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Achalasia Cardia (Esophageal Achalasia) : Causes, Types, Symptoms, & Treatment

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

  1. Primary achalasia
  2. Secondary achalasia: By chagas disease
  3. 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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