Achalasia Cardia- Clinical Features, Diagnosis And Treatment

 An Esophageal motility disorder marked by decreased relaxation of the lower esophageal sphincter (LES) as a result of myenteric plexus neuron loss and missing peristalsis

The causes of esophageal motility disorders are divided into four categories: skeletal muscle dysfunction; pharyngeal paralysis - transfer dysphagia; affected transfer from pharynx to esophagus; achalasia cardia; presentations of dysphagia; liquids > solids; however, these conditions are now classified as dysphagia to both liquids and solids.

Chicago Classification Of Esophageal Motility Disorders

1. Median IRP between 0 and 15 mm Hg
2. The integrated relaxation pressure (IRP) is determined using high-resolution manometry. The median IRP is ↑. The conditions that they represent include esophagogastric obstruction disorder, achalasia cardia, and esophagogastric outlet blockage. The median IRP is normal.  Peristalsis disorders, diffuse esophageal spasm, nut cracker esophagus, and jackhammer esophagus are among them.

Achalasia cardia is further classified into 

1.   Type I (classical achalasia)

2.   Type II 

3.   Type III  (vigorous achalasia) 

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This Classification is Based on Manometry Findings 

Manometry 

The patient is instructed to swallow and is also instructed to swallow a tube containing pressure transducers. The patient is positioned beyond the lower esophageal sphincter. The data is presented as a colored graph. The X and Y axes represent time and distance from the upper esophageal sphincter, respectively. The pressure change is then plotted in the graph. If the food bolus reaches 2 cm from the upper esophageal sphincter 0.5 seconds after swallowing, there is an increase in pressure immediately behind the food bolus as the contraction pressure of peristalsis attempts to push the food bolus forward.

 The pressure should decrease because the LES relaxation is also displayed; additionally, the pressure change is color-coded.
Blue denotes zero pressure, green denotes low pressure, yellow, orange, red, and purple denotes high pressure.  The LES is first displayed as green because it has some resting pressure; as the food bolus reaches the LES, it relaxes and is displayed as blue.

When swallowing occurs, the UES opens and pressure drops to zero. As food is swallowed and the food bolus is processed, the pressure starts to rise. The LES should show a reduction in pressure, but in this case, there is a tiny rise in pressure. This is because of an anomaly.

Perform 10 wet swallows with high-resolution manometry. If the median IRP is normal, there is a peristalsis disorder. If the median IRP is abnormal and greater than 15 mm Hg, search for peristaltic activity. If 100% of swallows reveal no peristaltic activity or premature peristaltic activity. If 100% of swallows fail peristalsis without PEP. PEP, or pan-esophageal pressurization, is required for Achalasia I. In >/= 20% of swallows, peristalsis with PEP is 100% unsuccessful, leading to Achalasia Cardia II. In

Underlying Pathology  

Neurons in the esophageal myenteric plexus are lost; this condition is thought to be caused by an autoimmune process; environmental variables

• Contaminations
• HSV1 is known to have a strong correlation with viruses.
• Genetic elements
  HLA DQA1 0103 and HLA DQB1 0603; abnormal auto-immune response; cytotoxic T cell activation; ganglionitis and neuronal death.

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Causes For Secondary Achalasia

• Carcinoma stomach
• Chaga's disease
• Lymphoma
• Drugs 
• Bariatric surgery 

Clinical Features

Dysphagia: Inadequate peristalsis;  Regurgitation: Inadequate LES relaxation;  Mild weight loss, Chest pain, mucositis of the esophagus caused by food fermentation, esophageal distension, aspiration pneumonia, sudden cessation of pain due to sudden opening of the LES and release of esophageal pressure, high-resolution manometry as the gold standard for achalasia cardia investigation, and endoscopy's potential for limited resultsThe results could show that there is mucositis or difficulty getting past the LES.

Clinical Severity Score 

Eckardt Score 

• Level I - 2-3 
• Level II - 4-6
• Level III - > 6
• 0-1 - level 0 achalasia cardia 

Investigations 

• Chest X-ray 
  • The fundal gas shadow is normally observed, but when the LES is not relaxing well, the swallowed gas may not reach the fundus. • Can be normal in the early stages. • Abnormalities picked up in CXR may be megaesophagus. • Dilatation of mediastinum. • Absence of fundal gas shadow.

• Widened upper mediastinum
• Absence of fundal gas shadow 

Barium Swallow

The LES sphincter is not relaxing well; the esophagus is dilated, particularly in the lower esophagus; the emergence of a rat tail in lower esophageal cancer;  Because of cancer, the outside border of the rat tail is corrugated and lacks smoothness.

Manometry

Uses color topographic images;  High-resolution manometry;  Gold standard for achalasia cardia diagnosis;  Most sensitive investigation
 PEP + → type II in at least 20% of swallows; PEP - → type I; Type III - spasmodic contractions in >/= 20% of swallows; median IRP-integrated relaxation pressure raised > 15 mm Hg; LES resting pressure > 35 mm Hg; 100% absent peristalsis.

First image

There should be a normal pressure rise over time as the food bolus is pushed by peristalsis; however, in type I achalasia cardia, there is neither a rise in pressure nor any peristalsis; the LES-MIRP is increased and the LES's relaxation is impaired; and there is a complete absence of peristalsis. At rest, the UES is closed, therefore this pressure is resting pressure.

Second image 

 There is no propulsive type of pressure build-up, hence there is no peristalsis. There is a diffuse rise in pressure, or pan-esophageal pressurization.  In less than 20% of swallows, there is a complete lack of peristalsis with PEP (type II achalasia cardia).

Third image 

The prognosis is best for type II achalasia cardia because it responds better to treatment and the esophagus exhibits a better degree of pressure to push food boluses into the stomach when the LES is relaxed with medication. This is type III achalasia cardia. Approximately 20% of swallows result in esophageal spasm for achalasia. Since there are just spasmodic contractions, Type III has the worst prognosis and reaction. Type I has an intermediate response.

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Management Of Achalasia Cardia

Objectives: LES relaxing; gravity pushes food into the stomach even in the absence of peristalsis; medicinal; endoscopic; surgical; better long-term results.

Medical management 

Taken 20 to 30 minutes before meals;  To relax the smooth muscle of the LES;  Nitrates: little reaction;  CCB: nifedipine, verapamil;  PDE 5 #: sildenafil; •The efficacy decreases after usage for a few months.

Endoscopic treatments

• Endoscopic balloon dilatation
  • To unwind LES
  • High risk of perforation; increased risk of reflux gastroesophageal reflux; short-term (12–18 months) effects of balloon dilatation; therefore, recurrent dilatation may be necessary.
• Injecting botulinum to LES
  • Effect is short term - 6 - 7 months 

Surgical

Heller’s myotomy

• The earlier approach has been replaced by laparoscopy; the circular rim of the LES is cut; the LES loses tone; the patient must remain upright for one to two hours after eating in order for gravity to aid in the entry of food into the stomach; and the safety profile of the laparoscopic surgery is improved.
• Risk of puncture less than 1%
• Dor's fundoplication is performed in addition to Heller's myotomy since there is GER risk; this is a Heller-Dor procedure.

Poems

Per-oral Endoscopic Myotomy – an incision is made in the mucosa a few centimeters before the LES. – Through the submucosal plane, the LES is reached, incising smooth muscle.

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Hope you found this blog helpful for your NEET SS Gastroenterology and Hepatobiliary preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.