Achalasia Cardia Types

Plummer-Vinson Syndrome

Plummer-Vinson Syndrome Introduction: It is also called Paterson-Kelly syndrome. It is a precancerous condition in which there is a severe spasm of circular muscle fibres at the cricopharyngeal sphincter level or pharynx-oesophageal junction, and it is associated with development of post cricoid web.

Aetiopathogenesis

• Aetiology is not known
• It is seen in women who have worries and anxiety.
• As a result of the spasm and web, dysplasia occurs and leads to features of anaemia later. The proximal mucosa is constantly irritated due to stasis. It undergoes hypertrophy, hyperkeratosis and desquamation. This, over a period of years, predisposes to carcinoma oesophagus (carcinoma oropharynx).

Read And Learn More: Gastrointestinal Surgery Notes

Plummer-Vinson Syndrome Clinical Features

• Women in the middle age group are affected.
• Increasing dysphagia for solids and liquids due to spasm.
• Features of anaemia—pallor, stomatitis, ulcerations, bald tongue (without papillae), koilonychia, splenomegaly and microcytic hypochromic anaemia.
• As a result of obstruction, the fluid tends to spill over into the larynx giving rise to recurrent aspiration, respiratory tract infection, cyanosis or choking.

Plummer-Vinson Syndrome Treatment

• Reassurance
• Improving anaemia: Iron tablets or blood transfusion and correction of nutritional deficiencies.
• Regular dilatation by using gum elastic bougies.

Plummer-Vinson Syndrome Summary

• Post cricoid web
• Low haemoglobin-anaemia-correction by iron supplements
• Upper cricopharyngeal sphincter spasm
• Middle aged woman
• Malignancy is a complication
• Elastic bougie—dilatation
• Respiratory complications
• You can remember as PLUMMER

Achalasia Cardia

Achalasia means failure to relax. It is a primary oesophageal motility disorder. It is also called cardiospasm because of severe spasm of the circular muscle fibres of the lower end of the oesophagus. The contracted segment does not relax during the act of swallowing (achalasia = failure of relaxation).

As a result of this, there is dilatation, tortuosity and hypertrophy of the oesophagus above. Incidence: 6 in 1,00,000 population/year. Importantly it is a pre-cancerous condition for carcinoma oesophagus.

Achalasia and Carcinoma Oesophagus

• 8% chances of carcinoma over 20 years period.
• Squamous cell carcinoma is the most common due to prolonged stasis and irritation.
• Sometimes adenocarcinoma can occur in the middle third.
• A long-term surveillance program is required.

Aetiopathogenesis

1. Idiopathic: This occurs due to absence or degeneration of Auerbach’s plexus throughout the body of the oesophagus leading to improper integration of the para¬sympathetic impulse. This is called primary achalasia.
2. Acquired variety is seen in South American countries caused by Trypanosoma cruzi (sleeping sickness)— Chagas disease. This organism destroys the ganglion cells of the Auerbach’s plexus.
3. Stress emotional factors and vitamin deficiencies are also associated with this disease.

Achalasia Cardia Pathophysiology

Types of Achalasia Cardia

1. Classical achalasia: Loss of ganglion cells and neural fibrosis occurs (Type 1—classic)
2. Achalasia with oesophageal compression (Type 2)
3. Vigorous achalasia: No loss of ganglion cells but ganglions occurs (Type 3/spastic achalasia)

Achalasia Cardia Clinical Features

• Women around 30-40 years of age are commonly affected. The ratio of affected females to males is 3:2.
• Most patients find eating slow and the drink large volume of fluid. As a result, when peristalsis starts retrosternal pain develops.
• Dysphagia develops slowly, and it is progressive. Solids, by forming a bolus and aided by gravity, as they touch the contracted segment, may partially open up the sphincter. Thus, there is no dysphagia for solids. Dysphagia for liquids is an important feature and it results in regurgitation (oesophageal pseudovomiting). The regurgitant material contains foul-smelling oesophageal contents. Malnourish- ment, ill health and weight loss follow soon.
• Dysphagia, regurgitation and weight loss form the triad of achalasia cardia.
• Recurrent respiratory tract infections due to spillage of liquids can also occur.
• Features of anaemia—glossitis, stomatitis, pallor, bald tongue.
• Retrosternal discomfort and radiation of pain to the interscapular region may be present.
• Pseudoachalasia: Tumours of cardia mimicking achalasia. Often patients present with features of recent achalasia (dysphagia). During endoscopy, some difficulties are encountered and once the scope enters the GE junction, growth will be seen.
• Inhalation of amyl nitrite leads to sphincter relaxation in achalasia but not in pseudo achalasia.

Achalasia Cardia Investigations

1. Barium swallow
   • Uniformly dilated oesophagus above, with a smooth tapering segment below—cucumber oesophagus.
   • In chronic cases, it may be sigmoid-shaped.
2. X-ray chest
   • Mediastinal mass (pseudotumour)1 produced by dilated oesophagus can be seen.
   • Retrocardiac air-fluid level is seen in the lateral view.
   • Aspiration pneumonitis can be diagnosed.
3. Plain X-ray abdomen erect: Fundic air bubble is absent because of the stasis of fluid in the oesophagus.
4. Oesophagoscopy: It reveals a dilated sac containing stagnant food and fluid due to stasis which splashes out with each heartbeat and with each respiratory movement.
   • LES: Lower oesophageal sphincter is closed, with air insufflation. It has a ‘rosette’ appearance.
   • Oesophagoscopy is also done to rule out proximal malignancy.
   • Also done to evaluate oesophagitis, stricture or a tumour at cardia.
5. Oesophageal manometry: Following features are characteristic of achalasia cardia:
   • Hypertensive lower oesophageal sphincter (LOS): It does not relax on swallowing.
   • Aperistalsis in the body of the oesophagus
   • Increased resting pressure in the oesophagus
6. Ultrasound: It may detect subepithelial tumour infiltration in secondary achalasia due to a distal carcinoma.

Achalasia Cardia Treatment

• Aim of the treatment is to cure the disease.
• More importantly, the obstruction at the lower end of oesophagus must be relieved.
• Modified laparoscopic Heller’s cardiomyotomy is the choice of surgery now.

1. Heller’s cardiomyotomy: The aim is to reduce out-flow resistance at the lower oesophageal sphincter. With a left thoracoabdominal incision, the oesophagus and the

• The contracted segment is felt between the fingers.
• A 7-10 cm long incision is made through the lower end of the oesophagus and carried over to the stomach.
• The muscles are cut till the mucosa bulges out. The myotomy should extend proximally up to the aortic arch and distally up to the stomach to 1-2 cm below the junction. Success rate is around 90%. 3 to 5% of the patients develop reflux oesophagitis which needs to be treated conservatively.

2. Forceful dilatation: By using pneumatic balloon— the balloon is positioned under fluoroscopic control within LOS. It should be rapidly inflated to a pressure of 300 mmHg for 15 seconds. Success rate is around 70%. 20% chances of reflux are present. Oesophageal perforation can also occur. Recurrences are common.

3. Injection treatment: Inj. botulinum toxin A is injected into the lower oesophageal sphincter (LOS) endoscopically. It is temporary and repeat injections are necessary.

4. Drugs: Sublingual nifedipine can produce short-term relief.

Injection Botulinum Toxin A

• Endoscopic botulinum toxin is the most common initial treatment of achalasia cardia in USA.
• It is a neurotoxin.
• 8 different types of toxin are available. Most common being type A.
• Injection acts by interfering with cholinergic excitatory neural activity at LOS and blocking acetylcholine release from nerve terminals.
• It is an excellent endotherapy for the short-term treatment of achalasia cardia.
• In about more than 50% cases, relapse occurs within few months and repeat injections are necessary.

5. Endoscopic myotomy—popularly called POEM: In this, through endoscope, a submucosal injection of a mixture of indigo rouge, epinephrine, and saline is done. A 2 cm long incision is made and submucosal tunnel is created. Then circular muscle bundle is cut slowly, beginning from 7 to 8 cm above GE junction to 2-3 cm below GE junction. Mucosa is sutured with absorbable sutures.

Achalasia Cardia Complications

• Reflux is the most common complication. As a result of which recurrent aspirations and lung complica¬tions including pneumonia are the features.
• Due to prolonged stasis and chronic irritation, it can predispose to carcinoma of the mid and lower oesophagus (due to metaplasia). Hence, it is a precancerous condition. Squamous cell carcinoma is the most common type identified in a patient with achalasia.
• Nutritional deficiencies—anaemia and weight loss.