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Achalasia Cardia
Presented By-Dr Santosh M Narayankar
• Rare
• Primary oesophageal motor disorder
• Unknown aetiology
• Complex pathophysiology
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
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.
Pathophysiology
Proposed Patterns of Neuronal Function
(Postganglionic and Vagal)
Among Achalasia Subgroups
Clinical features
•Solids> liquids
•Gradual onset
over2yrs
•Fluctuates and
eventually
plateaus
Dysphagia
•10% patients,
•May be presenting
complaint,
•Stridor and airway
compramise
Bronchopulmonary
complications
•No acid reflex
•Fermentation
of retained
food by
bacteria
Heart
Burn
• Due to spasm
?longitudinal
muscle,
• Not relived by
treatment
• spontaneous
resolution
Chest
pain
Diagnosis
HRM
Barium swallow
Endoscopy
EUS
CT scan
High Resolution Manometry
Barium studies
X-Ray chest
Endoscopy
Role Of EUS
CT Chest
Differential Diagnosis
Distal Esophageal Spasm
Chaga’s Disease
Pseudoachalasia
Post Surgical
• Trypanosoma cruzi
• Reduvid bug
• Chronic phase over
20yrs,50% first
detetction,90% dilatation
• Megarectum colon
ureter duodenum
Chagas’
disease
• Occurs in 5% pts
manometricaly diagnosed
achalasia
Pseudoachalasia
• Suspect when Age >50yrs
• Abrupt &recent onset <1year
• Early weight loss>7kg,resistance on endoscopy.
• Adenocarcinoma of EGJ >50% cases
• EUS/CT chest
•Fundoplication
•Adjustable
gastric bands
• Difficult to asses
Post
surgical
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
Modalities
Pharmacologic
Botox injection
Forceful dilatation-Pneumatic
Surgical Myotomy-Hellers
POEM
SEMS
Esophagectomy
Pharmacologic
• Amyl nitrate,NTG,Theophylline,B2
agonistsOthers
• 5-10mg
• Reduces LES pressure by 66%,
• Headache
Isosorbide
dinitrate
• 30-40mg/day sublingual
• Flushing,dizziness,edema,headcheNifidipine
• 50mg
• Side effectsSildenafil
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
Pneumatic Dilatation
Rigiflex dilator
• Long cylindrical non
compliant balloon
• Sizees 3,3.5,4 cm
• Positioned across LES
fluroscopicaly
• Esophageal
perforation(1%)
• Efficacy 32-98%
Rigiflex
Dilator
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
•Based on
NOTES
•90 % efficacy
•Comparable to
lap myotomy
POEM
Other Modality
• SEMS
• Esophagectomy
Patient follow up
• Assessment of symptom relief
• Barium swallow
• Endoscopy not recommended for cancer screening
Eckardt symptom scoring
and staging
Risk of cancer
Squamous cell carcinoma
Stasis hypothesis
0.15% risk annual
Screening not required, can be done after 15years (ASGE)
Dilated sigmoid esophagus
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
Thank You
History

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