. This patient's advanced age (>60>60 years), short symptom duration (<6<6 months), profound weight loss, and significant smoking history strongly suggest pseudoachalasia. Pseudoachalasia is most commonly caused by a malignancy (e.g., gastric adenocarcinoma) infiltrating the gastroesophageal junction and myenteric plexus. Standard endoscopy often appears normal because the tumor may be submucosal or cause extrinsic compression. Therefore, the most appropriate next step is an endoscopic ultrasound (or computed tomography scan) to evaluate for a submucosal tumor before initiating definitive achalasia therapy.

Step-by-Step Solution

1
Analyze the clinical presentation and diagnostic findings.
The patient has dysphagia to solids and liquids, a 'bird's beak' appearance on esophagram, and manometry showing absent peristalsis and incomplete lower esophageal sphincter relaxation.
To establish the primary manometric diagnosis of an achalasia-like motility disorder.
2
Evaluate the patient's demographics and history for atypical features.
The patient has advanced age (>60>60 years), rapid onset of symptoms (<6<6 months), and severe weight loss.
Primary achalasia typically presents in younger patients with an indolent course, whereas these rapid-onset red flags strongly suggest a secondary cause, known as pseudoachalasia.
3
Determine the next best diagnostic step to evaluate for secondary causes.
An endoscopic ultrasound of the gastroesophageal junction is the most appropriate next step.
Standard endoscopy can easily miss submucosal infiltrating tumors (like gastric adenocarcinoma) that cause pseudoachalasia. Endoscopic ultrasound is highly sensitive for detecting these lesions.

Key Concept

Patients presenting with an achalasia motility pattern who have clinical red flags (age >60>60 years, rapid symptom onset <6<6 months, and profound weight loss) must be evaluated for pseudoachalasia (malignancy) using endoscopic ultrasound or computed tomography before initiating definitive therapy.
Estimated Time:2m 30s
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Topics

Gastroenterology and Hepatology

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