A 62-year-old man comes to the office due to difficulty swallowing both solids and liquids. The patient's symptoms have progressively worsened over the last 3 months. He also has occasional regurgitation of undigested food and a nighttime cough that disturbs his sleep. He has never had similar symptoms before. The patient has lost 4.5 kg (10 lb) during this period but has no other medical problems and takes no medications. He has a 20-pack-year smoking history but quit 10 years ago. He drinks wine on the weekends. Vital signs are normal, and his physical examination is unremarkable. Chest x-ray reveals a widened mediastinum, and barium studies show a dilated esophagus with smooth tapering of the distal esophagus. Which of the following is the best next step in management of this patient?

Question

A 62-year-old man comes to the office due to difficulty swallowing both solids and liquids.  The patient's symptoms have progressively worsened over the last 3 months.  He also has occasional regurgitation of undigested food and a nighttime cough that disturbs his sleep.  He has never had similar symptoms before.  The patient has lost 4.5 kg (10 lb) during this period but has no other medical problems and takes no medications.  He has a 20-pack-year smoking history but quit 10 years ago.  He drinks wine on the weekends.  Vital signs are normal, and his physical examination is unremarkable.  Chest x-ray reveals a widened mediastinum, and barium studies show a dilated esophagus with smooth tapering of the distal esophagus.  Which of the following is the best next step in management of this patient?

Quizplus · Accepted Answer

Explanation: This patient's presentation, with dysphagia to solids and liquids and a dilated esophagus with smooth tapering of the distal esophagus, suggests either primary achalasia (ie, loss of peristalsis in the distal esophagus with lack of lower esophageal sphincter relaxation) or pseudoachalasia due to esophageal cancer. Several clues point to pseudoachalasia (eg, narrowing of distal esophagus not due to denervation) caused by malignancy, including the following: Tobacco use is a major risk factor for esophageal adenocarcinoma and squamous cell carcinoma; alcohol use is also an important risk factor for esophageal squamous cell carcinoma. Significant weight loss, rapid symptom onset (<6 months), and presentation at age >60 all increase the likelihood of malignancy (by comparison, patients with achalasia have symptoms for approximately 5 years before receiving a diagnosis, and they typically only have mild weight loss). Tumor metastasis (eg, mediastinal lymph nodes) or local involvement may give a radiologic appearance similar to that seen with a widened mediastinum. Endoscopic evaluation can differentiate between achalasia and pseudoachalasia. In achalasia, this evaluation usually shows normal-appearing esophageal mucosa and a dilated esophagus with possible residual material; in addition, it is generally possible to easily pass the endoscope through the lower esophageal sphincter (unlike in malignancy). (Choice A) If endoscopy shows a malignancy, a CT scan can be performed for staging. CT scan can also be obtained if endoscopy is nonrevealing and there is still concern for malignancy. However, endoscopy is required because CT can miss some malignancies. (Choices C and F) Esophageal pH monitoring is typically used to confirm gastroesophageal reflux disease (GERD) in patients who have symptoms consistent with GERD but who fail a trial of proton pump inhibitor (PPI) therapy. Although GERD can present with food regurgitation and nighttime cough, it would not explain the patient's weight loss and dysphagia or the barium esophagram findings. In addition, PPIs do not significantly improve the symptoms of achalasia. (Choices D and E) Laparoscopic myotomy and pneumatic balloon dilation are the treatments of choice for patients with achalasia who are at low surgical risk. Options for patients at high surgical risk include botulinum toxin injection, nitrates, and calcium channel blockers. However, all these treatments should be considered only after malignancy is excluded and the diagnosis of achalasia is confirmed. Given the concern for malignancy, reassurance and medical management alone is inappropriate. Educational objective: Pseudoachalasia, which is due to narrowing of the distal esophagus secondary to causes other than denervation (eg, esophageal cancer), can closely mimic achalasia. Clues pointing to pseudoachalasia include significant weight loss, rapid symptom onset, and presentation at age >60. Consequently, endoscopy is recommended to exclude malignancy in all patients with suspected achalasia. References: Achalasia. (http://www.ncbi.nlm.nih.gov/pubmed/23871090) Achalasia: A review of clinical diagnosis, epidemiology, treatment and outcomes. (http://www.ncbi.nlm.nih.gov/pubmed/24124325)

A 62-Year-Old Man Comes to the Office Due to Difficulty