Achalasia Cardia: Diagnosis, Types, Treatment & Management

Q1. A patient presented with dysphagia and foul regurgitation. HRM showed increased median IRP. The manometry image is given below. What is the diagnosis?

A. Diffuse esophageal spasm

B. Nutcracker esophagus

C. Achalasia cardia type I

D. Achalasia cardia type III

Ans. C

• Median IRP is elevated, indicating EJG obstruction disorder (achalasia cardia).
• No peristalsis and no PEP—type I achalasia cardia
• Diffuse esophageal spasm and Nutcracker esophagus are disorders of peristalsis

Chicago classification V 4.0 

• Classifies achalasia cardia based on HRM
• Classifies esophageal motility disorders into two types
  • Esophagogastric junction obstruction
  • Disorders of peristalsis
• The classification is based on the median IRP
  • In EGJ obstruction disorders, median IRP is elevated
    • As in achalasia cardia
    • Also, Esophagogastric Junction outlet obstruction
  • In disorders of peristalsis, median IRP is normal
    • Diffuse esophageal spasm
    • Nutcracker esophagus
• In achalasia, there is a disorder of peristalsis also, but the classification mainly looks at median IRP

Also Read: Cancers of Biliary Tract

Achalasia Cardia is Classified Into

• Primary
  • Autoimmune
  • HSV

• Secondary
  • Carcinoma stomach
  • Chagas disease
  • Lymphoma
  • Drugs
  • Bariatric surgery
• Achalasia cardia is divided into
  • Type I
  • Type II
  • Type III
• In all 3 types, there is a 100 % absence of peristalsis and median IRP elevated
  • Type I - classical - no PEP
  • Type II - PEP in ≥ 20 % of swallows
    • The fluid bolus builds up the pressure—pan-esophageal pressure.
  • Type III - =/> 20% swallow shows spasmodic contractions

Normal Peristalsis

• When the food enters the esophagus, behind the food bolus there is a contraction that pushes the food bolus
• Beyond food bolus, there is relaxation.
• The wave then progresses further down to push food bolus into the stomach
• In type III achalasia, there are random spasmodic contractions that do not effectively push food bolus

Manometry

• The data is given out as a colored graph
  • X axis represents time
  • Y axis represents the distance from the upper esophageal sphincter
  • The pressure change is then plotted in the graph—colorcoded
  • At rest, both UES and LES have some pressure to prevent reflux
  • The pressure grades are from low to high
    • Blue: 0 pressure
    • Green: low pressure
    • Yellow
    • Orange
    • Red
    • Purple
• When the swallow begins, the UES relaxes—represented as blue
• As bolus reaches further down, the pressure builds up (red), and it travels in space and time through the esophagus
• As the food bolus reaches LES, it relaxes (blue) to allow smooth travel of bolus into the stomach
• This is a normal peristaltic activity
• In type I - peristaltic activity is absent, so there are no pressure changes in the esophagus
• In type II - there is pan-esophageal pressurization and there is a diffuse rise in pressure
• Type III - high pressure at some random points in time and space due to spasmodic contraction

• Showing types of achalasia cardia
  • Type
    • Impaired LES relaxation; sustained high pressure of LES; median IRP high
    • No peristalsis
  • Type II
    • PEP - Pan-Esophageal Pressurization
    • No peristalsis
    • Median IRP high
  • o Type III
    • Spasmodic contractions
    • No peristalsis
    • Median IRP high

Also read: Periarticular Disorders: Soft Tissue Rheumatism & Treatments

Q2. Which of the following criteria is used for the classification of esophageal motility disorders? 

A. Chicago classification

B. Pittsburg classification

C. Montreal classification

D. Chapel-hill classification

Ans. A

Chicago classification divides esophageal motility disorders into two types

Esophagogastric junction obstruction

• Median IRP is elevated
• May or may not have abnormal peristalsis also
  • Achalasia cardia
  • Esophagogastric outlet obstruction

Disorders of peristalsis

• Median IRP is normal - LES relaxation is normal
  • Absent contractility
  • Diffuse esophageal spasm
  • Hypercontractile esophagus
  • Ineffective esophageal motility
  • Nutcracker esophagus

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Q3. False statement about Achalasia cardia

A. It is a premalignant condition

B. Malignant risk persists even after Heller's myotomy

C. Most common malignancy seen in AC is adenocarcinoma

D. Heller's myotomy is often combined with toupet or Dor's fundoplication

Ans. C

• There is an 8 % risk of development of carcinoma in AC
  • Fermented food leads to mucositis of the esophagus
  • Chronic sustained mucositis becomes a premalignant condition
• The risk of malignancy persists even after medical or surgical management
  • So the patient should be ideally put under surveillance
  • In Heller's myotomy, only the LES is relaxed
  • The peristalsis is not restored and they do not reverse the injury already caused
• Most common malignancy is squamous carcinoma and not adenocarcinoma
  • Adenocarcinoma can occur in the site where food stagnates
• Heller's myotomy is the best treatment
  • But there is still a risk of GERD
  • So this is combined with Toupet or Dor's procedure

Treatment of achalasia cardia

• Goal is to relax LES
• Medical
• Endoscopic
• Surgical - Better long-term effects

Also read: Recurrent and Autoimmune Pancreatitis

Medical management

• Nitrates - not much response
• CCB - Nifedipine, Verapamil
• PDE 5 # - Sildenafil
• The efficacy reduces after using for a few months

Endoscopic treatments

• Endoscopic balloon dilatation/pneumatic dilatation of LES
  • > 50 % of patients develop symptoms within 1 year
• Injecting botulinum to LES
  • Cause paralysis of LES
  • Effect is short-term (6-7 months)

Surgical

Heller's myotomy

• Laparoscopy has replaced the older procedure
• GERD risk is there
• It can be severely symptomatic and can develop BE
• This can further increase the risk of malignancy
• So fundoplication is done along with the procedure

POEMS

• Peroral Endoscopic Myotomy
• Few cm before LES, a cut is made in the mucosa
• Through submucosal plane, it reaches the LES and incise smooth
• Heller's myotomy + fundoplication shows best outcome
• There is a higher risk of perforation with endoscopic procedures

Q4. False about Achalasia cardia 

A. Elevated IRP

B. Increase LES pressure

C. 100 % absent peristalsis

D. Normal IRP

Ans. D

• Achalasia cardia
  • Median IRP > 15 mm Hg
  • There is increased LES pressure
  • 100 % absent peristalsis
• When IRP is normal - it is a disorder of peristalsis

Also read: Menetrier's Disease: Epidemiology, Etiopathogenesis

Q5. Esophagectomy is indicated in achalasia cardia patients in which of the following circumstances? 

A. Megaesophagus

B. Segmented esophagus

C. Failure of > 1 myotomy

D. All of the above

Ans. D

Esophagectomy is indicated in case of

• Megaesophagus
• Sigmoid esophagus
• Failure of > 1 myotomy
• In megaesophagus, the esophagus is so distended that gravity may not be able to push the bolus into the stomach
• In the sigmoid esophagus, also due to the shape, the gravity may not be able to push bolus even after other procedures which relax LES
• Continuing myotomy will increase the risk of perforation and esophageal injury
• So esophagectomy may be indicated

Does esophagectomy reduce cancer risk?

• Risk is reduced
• Risk in the affected segment is reduced
• But the segment which has been exposed to food particles that are not resected may still pose some risk of malignancy

Also read: Mesenteric Ischemia: Causes, Symptoms, and Treatments

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