A 62-year-old man comes to the office due to difficulty swallowing solids and liquids. His symptoms have progressively worsened over the past 3 months. The patient also has occasional regurgitation of undigested food and a nighttime cough that disturbs his sleep. He has never had similar symptoms. The patient has lost 4.5 kg (10 lb) during this period but has no other medical conditions and takes no medications. He has a 20-pack-year history but quit smoking 10 years ago. He drinks wine on the weekends. Vital signs are normal, and physical examination is unremarkable. Chest x-ray reveals a widened mediastinum, and barium swallow study shows a dilated esophagus with tapering of the distal esophagus. Which of the following is the best next step in management of this patient?
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This patient has progressively worsening dysphagia with solids and liquids, and barium swallow reveals a dilated proximal esophagus with distal tapering. This presentation is consistent with achalasia. Achalasia is an esophageal motility disorder that can be idiopathic (ie, primary achalaisa) or due to a mass (eg, esophageal cancer) obstructing the lower esophageal sphincter (LES) (ie, pseudoachalasia).
The patient has several risk factors suggesting pseudoachalasia due to a malignant cause, including:
Tobacco use, which increases the risk for esophageal cancer
Alarm features, including older age (>60), rapid symptom onset (<6 months), and significant weight loss; in contrast, idiopathic achalasia occurs in younger patients (eg, age 20-50), has an insidious onset, and is associated with mild weight loss
Widened mediastinum on x-ray, which can be caused by tumor metastasis (eg, mediastinal lymph nodes) or local tumor invasion
In addition to esophageal manometry (required to confirm the diagnosis), patients with suspected achalasia should undergo upper gastrointestinal endoscopy due to the potential for malignancy. In idiopathic achalasia, endoscopic findings typically include retained undigested food, normal-appearing esophageal mucosa, and a dilated esophagus; the LES is closed but can be easily traversed with the scope. In contrast, mucosal lesions are typically seen in malignancy, and the LES is difficult to traverse due to obstruction by the tumor.
(Choice A) CT scan of the chest is appropriate for staging if endoscopy shows a malignancy or if endoscopy is negative despite a high concern for malignancy. However, upper gastrointestinal endoscopy should be performed first.
(Choices C and F) Esophageal pH monitoring is typically performed to confirm the diagnosis of gastroesophageal reflux disease (GERD) in patients with symptoms refractory to proton pump inhibitor therapy. GERD does not cause weight loss, significant dysphagia, or tapered narrowing of the distal esophagus.
(Choices D and E) Myotomy is a recommended treatment for idiopathic achalasia for patients at low surgical risk. In contrast, calcium channel blocker therapy is much less effective and is considered for patients who want to avoid invasive procedures. These treatments should be considered only after malignancy is excluded and achalasia is confirmed with manometry.
Educational objective:
Pseudoachalasia results from a malignancy near the lower esophageal sphincter (eg, esophageal cancer) and can mimic idiopathic achalasia in clinical presentation and barium swallow study findings. Therefore, patients with suspected achalasia should undergo upper gastrointestinal endoscopy to rule out malignancy.