Achalasia cardia

loss of ganglion cells within the esophageal myenteric plexus
• The disease involves both excitatory (cholinergic) and inhibitory (nitric oxide)
ganglionic neurons.
• 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.

Bird Beak appearance on Barium swallow, Case courtesy of Dr MT Niknejad, Radiopaedia.org. From the case rID: 23554

Clinical features
o Dysphagia
o Regurgitation
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

Diagnosis
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
pressurisation.
▪ 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.

Management
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
meals.
▪ 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
through diathermy.
o Surgical myotomy → laparoscopic heller myotomy

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