ACHALASIA CARDIA (CARDIOSPASM) : DEFINITION, ETIOLOGY, PATHOPHYSIOLOGY, TYPES, SIGNS, SYMPTOMS, DIAGNOSIS, TREATMENT, SURGERY, & PPT
DEFINITION
- Achalasia Cardia is a rare neurodegenerative disorder characterized impaired relaxation of the lower esophageal sphincter (LES) in response to swallowing.
- Age group: 20-40 years
- Occurrence: Females > Males
PATHOPHYSIOLOGY
Myenteric plexus (auerbach’s plexus) : Motor muscular plexus
↓
Inhibitory fibres absent
↓
Lower esophageal sphincter (LES) fails to relax
↓
Primary achalasia
TYPES
- Primary achalasia
- Secondary achalasia: By chagas disease
- Pseudoachalasia: By cancer
CLINICAL PRESENTATION
1. Dysphagia
- Progressive initially (Liquids > Solids)
- Non progressive later (Liquids = Solids)
2. Regurgitation (halitosis) ⇒ Aspiration pneumonitis
3. Weight loss
Triple A Syndrome
- A: Achalasia cardia
- A: Alacrimia
- A: ACTH resistance (Adrenocortical insufficiency)
COMPLICATIONS
- Aspiration pneumonitis
- Cancer (SCC) risk increases by 10-20%
INVESTIGATIONS
1. X-ray
Findings
- Absence of fundal gas shadow
- Widened mediastinum
- Air fluid level in mediastinum
2. Monometery
- Gold standard investigation
Findings
- Non relaxation of LES
- High tone of LES
- Atonic esophagus ⇒ Increased tone (Segmental non propagatory)
3. Barium Swallow
- Investigation of choice
Findings
- Bird beak sign
- Rat tail sign
TREATMENT
A. Medical
- Efficacy < 10%
- Drug of choice: Calcium channel blocker > Nitrates
B. Injection of toxin
- Recurrence > 50% (Highest)
C. Surgical
1. Balloon dilatation
Balloon dilatation
⇓
45-60 Fr (usually 50 Fr)
Result: 60-70%
Risk of rupture: 5%
Note:
- 3 Fr: 1 mm diameter
- Fr: Outer circumference
2. Myotomy
- Heller’s myotomy
- Process: Laparotomy > Laproscopy
- Result: >90%
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