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.

This normally happens between the age of 20-50 yrs and involves both males and females equally.

 Signs / Symptoms / Clinical Presentation-

• Mostly patient complains of gradually increasing difficulty in swallowing food (both solids and liquids). There is 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 chest pain in 30-40% of patients

How to Diagnose-

• Barium meal (Esophagogram)- ?Bird?s Beak? 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 suggest improper motility ( peristaltic ) activity.

  Endoscopy Upper GI- 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.

How to manage it?

The goal of therapy (medical or surgical) is not to treat the underlying disease but only to provide palliation-means adequate emptying / drainage of esophagus only.

Medical treatment ?

• Drugs ( nitrates / calcium channel blockers) are there to increase the effective motility and to relieve pressure at the lower end so that there is better emptying of esopahgus. Indicated in those patients only, who can not undergo surgery. Side effects of medical therapy- unpredictable response due to ineffective absorption in the presence of lots of food particles and residue and fluids in esophagus and inability of drugs itself to get into stomach because of tightly closed lower end of esophagus- so absorption is inadequate. Headache and tissue swelling (edema) are common.

• Botox injection-effective in 60-80% of patients but recurrence rate is high (50%). Advantage?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). This is done under vision and repeated after some days. 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- Most effective and safest. Heller was the surgeon who described surgery for this condition long back in 1914 and performed myotomy (division of muscle fibres) both anteriorly as well as posteriorly. Nowadays it is done only on the anterior aspect of the esophagus.

Can be done through chest (trans-thoracic) approach or through abdomen (trans-abdominal) approach.

Nowadays this surgery is preferred by laparoscopic approach because it gives better and wide view during surgery and results in early mobilization and rapid recovery.

Procedure / Operation (HELLER?s MYOTOMY) details  Normally five small ( <1 cm) are given on abdomen. Stomach is mobilized along with lower part of esophagus and muscle fibres are divided (up to 6 cm proximally on esophagus and upto 3 cm distally on stomach) till the innermost part of the wall of esophagus is exposed. Then covering fundoplication (Toupet?s) is done to prevent gastro-esophageal reflux. Oral liquids are started on 2^nd day of surgery and patient is also discharged in 48-72 hours. Normal diet is started in 2 weeks.