Achalasia Cardia
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Direct answer
Achalasia cardia is a primary oesophageal motility disorder defined by impaired relaxation of the lower oesophageal sphincter with absent oesophageal peristalsis, resulting from degeneration of inhibitory (nitric oxide and VIP-containing) myenteric plexus neurones. The patient, typically aged 30–50 with no sex predilection, reports progressive dysphagia to both solids and liquids, regurgitation of undigested food, weight loss and often early chest pain. Barium swallow shows the bird-beak or rat-tail narrowing at the cardia with a dilated oesophagus and an air-fluid level, but high-resolution manometry is the diagnostic gold standard, and every patient needs endoscopy to exclude pseudoachalasia from a cardia malignancy. Effective treatments are graded pneumatic dilation, laparoscopic Heller myotomy with a partial fundoplication, and per-oral endoscopic myotomy (POEM).
What you must remember
- Pathophysiology: loss of inhibitory ganglion cells in Auerbach's myenteric plexus leaves unopposed cholinergic tone — a hypertensive, non-relaxing sphincter with aperistalsis proximal to it.
- Symptom signature: dysphagia to solids and liquids equally from early on (unlike the progressive solid-first pattern of carcinoma), regurgitation of stale food, nocturnal cough or aspiration, and weight loss.
- Barium swallow: smoothly tapered bird-beak (or rat-tail) lower oesophagus, dilated oesophagus, poor emptying, and to-and-fro movement of contrast; an air-fluid level on chest radiograph is suggestive.
- Diagnosis: high-resolution oesophageal manometry (Chicago classification) showing an elevated integrated relaxation pressure with absent or abnormal peristalsis; endoscopy is mandatory to exclude pseudoachalasia (cancer of the cardia or a post-band stricture).
- Heller myotomy divides the circular muscle of the lower sphincter and is combined with a partial fundoplication (Dor anterior or Toupet posterior) to limit reflux; a full Nissen wrap is avoided as it obstructs the aperistaltic oesophagus.
- POEM is an endoscopic myotomy with excellent symptom relief but no antireflux procedure, hence higher rates of post-procedure gastro-oesophageal reflux reported in most series.
- Pharmacological options — nitrates, calcium channel blockers, botulinum toxin — suit only elderly or unfit patients; long-standing achalasia predisposes to squamous carcinoma, with surveillance endoscopy commonly advised after 10 or more years of disease.
Common confusion
Achalasia is confused with carcinoma of the lower oesophagus or cardia because both obstruct the cardia. Malignant dysphagia is relentless, solids-first and accompanied by anorexia and rapid weight loss in an older patient, while achalasia is longstanding, intermittent and affects liquids early; endoscopy with biopsy separates them, and pseudoachalasia is exactly why manometry alone is never enough. The second error is choosing a Nissen fundoplication after Heller myotomy — the aperistaltic oesophagus cannot overcome a 360-degree wrap.
Exam-focused takeaway
Expect the manometry definition (failed LES relaxation plus absent peristalsis), the bird-beak radiograph, and the treatment ladder ending in Heller with partial wrap or POEM. Stems describe dysphagia to liquids and solids for years with regurgitation and a chest radiograph air-fluid level. Subsidiary marks lie in pseudoachalasia, in botulinum toxin for unfit patients, and in the association with squamous carcinoma after longstanding disease.
Frequently asked questions
Which investigation is the gold standard for achalasia?
High-resolution oesophageal manometry demonstrating an elevated integrated relaxation pressure with absent peristalsis; barium swallow supports, and endoscopy excludes pseudoachalasia.
Why is a partial rather than total fundoplication done after Heller myotomy?
Because the aperistaltic oesophagus cannot generate the propulsive force needed to open a complete 360-degree wrap, so a Dor or Toupet partial wrap balances reflux control against obstruction.
What is pseudoachalasia?
A mimic of achalasia produced most often by adenocarcinoma of the gastro-oesophageal junction (or by other infiltrating pathology), which is why endoscopy with biopsy is mandatory in every new case.
Which patients are treated with botulinum toxin?
Elderly patients or those unfit for definitive procedures; injection into the lower sphincter gives temporary improvement lasting months and can be repeated.