3. Definition
• Manometrically by insufficient relaxation of the
lower esophageal sphincter (LES) and loss of
esophageal peristalsis
• Radiographically by aperistalsis, esophageal
dilation, with minimal LES opening, “ bird-beak ”
appearance, poor emptying of barium.
• Endoscopically by dilated esophagus with
retained saliva, liquid, and undigested food
particles in the absence of mucosal stricturing or
tumor
4. Epidemiology
Incidence is about 1 per 100000
Male = Female
Presents between age 25 and 60.
Prevalence greatly exceeds its incidence
Prevalence estimates range from 7.1 to 13.4 per 100,000 .
Familial clustering of raise the possibility of genetic
predisposition.
Has been reported in monozygotic twins,siblings and
children of affected parents.
26. Management
In practical terms, this amounts to reducing LES pressure
so that gravity promotes esophageal emptying.
Treatment is directed at compensating for the poor
esophageal emptying and preventing complications.
Underlying neuropathology of achalasia cannot be
corrected
29. Botulinum Injection
• Intrasphincteric,4 quadrants
• 80 units
• Decreases LES pressure by 33%
• Improves dysphagia in 66% pts
over 6 month period
• chest discomfort and rash
• Pts not fit for definitive
treatment
Botox
injection
31. • Long cylindrical non
compliant balloon
• Sizees 3,3.5,4 cm
• Positioned across LES
fluroscopicaly
• Esophageal
perforation(1%)
• Efficacy 32-98%
Rigiflex
Dilator
32.
33. Surgical Myotomy
Hellers myotomy 1913
Anterior myotomy
Lap > Thoracotomy
Excellent results 62-100%
Persistent dysphagia in <10% patients
Overall mortality 2%
Reflux disease managed by PPI
Toupet/Dor – Lap myotomy+partial fundoplication
48. Risk of cancer
Squamous cell carcinoma
Stasis hypothesis
0.15% risk annual
Screening not required, can be done after 15years (ASGE)
Dilated sigmoid esophagus
49. Take Home Message
• Rare disease
• Complex pathophysiology
• Dysphagia main symptom
• HRM for diagnosis
• Endoscopy to rule out pseudoachalasia
• Treatment –PD,LHM,POEM according to
achalasia type