Achalasia Cardia is primarily a disease of esophagus in which lower end of esophagus (Lower Esophageal Sphincter-LES) does not relax properly in response to food intake and there is inappropriate and ineffective motility (Peristalsis) of the body of esophagus. This leads to impaired emptying of esophagus and gradual dilatation of this tubular structure. There is thickening of lower end along with increase in fibrous tissue in muscular layer of esophagus.

Primary achalasia is the most common subtype and is associated with loss of ganglion cells in the esophageal myenteric plexus.

Secondary achalasia is relatively uncommon. Causes are secondary achalasia include certain malignancies, diabetes mellitus, and Chagas disease.

Clinically important features defined in achalasia cardia

• Peristalsis in the distal smooth muscle segment of the esophagus may be lost.
• Contractions occur, but they are weak; simultaneous; uncoordinated; and, therefore, nonpropulsive
• The LES fails to relax, either partially or completely
• LES pressure is elevated in some patients
• The coordination of LES relaxation in response to swallowing and esophageal contraction is lost

Signs and symptoms

• Mostly patient complains of gradually increasing difficulty in swallowing food (both solids and liquids)
• Feeling of stickiness in throat
• These symptoms tend to increase whenever there is stress or cold
• Undigested food tends to come back into mouth (Regurgitation)
• Chest infections / pneumonia tend to occur due to aspiration of food into windpipe
• Heartburn / acidity sensation
• Severe retro sternal chest pain in 30-40% of patients
• Weight loss in advanced esophageal disease
• Patients with achalasia are at increased risk for esophageal cancer

Tests and diagnosis

Barium meal or esophagogram

• Bird beak appearance is the typical presentation in which there is tapering of lower end of esophagus after massively dilated middle part of it
• Air-Fluid levels are seen in the middle part of esophagus
• Image intensifier (Fluoroscopic) evaluation suggests improper motility (peristaltic) activity

Upper GI endoscopy

• Undigested food particles along with ingested fluid filled esophagus
• Lower end of esophagus fail to open even after air insufflation

Manometry

• Considered the gold standard in the diagnosis of this disease
• There is failure of relaxation of lower end of esophagus
• Pressure at lower end may be high but it may be normal also

Endoscopic ultrasound

Thickened muscle layers in the lower part of esophagus.

CT scan

CT scanning with oral contrast enhancement may demonstrate the gross structural esophageal abnormalities associated with achalasia, especially dilatation, which is seen in advanced stages.

Treatment

Treatment options for achalasia include pharmacologic, mechanical, botulinum toxin, and surgical-based therapies.

Four main classes of drugs have been used for this purpose and include the following:

 

• Calcium channel blockers - Nifedipine and verapamil
• Anticholinergic agents - Cimetropium bromide
• Nitrates - Isosorbide dinitrate
• Opioids – Loperamide

Botox injection

• Botulinum toxin therapy works by inhibiting the release of acetylcholine from presynaptic nerve terminals.
• An endoscopist injects botulinum toxin into the LES.
• Effective in 60-80% of patients but recurrence rate is high (50%)
• It can be used as a diagnostic test to identify those patients who will respond to surgical therapy

Endoscopic dilatation

• Endoscope passed (with balloon at the tip of it) and muscle fibres at the lower end of esophagus are forcefully disrupted (damaged).
• Patient responds initially (60-90%) and about 70% of the responders get relief of symptoms till the end of one year.
• Young patients do not respond well. There is about 2% chance of perforation of lower end of esophagus

Surgical therapy- Hellers Myotomy

• Most effective and safest, division of muscle fibres of lower part of esophagus is done.
• Nowadays the surgery is preferred by laparoscopic approach, because it gives better and wide view during surgery and results in early mobilization and rapid recovery.