loss of ganglion cells within the esophageal myenteric plexus
• The disease involves both excitatory (cholinergic) and inhibitory (nitric oxide)
• Their absence leads to impaired deglutitive LES relaxation and absent peristalsis.
• Long-standing achalasia is characterized by progressive dilatation and sigmoid
deformity of the esophagus with hypertrophy of the LES.
o Chest pain
o Weight loss
o Most patients report solid and liquid food dysphagia.
o Tumour infiltration, most commonly seen with carcinoma in the gastric
fundus or distal oesophagus, can mimic idiopathic achalasia. The resultant
“pseudoachalasia” accounts for up to 5% of suspected cases
o Barium swallow x-ray- dilated oesophagus, air-fluid level, tapering at the LES Bird Beak appearance.
o Oesophageal manometry- impaired LES relaxation and absent peristalsis.
▪ Classic achalasia- swallowing results in no change in oesophageal
▪ Achalasia with compression- swallowing results in simultaneous
pressurisation that spans the entire length of the oesophagus.
▪ Spastic achalasia- swallowing results in premature and lumen
obliterating contractions or spasms.
o Pharmacologic Rx:
▪ Least effective.
▪ Can be considered in patients who are unwilling or unable to tolerate
invasive therapy for achalasia.
▪ Nitrates →
• sublingual isosorbide dinitrate (5mg) 10-15 minutes before
▪ Other agents → nifedipine, sildenafil, etc.
o Pneumatic dilatation → most cost effective for achalasia.
o Botulinum toxin injection →can be considered in individuals who are not
good candidates for pneumatic dilatation, surgical myotomy or POEM.
o POEM → peroral endoscopic myotomy- endoscopically incision is made in the
esophageal mucosa and the endoscope is passed through that incision into
esophageal submucosa creating a submucosal tunnel that is extended distally
into the gastric cardia. The muscle of the muscularis propria is severed
o Surgical myotomy → laparoscopic heller myotomy