Achalasia Cardia - Standard Treatment Guidelines

Esophageal dysphagia involves difficulty in transferring food from the upper esophageal region to the stomach. It is subdivided into mechanical dysphagia (caused by structural lesions in the path of food bolus transit) or motor dysphagia (caused by neuromuscular dysfunction). Mechanical dysphagia is generally progressive in nature with dysphagia to solids being noticed at first followed in time by dysphagia to liquids. On the other hand, in motor dysphagia, there is simultaneous dysphagia to both solids and liquids from the onset. Among the various causes of motor dysphagia, achalasia cardia remains the most recognised. Besides dysphagia, clinical features of achalasia include regurgitation, chest pain, hiccups, halitosis, weight loss, aspiration pneumonia and heartburn. The exact pathophysiology of this entity is unknown although increasing evidence suggests that loss of ganglion cells within the myenteric plexus supplying the smooth muscles of the esophagus (including the lower esophageal sphincter) to be responsible

The Ministry of Health and Family Welfare has issued the Standard Treatment Guidelines for Achalasia Cardia. Following are the major recommendations :

Case definition

Achalasia (a Greek term meaning "failure to relax") is a disease of unknown etiology characterised by loss of peristalsis in the distal esophagus and a failure of lower esophageal sphincter (LES) relaxation.

Incidence :

In the West, the incidence of achalasia cardia is reported to be approximately 1/100,000 population per year with prevalence being 7.1 to 13.4/100,000 population per year. There is hardly any data on the incidence and prevalence of the disease in India. A recent retrospective study from Lucknow conducted in a tertiary care centre reported that achalasia cardia constituted 77% of the patients who presented with motor dysphagia. This disease affects people of both genders equally and presents between 25 to 50 years age.

Differential Diagnosis :

Other motor disorders: Diffuse esophageal spasm

Nutcracker esophagus

Hypertensive LES

Diseases with distinct etiology but with functional consequences mimicking achalasia:

Pseudoachalasia (associated with malignancies and infiltrative diseases)

Chagas disease

Post surgical (following fundoplication and bariatric surgery)

Prevention and Counselling :

There are no particular preventive measures for achalasia cardia. Long standing dilatation of esophagus can result in development of squamous cell carcinoma of the esophagus although there are no recommendations for surveillance.

Optimal diagnostic criteria, Investigations, Treatment and Referral Criteria

Situation 1: At Secondary Hospital/Non-Metro situation

Clinical diagnosis :

Presence of insidious onset, gradually progressing dysphagia to both solids and liquids with varying combinations of regurgitation, chest pain, hiccups, halitosis, weight loss, heart burn and aspiration pneumonia may suggest achalasia cardia. The patient may give history of adopting certain manoeuvres like lifting the head or throwing the shoulders back to facilitate esophageal emptying.

Investigations :

• Chest X ray: Mediastinal widening with presence of air fluid level and absence of gastric bubble maybe seen.


• Barium swallow:

i) Dilated esophagus with a characteristic “bird beak” deformity in the lower esophagus

ii) Massively dilated esophagus may produce a “sigmoid” esophagus

• Upper GI Endoscopy: Typical findings include

i) Dilated esophagus with food or fluid residue

ii) Esophageal mucosa that is generally normal may show inflammation caused by retained food, pills or Candidiasis

iii) Contracted LES that can be negotiated with a gentle push of the endoscope.

Endoscopy is essential to rule out neoplasms in the cardia and the fundus.

Treatment :

Treatment of achalasia cardia maybe pharmacological, endoscopic or surgical.

• Surgical therapy is generally not performed in secondary hospitals.


• Endoscopic therapy can be attempted only if endoscopes, appropriate accessories, fluoroscopy and preferably, a surgical backup are available.


• Pharmacological therapy includes sublingual nitrates and calcium channel blockers given immediately before meals. These have the advantage of being non-invasive but they are not as effective as the other therapeutic options. Side effects and tachyphylaxis limit their use. Generally these are given to patients who cannot tolerate or are unwilling for invasive therapies.

Standard Operating Procedure

If adequate facilities of investigation and invasive therapy are available, patients in secondary hospitals are generally managed as outpatients. If endoscopic therapy is possible, this can be done as a day care procedure.

Referral criteria :

- Non-availability of endoscopy, endoscopic accessories, manometry, fluoroscopy and surgery

- Patients with systemic illness requiring specialised care

Situation 2: At super speciality facility in Metro Location where higher-end technology is available

Clinical diagnosis:

Presence of insidious onset, gradually progressing dysphagia to both solids and liquids with varying combinations of regurgitation, chest pain, hiccups, halitosis, weight loss, heart burn and aspiration pneumonia may suggest achalasia cardia. The patient may give history of adopting certain manoeuvres like lifting the head or throwing the shoulders back to facilitate esophageal emptying.

Investigations :

In addition to those discussed in situation 1, other investigations that can be performed include conventional manometry and high resolution manometry of the esophagus.

- Characteristic findings on conventional manometry include failure of the LES to relax, elevated basal LES pressure and aperistalsis of the esophageal body.

- High resolution manometry (HRM) of the esophagus is an advanced form of manometry. Based on HRM, achalasia is classifed into 3 subtypes:

Type 1(Classic) achalasia, where swallowing results in no significant change in esophageal pressurization.

Type II achalasia, where swallowing results in simultaneous pressurization that spans the entire length of the esophagus.

Type III (spastic) achalasia, where swallowing results in abnormal, lumen-obliterating contractions (spasms).

Responses to all types of achalasia treatment (both endoscopic and surgical) were best in type II patients and worst in type III patients.

- It is preferable to quantify the basal LES pressure by manometry prior to endotherapy or surgery to assess the efficacy of the same on follow up especially in the event of recurrence of symptoms.

Treatment :

Treatment of achalasia cardia maybe pharmacological, endoscopic or surgical.

• Surgical therapy is generally not performed in secondary hospitals.


• Endoscopic therapy can be attempted only if endoscopes, appropriate accessories, fluoroscopy and preferably, a surgical backup are available.


• Pharmacological therapy includes sublingual nitrates and calcium channel blockers given immediately before meals. These have the advantage of being non-invasive but they are not as effective as the other therapeutic options. Side effects and tachyphylaxis limit their use. Generally these are given to patients who cannot tolerate or are unwilling for invasive therapies.

The other forms of therapy are broadly divided into endoscopic or surgical.

Endoscopic therapy:

a. Pneumatic dilatation: Forceful stretching of the LES is accomplished using a Rigiflex pneumatic balloon dilator in a graded fashion under fluoroscopy. Although this is quite effective and can be done as a day care procedure, there is a 3-5% risk for perforation which can warrant additional surgery. If two sessions of pneumatic dilatation are unsuccessful, surgery should be performed.

b. Botulinum injection: Botulinum toxin A is injected into the LES so as to reduce its pressure. Its effect may not be as durable as that following pneumatic dilatation. Minor side effects of this procedure include rash and transient chest pain. It is ideal for high risk surgical patients in providing short term relief.

Surgery:

a. Open Heller’s myotomy (either transabdominal or transthoracic)

b. Minimally invasive (either laparoscopic or thoracoscopic)

Surgical therapy is more effective and long lasting than endotherapy. Reflux esophagitis can be a problem after surgery but can be ameliorated by performing an antireflux procedure (fundoplication). Overall mortality from Heller’s myotomy is less than 2%. Current evidence suggests that a laparoscopic approach is associated with similar efficacy, reduced morbidity, and shorter hospital stay when compared with surgical myotomy via other approaches. Direct referral for laparoscopic surgery can be considered for a low risk patient rather than pneumatic dilatation.

Standard Operating Procedure

Out Patient : Patients who do not have major comorbidities can be managed as outpatients. Post procedure patients can also be followed up.

In Patient : This would be required for those with major comorbidities that need stabilisation, for postoperative care and for monitoring those with anticipated complications following endotherapy. This may also be required for those who have copious food residue on initial endoscopy for clearing the contents (using a Ryle’s tube) before the next endoscopy.

Day Care : This would be required for patients who are dehydrated for stabilisation following which they can nourish themselves at home. Endotherapy can also be performed as a day care procedure.

Referral criteria:

A superspecialty center is expected to have the required diagnostic and therapeutic facilities for management of achalasia. However, a patient maybe referred if required equipment or expertise is unavailable.

Who does what and timelines?

a. Doctor :

If patient is sick (within 1 hour of arrival to hospital)

Initial resuscitation and assess if patient is dehydrated, has pneumonia, etc and requires admission.

If patient is otherwise well

History and clinical examination

Planning diagnostic tests

Discussion of condition and management options with patients and responsible well wishers and relatives and obtaining informed consent

Definitive treatment

Referral to other specialists in the same hospital or to a higher center

b. Nurse :

Measure vital signs and assess level of consciousness

Inquire chief complaints and inform the doctor accordingly

Obtain intravenous access for blood investigations

Carry out treatment orders as directed by the doctor

Assist the doctor in performing therapeutic procedures

c. Technician :

Maintain equipment required for emergency or elective care

Assist the doctor and the nurse during therapeutic procedures

Guidelines by The Ministry of Health and Family Welfare :

Dr Ashok Chacko Department of Gastroenterology Christian Medical College