Achalasia: The Daily PANCE Blueprint
A 42-year-old woman describes one year of food sticking in her chest. From the very beginning she has had equal difficulty swallowing solids and liquids. She regurgitates undigested food, sometimes hours after eating and occasionally onto her pillow at night, and has developed a nocturnal cough. She has learned to eat slowly, lift her chin, and drink extra water to push food down. She has lost 15 pounds. Antacids and a proton pump inhibitor produced no benefit. A barium swallow shows a dilated esophagus tapering to a smooth, symmetric “bird’s-beak” narrowing at the gastroesophageal junction. Upper endoscopy excludes a mass, and manometry demonstrates absent peristalsis with failure of the lower esophageal sphincter to relax. Which of the following is the most likely diagnosis?
A. Gastroesophageal reflux disease with peptic stricture
B. Esophageal carcinoma
C. Achalasia
D. Diffuse esophageal spasm
E. Eosinophilic esophagitis
Answer and topic summary
The answer is C. Achalasia
Dysphagia to solids and liquids simultaneously from the outset, with a bird’s-beak on barium and manometry showing aperistalsis plus a non-relaxing lower esophageal sphincter, is achalasia. The mechanism is a loss of inhibitory ganglion cells in the myenteric (Auerbach) plexus of the distal esophagus. Those neurons release nitric oxide and vasoactive intestinal peptide, and their job is to tell the LES to relax; destroy them and excitatory cholinergic tone is unopposed, so the sphincter stays shut while the esophageal body loses its coordinated peristaltic wave. That single lesion produces both manometric findings and explains the whole clinical picture. The pattern of dysphagia is the highest-yield discriminator on the exam. A mechanical obstruction — a stricture, a ring, a tumor — narrows the lumen, so trouble begins with solids and only later, as the lumen narrows further, involves liquids. A motility disorder disables the pump itself, so solids and liquids are difficult together from day one. When a stem tells you liquids were hard from the start, stop thinking about masses. Supporting details here fit: patients adopt maneuvers to force the bolus through, regurgitate undigested food without acid taste (distinguishing it from reflux), and can aspirate at night. Diagnosis proceeds as barium swallow, then manometry as the gold standard, then endoscopy in every patient specifically to exclude pseudoachalasia — a malignancy at the gastroesophageal junction that mimics achalasia perfectly. Suspect it when the patient is older, symptoms came on over months rather than years, and weight loss is disproportionate. Treatment is mechanical disruption of the sphincter, because the lost neurons cannot be restored: pneumatic balloon dilation or laparoscopic Heller myotomy with a partial fundoplication, with peroral endoscopic myotomy (POEM) now widely used. Botulinum toxin injection is reserved for poor surgical candidates because its effect fades in months. Nitrates and calcium channel blockers are weak temporizing measures. Counsel patients that achalasia carries a long-term increased risk of esophageal squamous cell carcinoma. Sorting the distractors: reflux with a peptic stricture gives progressive dysphagia to solids first in a patient with a heartburn history who responds to acid suppression, and barium shows an irregular narrowing without proximal dilation; esophageal carcinoma also causes solid-first, rapidly progressive dysphagia with weight loss, typically in an older patient with tobacco, alcohol, or Barrett esophagus, and the barium narrowing is irregular and asymmetric rather than a smooth taper; diffuse esophageal spasm produces intermittent chest pain and dysphagia with a corkscrew esophagus on barium and normal LES relaxation with simultaneous uncoordinated contractions; and eosinophilic esophagitis presents in younger atopic patients with food impaction, showing rings, furrows, and white exudates on endoscopy with more than 15 eosinophils per high-power field on biopsy.
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