ACHALASIA CARDIA

It is failure of relaxation of cardia (oesophagogastric junction)

due to disorganized oesophageal peristalsis, as a result of failure

of integration of parasympathetic impulses causing functional

obstruction (Achalasia means failure to relax –Greek), It is first

identified by Thomas Willis in 1672.

Aetiology

There is absence or less numbered ganglions in myenteric

plexus.

 Stress.

 Vit.B1 deficiency

 Chaga’s disease. It is caused by Trypanosoma cruzi, which

is common in South America called as sleeping sickness.

 Diffuse oesophageal spasm (Corkscrew oesophagus)

  Most commonly it is idiopathic. There is degeneration of

Auerbach’s myenteric plexus along the entire length of

oesophagus more so in LOS.

There is pencil shaped narrowing of cardia (O-G junction) with

enormous dilatation of proximal oesophagus, which contains

foul smelling fluid and is more prone for aspiration pneumonia.

Achalasia cardia is a precancerous condition- seven times

chances of getting squamous cell carcinoma (8% after 15 years)

Pathology

 Thickening of circular muscle fibres in distal oesophagus.

 Myenteric inflammation, depletion of ganglion cells,

neural fibrosis, reduced nitric oxide and VIP (mediators of

LES relaxation)

 Absence of peristalsis, raised LES pressure, failure of

relaxation with functional obstruction of OG junction.

  Dilatation of proximal oesophagus with atony.

Clinical Features

 Common in females between 20 and 40 years age group.

 Incidence is 6 per 1,00,000 population.

 Chest pain occurs in early stage.

 Achalasia with diffuse oesophageal spasm is called as

‘vigourous achalasia’

 Presents with progressive dysphagia, which is more for

liquid than to solid food.

 Regurgitation and recurrent pneumonia are common

(10%).

 Walking while eating, chin thrusting, neck and shoulder

extension, Valsalva manoeuvre facilitates emptying of

food from the oesophagus.

 Heartburn (50%) is common.

 Malnutrition and general ill health.

 Lung abscess formation.

  Odynophagia and weight loss.

Triad

 Dysphagia

 Regurgitation

 Weight loss

Staging

 Proximal dilatation<4 cm.

 Dilatation between 4-6 cm.

 Dilatation > 6cm.

 Sigmoid dilatation.
Investigations

Barium swallow is diagnostic – shows

 Pencil like smooth narrowing of lower oesophagus – Bird

beak appearance.

 Dilatation of proximal oesophagus

 Absence of fundic gas bubble.

 Sigmoid oesophagus or megaoesophagus

 Chest X-ray shows patches of pneumonia. Double

mediastinal strup of dilated oesophagus is typical with air

fluid level in posterior mediastinum on lateral view.

 Oesophageal manometry shows unrelaxed lower

oesophageal sphincter with high resting pressure- very

useful and gold standard. It shows failure of LES to relax

completely during swallowing and complete absence of

peristalsis. LES pressure is >35mmHg. Base line

oesophageal pressure will be high without progressive

oesophageal peristalsis with low amplitude muscular tone.

Intraoesophageal pressure in relation to intragastric

pressure is elevated.

 Oesophagoscopy is done to confirm the diagnosis and to

rule out carcinoma oesophagus. It shows totally closed

LES with atonic, dilated proximal oesophagus. Biopsy of

mucosa at LES should be done.

 Carcinoma oesophagus.

 Stricture oesophagus.
  Scleroderma.

TREATMENT

Treatment of achalasia

 Forcible dilatation:

o Plummer’s pneumatic dilatation

o Negus hydrostatic balloon dilatation

 Modified Heller’s cardiomyotomy

 Drugs

o Botulinum toxin A

o Nitroglycerine, nifedipine

Surgery

 Modified Heller’s Operation (Heller-German, 1913):

Oesophagocardiomyotomy, Success rate is 85%.

 Either through thoracic or through abdominal approach,

thickened circular muscle fibres are cut longitudinally for

about 8-10 cm, 2cm. proximal to the thickened muscle to 1

 cm. distal to OG junction. Care should be taken not to

open the mucosa (Anterior myotomy is done now. Original

Heller’s is both anterior and posterior myotomies.).

 Nissen’s or Toupet’s fundoplication is done along with the

above procedure to prevent reflux.

 Resection is done when failure of myotomy occurs or

when megaoesophatus or metaplasia is present. Transhiatal

total oesophagectomy with gastic pull up and

oesophagogastric anastomosis in the neck is a good option

in such patients.

 Lapraroscopic/thoracoscopic cardiomyotomy- ideal

 Robotic assisted laparoscopic cardiomyotomy improves

the dexterity and three dimensional vision.

Forcible Dilatation

 It stretches spasmodic segment. Gradual repeated

dilatations are required. Dilatation upto 54 french bougie

can be done. Two types of dilatations are used – pneumatic

and hydrostatic.
  Plummer’s pneumatic dilatation is done using balloons of

30-40 mm. diameters. It is inserted over a guidewire. Risk

of perforation (1%), need for repeated dilatations are the

problems.

 Negus hydrostatic dilatation is done to dilate O-G junction.

It is not very well accepted method as chances of

perforation is high, success rate is 65%. 30 mm diameter

balloon is inflated for 3 minutes.

Drugs

 Endoscopic injection of botulinum toxin to sphincter- high

recurrence rate.

 Calcium channel blocker, nitroglycerin sublingually.