Achalasia Cardia : An Overview

What is Achalasia Cardia?

Achalasia Cardia (AC) is a rare disease where the nerve supply to the food pipe ("esophagus") is defective, leading to a failure to relax when it is required or remaining contracted when it is not needed, eventually resulting in an eventually dis-coordinated esophagus leading to difficulty in swallowing. This condition is congenital but manifests by the age of around 40s which suggests that it progresses gradually. Coordinated nerve functions are mandatory for the "Esophagus" to propel swallowed food down to the stomach, and that mechanism is lost in AC. If untreated, this can lead to a completely redundant esophagus, resulting in complete difficulty swallowing and its consequences, such as weight loss, food regurgitation back into the chest, recurrent chest infection, or even esophageal cancer.

Read: Esophagus Cancer: Symptoms, Diagnosis & Treatment | Medanta

What are the symptoms of achalasia cardia?

Patients experience gradual difficulty in swallowing, which might be for both solid and liquid foods. Often, the symptoms are so subtle that family members notice you losing weight. The condition progresses over a period of years to complete difficulty in swallowing.

Typically, a habit is noticed by relatives when the patient takes lots of water with the solid food to swallow and takes a longer time to finish the meal.

Other symptoms are:

• Intermittent chest pain
• Coughing at night, hoarseness of voice
• Regurgitation of undigested food
• Bad odour in the mouth

Alarming symptoms

• Weight loss
• Bleeding

The major impacts on the health of the patient are significant nutritional debilitation and cachexia, irreversible damage of the lungs due to recurrent food regurgitation, and a crippling quality of life.

How Achalasia Cardia is diagnosed?

The symptoms described above are indicative and supportive. Symptoms persisting for more than 6 weeks should be an indication for immediate medical screening.

The following tests are recommended to check for achalasia:

• Barium wallow: it is like X- ray taken while the patient is given 100ml of barium liquid, which might taste like strawberry or orange juice, and the motility of esophagus is assessed. It is a OPD procedure, safe and takes 10 minutes to complete. It almost always results in a diagnosis in more than 80% of people.
• Esophageal manometry: Here a small wire, around 2 mm in size, is passed down the esophagus to assess the motility. It provides a map of the disease's scope and the best treatment options. It is not required in all cases, except when the diagnosis is in doubt and when surgical treatment is considered for the patient. It is a safe and day care procedure, no need for admission.
• Upper endoscopy: It is mandatory to do this test to exclude sinister diseases like cancer, which have similar presentations at times. An endoscope is passed from mouth under sedation to assess the inside of the esophagus lumen. It is a safe day care procedure, takes about 10 minutes, and you need to stay in the hospital for 4 to 6 hours.

What are the treatment options for Achalasia Cardia and what is best for me?

The objective of achalasia treatment is to restore coordinated movement of the esophagus to allow for effective swallowing.

Treatment options include:

• Medical management: The pills used to treat AC are largely ineffective.
• Endoscopic injections: Endoscopic injection of "Botulin toxin-BOTOX" relaxes the lower end food pipe sphincter and may lead to temporary symptomatic relief, but it does not last for more than 3 months and hence is not a suitable long term option. Hence, this form of treatment is only suitable for old, frail, and unfit individuals.
• Endoscopic dilatation: The process also breaks the lower end esophageal sphincter and helps to relax it and restore swallowing, but it needs to be repeated every 6 months, which is quite uncomfortable for a patient who is otherwise expected to live 20 years or more. So, in a nutshell, this option also does not provide a long term solution.
• Endoscopic myotomy (POEM): This is a modern day innovation where the spastic/contracted muscles of the esophagus that are causing difficulty in swallowing are torn endoscopically. The advantage here is no surgical scar and may be quick recovery, but at the cost of more chances of perforation of the esophagus which can be life threatening. There are high incidences of reflux of acid back into the lower esophagus and its attended consequences after POEM, which makes it an unfavourable option for long term symptom control in patients suffering from AC.
• Surgical myotomy "Heller’s Myotomy": This is the gold standard treatment among all the options currently. The procedure is done laparoscopically with keyholes. Spastic muscles are split under vision to restore swallowing and a stomach wrap is applied around the lower esophagus to prevent reflux of acid. The component of surgical myotomy addresses all the possible mechanical angles to make it the most safe, most durable, and most cost effective treatment. At 10 years after treatment, it offers the best symptom control and the best quality of life.
• Esophagectomy: When the disease is neglected for too long, the esophagus tube starts dilating and becoming redundant to a point where it cannot be salvaged. There is always a risk of cancer developing in such redundant esophagus, so surgical removal and food pipe are required. The continuity is restored by using the stomach as a neo-pipe.

It is noteworthy that in achalasia cardia, the basic problem of "defective nerves" cannot be altered. All the available treatment options aim to relieve symptoms by restoring effective swallowing. The treatments should be tailored according to the patients’ fitness and tolerability. Since most patients are young and fit, surgical myotomy remains the best available treatment so far, with the best and most durable outcome. Delay in seeking medical help eventually leads to end-stage esophagus which needs resection with its attended risks and hence "DON’T IGNORE IT".

Post-treatment follow-up

The patient is required to stay in the hospital for a couple of days following surgical myotomy and can resume a normal diet in 3 to 4 days and return to work in a week. The first follow-up will be in two weeks, and no long-term post-surgery medications will be required. There is no mandatory long term follow-up except when the patient notices some problem which is addressed on an actual basis.

Quality of life after surgery

More than 95% of patients report a good quality of life even after 5 years of treatment. Treatment failure or recurrence of symptoms is reported to be very low and in the range of 1% per year after 5 years, which suggests that even after 10 years of surgical myotomy, 90% of patients will be symptom free and enjoy a normal life. In the event of failure, it is largely managed with endoscopic dilatation and less likely to require reoperation.

Determinants of successful treatment

The single most determinant of a successful outcome in the treatment of AC is "Don't Ignore" the symptoms. Get treated on time and choose clinicians with adequate experience for the best long-term outcome.