Definition: 

   Achalasia cardia is defined as a triad of disorder which are as follow,

1-Failure of lower esophageal sphincter to relax.  
 2-Aperistalsis  
 3-increased lower esophageal sphincter

Introduction:

  Achalasia cardia is the motility or movement disorder of esophagus in which lower esophageal sphincter fails to relax, which causes difficulty in movement of food from esophagus to stomach. In achalasia cardia there is loss of peristalsis, involuntary movement of muscles which pushes food from esophagus to stomach. Peristalsis occur in whole GIT, in stomach it mixes food, in intestine it moves digested food toward. In achalasia cardia, as lower esophageal sphincter is tight and there is no movement of esophageal muscles therefore, food cannot not move to stomach so that, it is collected in esophagus due to which esophagus is dilated.

Pathophysiology of Achalasia cardia

There is a degeneration of inhibitory neurons in the myenteric plexus, also called as Auerbach plexus in achalasia.

 Myenteric plexus

It is the collection of nerves present on the wall of gastrointestinal system between the layers of circular and longitudinal smooth muscles. Myenteric plexus controls movement or peristalsis of gastrointestinal system. In achalasia cardia  dysfunction of myenteric plexus can lead to failure of LES to relax.

Degenerated pre and post ganglionic nerve cell on electron microscopy.

Etiology/ causes of Achalasia:

On the basis of cause:

 Achalasia cardia is of two types.

1- Primary achalasia.

 2- Secondary achalasia.

 Primary achalasia:

It is an idiopathic, which means the cause is unknown but may also be due to:

•  autoimmune destruction. [Body’s immune system mistakenly attacks its own healthy cells].
•  Viral infection :

Viruses may trigger achalasia cardia, but no single virus has been definitively established as the cause.

• Herpes simplex virus type 1[HSV-1]  which is most common cause of primary achalasia.
•  Varicella zoster which cause chickenpox 
• Measles virus.

 2-Secondary Achalasia:

  It is also called as pseudoachalasia because it looks like primary achalasia however, it results from some other diseases mostly by some obstructions like malignancy which is esophageal cancer and gastric cancer.

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 Chagas Disease 

It is caused by tropical parasite Trypanosoma cruzi. The disease is spread by the bug bite and directly affect automatic nerve in esophagus and causes failure of LES to relax. It produces secondary achalasia.

Chicago classification:

 This classification is based on esophageal manometry which is a test used to measure .

• Pressure inside the esophagus 
• Relaxation of muscle in the LES 
• Contraction of muscle in the LES

               It is a gold standard test for achalasia .it also guides about treatment plan for achalasia according to type of achalasia .

Procedure of Esophageal Manometry:

 A pressure sensitive catheter is passed from nose to esophagus the patient swallows small amount of water and machine records pressure inside esophagus and relaxation of LES 

1- Type 1 Achalasia 

It is also called as classic achalasia the manometric findings shows:

•  No contraction of esophageal muscle
• Minimal pressure inside the esophagus .

Type 2 Achalasia

        Manometry shows pan esophageal pressurization [pan means hole ]which means pressure inside hole or complete esophagus at the same time during swallowing.

•  No peristalsis 
•  It shows best treatment response.

 Type 3 Achalasia 

              It is also called as spasm achalasia because esophagus develops sudden abnormal tight spasm and LES do not relax .Severe chest pain is present in type 3 achalasia because of sudden spasm.

Clinical Features of Achalasia Cardia:

 1-Dysphagia:

               It  is most common feature of achalasia ( which is the difficulty in swallowing) in mechanical obstruction there is only difficult to swallow solid content of food but in achalasia there is difficulty in both solid and then liquid .

2-Regurgitation:

            Undigested food return into mouth it mostly occur at night while sleeping.

3- Chest Pain:

           2/3 of  patient tell about chest pain, it is so severe that it can cause low food intake and weight loss it is most common in type 3 achalasia because of sudden spasm of esophageal muscle

 4-Weight Loss:

          Due to low intake of food .

5-Heartburn:

          Heartburn may occur due to retained food, esophageal irritation, or gastroesophageal reflux after treatment.

6- Cough at night
7- Wheezing sound
8- Aspiration Pneumonia

         Which is lung infection when food intake mistakenly enters lung instead of stomach.

 How To Diagnose  Achalasia Cardia?

1- Barium Swallow:

            It is an X-ray test used to examine esophagus and swallowing process.

 Procedure:

           Patient drinks a white liquid called barium which coats esophagus and can be seen or visible on the X-ray on barium swallow achalasia looks as;

• Esophagus is dilated above [wide at top]
• LES is narrow.
• Bird beak appearance.

2- Upper GI endoscopy for Achalasia Cardia:

         A flexible camera tube is passed from mouth to stomach it is used to differentiate between primary achalasia from cancer 

~usually scope can be passed from LES by putting small pressure even in primary achalasia.

 ~if achalasia is due to cancer it will be difficult to pass scope from LES it also shows;

•  Remains of food in esophagus even after fasting
•  Tight or closed LES  

It is the gold standard because it confirms:

• Impaired LES relaxation
• Elevated integrated relaxation pressure (IRP)
• Absent peristalsis

3-CT- scan

4- High Resolution Manometry of Achalasia Cardia

Differential Diagnosis: of Achalasia cardia

•  carcinoma 
• GERD .
• Diffuse esophageal spasm
• Esophageal stricture 

How to Manage Achalasia Cardia:

          Neuronal damage cannot be treated but some treatment plans are present.

1- Medical Treatment of Achalasia Cardia:

           It is a temporary or given to those patient who are not eligible for surgery.

•  Nitrates (isosorbide dinitrate )it relaxes LES.
• Calcium channel blocker (Nifidipine) decreases pressure in LES .

2- Endoscopic Treatment of Achalasia cardia :

• Pneumatic Dilatation for Achalasia Cardia:

          It is a process in which balloon dilation is used to treat achalasia when there is tight LES in this process the patient is given light anesthesia and the endoscope is passed from mouth to the esophagus then a guide wire is passed through the scope and then withdraw the scope or remove the scope leaving the guide wire Then introduce pneumatic balloon dilator over the guide wire position balloon across LES under endoscopic guide wire inflate balloon with air or contrast inflate for 30 to 60sec.

        Achalasia  balloon is filled with water because water puts same pressure on all the walls and water is safer and visible on fluoroscopy.

•Botulinum toxin injection:

         Botox is injected in LES it blocks acetylcholine release.  it gives temporary benefit .it is used in older patient who are at high risk.

3- Surgical Treatment of Achalasia Cardia:

Heller Myotomy:

        It is an operation to treat achalasia. It is usually performed laparoscopically through small abdominal incisions. then camera is inserted in lower esophageal sphincter then DR. cuts the tight muscle of LES so that the food can pass easily into the stomach after it mostly there is need to cut fundus called as fundoplication because of acid reflux. it gives long term relief.

POEM:

          It is a modern treatment for achalasia . 

• per oral means through the mouth
• endoscopic (using camera tube) 
•  myotomy (cutting muscle)

        Endoscope is passed via mouth small cut is made in inner esophageal lining tight fiber are cut entry side is closed with clips.

Technical steps of esophageal POEM

Advantages Of POEM :

•  No external surgical cut .
•  Fast recovery.
•  Very effective .
• Good for type 3 achalasia.

Disadvantage of POEM:

• Higher risk of GERD because fundoplication is not performed.

Complications of Achalasia cardia:

• Esophageal ulcers.
• Risk of cancer if achalasia remains untreated for long time.
• Esophagitis due to remains of food.
• Aspiration pneumonia ;due to regurgitation.
• Megaesophagus 
• Malnutrition 
•  Esophageal perforation (after treatment)

Conclusion:

Achalasia cardia is a motility disorder of esophagus which causes difficulty in swallowing and chest pain. Early diagnosis and timely treatment can relieve symptoms and also prevent complications. Regular follow ups are essential for better management.

Frequently Asked Questions FAQ :

1-what is the gold standard to test achalasia ?

Answer : Esophageal manometry is used for gold standard test for achalasia.

2-what are the findings on barium swallow ?

Answer: Bird beak appearance due to narrowing of the lower esophageal sphincter .

3- what is the most definite treatment for achalasia ?

Answer: 1- Medical Treatment of Achalasia Cardia:
  It is a temporary or given to those patient who are not eligible for surgery.
 Nitrates (isosorbide dinitrate )it relaxes LES.
Calcium channel blocker (Nifidipine) decreases pressure in LES . And most definite treament is :Hellers myotomy & POEM.

About the Author:-

Dr. Sufiyan Awan is a dedicated medical professional and healthcare researcher with comprehensive clinical exposure. He has formerly served as a House Officer at Nishtar Hospital, Multan, where he managed diverse clinical cases. Along with his foundational medical expertise, Dr. Sufiyan has completed specialized training with the World Health Organization (WHO), enhancing his insight into global health standards and infectious disease management. He actively writes evidence-based medical articles to bridge the gap between complex clinical science and public health awareness, helping readers access reliable and scientifically accurate medical information.