Questions: Which one of the following statements is true about the epidemiology, pathogenesis, or natural history of achalasia? A. Because there is a higher prevalence of achalasia in men than in women, and particularly in the Caucasian population, these patients with dysphagia should undergo prompt investigation for achalasia, whereas other patient populations should be observed and treated with a more aggressive regimen of PPI therapy. B. The end result of the inflammatory process in achalasia is that of upregulation of cholinergic activity, which subsequently overpowers the inhibitory neurotransmitters nitrous oxide and vasoactive peptide (VIP). This results in an imbalance between excitatory and inhibitory neurons, leading to incomplete relaxation of the lower esophageal sphincter (LES) and aperistalsis. C. Because there is a higher prevalence of achalasia in men than in women, and particularly in the Caucasian population, screening for esophageal squamous cell carcinoma should begin at the onset of diagnosis and subsequently every 2 years. D. Owing to poor esophageal emptying and stasis inflammation, patients with achalasia are at an increased risk of not only esophageal squamous cell carcinoma but also adenocarcinoma, although the latter carries a substantially lower risk than that for squamous cell carcinoma. Which one of the following statements is true about confirming a diagnosis of achalasia? A. The endoscopic finding of a strong resistance at the esophagogastric junction (EGJ) when intubating the stomach should lead the physician to a very high suspicion for achalasia; however, motility testing is still needed to confirm a diagnosis of achalasia. B. A barium esophagram demonstrating a dilated esophagus, a “bird-beak” appearance, aperistalsis, and poor emptying of barium has a very high sensitivity and specificity for making a diagnosis of achalasia, and thus, confirmation with esophageal manometry may be redundant. C. A barium esophagram demonstrating a dilated esophagus, aperistalsis, and poor emptying of barium can help confirm a diagnosis of achalasia in cases where esophageal manometry may have indeterminate findings. D. In patients with high clinical suspicion of achalasia, the absence of a mechanical obstruction on endoscopy or esophagram is sufficient evidence to make a diagnosis of achalasia with > 95% accuracy. In the following clinical scenarios, which treatment recommendation is the appropriate therapy for patients with achalasia? A. For all patients with a new diagnosis of achalasia, oral pharmacologic therapies (e.g., calcium channel blockers or long-acting nitrates) should be the initial therapy because they are effective and the least invasive options. B. For medically fit patients, either graded pneumatic dilation or laparoscopic surgical myotomy with a partial fundoplication are recommended as initial therapy because both methods provide good relief of symptoms with comparable success rates. C. In the patients who are undergoing surgical myotomy, preoperative Botulinum toxin therapy should be given to improve surgical outcomes. D. For patients who develop “end-stage” achalasia characterized by megaesophagus or sigmoid esophagus and significant esophageal dilation and tortuosity, per-oral esophageal myotomy (POEM) should be recommended because it has been shown to be a more effective treatment option than surgical esophagectomy in a randomized prospective trial.
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