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Achalasia Cardia : An Overview

Introduction

Achalasia is one of the rarest swallowing disorders. Achalasia is an oesophageal disorder that prevents food and liquid from moving smoothly between the oesophagus and stomach. Adults between 25 and 60 years old typically develop this condition. Men's chances of getting achalasia are high compared to women. The condition's symptoms can become quite severe. Patients often struggle to swallow both liquids and solids, experience chest pain, heartburn, food regurgitation, and face collateral weight loss.

This article explains what achalasia is, its causes, diagnosis, treatment options and practical advice to manage daily life with this long-term condition.

What is Achalasia?

Achalasia (also called cardiospasm) is a rare neurogenic disorder affecting the oesophagus. The condition causes two simultaneous problems. Your oesophagus muscles cannot contract properly, and the lower oesophageal sphincter (muscle ring at the bottom) fails to relax.

The disorder creates a blockage where food enters the stomach. People with this condition often regurgitate their food and drinks because they get stuck in the oesophagus. 

Symptoms

People with achalasia notice warning signs that develop slowly over several years. Many don't realise their symptoms are connected to one condition.

Difficulty swallowing ranks as the most common symptom. Food seems to get stuck in the throat before reaching the stomach. The condition makes it hard to swallow both liquids and solids as it gets worse.

Food often comes back up because it can't move down to the stomach properly. This undigested food might return unexpectedly, especially when someone lies down at night.

Many people feel intense chest pain that can be bad enough to wake them up. Abnormal contractions of the oesophagus muscles cause this pain.

Other symptoms are:

  • Heartburn that won't go away

  • Nighttime coughing spells

  • Choking while eating

  • Excess saliva or throwing up

  • Steady weight loss over time

These symptoms look similar to gastroesophageal reflux disorder (GERD), but they happen for different reasons. GERD occurs when the lower oesophageal sphincter is too loose, while achalasia makes it too tight.

Causes

Medical researchers still can't solve the mystery of what triggers achalasia. Scientists haven't found the exact cause of this condition, unlike many other digestive disorders.

The condition usually starts when nerve cells that control the swallowing muscles in the oesophagus vanish over time. Doctors still don't know why this happens.

Scientists have several theories about what might cause achalasia:

  • Autoimmune reactions that make the body attack its healthy oesophageal cells

  • Genetic factors that run in families

  • Viral infections like herpes simplex, human papillomavirus, and measles that might set it off

  • Inflammation that targets the oesophageal muscle-controlling nerves

People with achalasia face a higher risk of developing other autoimmune conditions like type 1 diabetes, rheumatoid arthritis, and lupus.

Some rare conditions can trigger "secondary achalasia" with matching symptoms. These include Chagas disease (a parasitic infection common in Central and South America), certain cancers, and uncommon disorders like sarcoidosis.

Types of Achalasia

The Chicago Classification system helps doctors categorise achalasia into three types. 

Type I (Classic Achalasia)

  • Minimal or absent oesophageal contractions

  • No pressurisation (pressure <30 mmHg)

  • Food moves down mostly by gravity

  • Young patients show this type more often

  • Makes up 20-40% of cases

Type II (Achalasia with Pressurisation)

  • Complete failure of peristalsis

  • Pan-oesophageal pressurisation (>30 mmHg)

  • Represents 50-70% of cases, making it the most common form

  • Patients experience more intense symptoms than Type I

  • Treatment works best 

Type III (Spastic Achalasia)

  • Shows spastic contractions in the distal oesophagus

  • Rare variant that affects only 5% of patients

  • Patients face the most challenging symptoms

  • Chest pain can feel similar to heart attack symptoms

Each type shares one common trait - the lower oesophageal sphincter doesn't relax properly. Identifying the specific subtype is vital because treatment success rates vary substantially between these categories.

Diagnosis

Doctors need a careful approach to diagnose achalasia because its symptoms often look like other digestive disorders like gastroesophageal reflux disease (GERD). A physical examination and medical history review precede specialised tests to get a full picture.

High-resolution oesophageal manometry stands as the gold standard to confirm achalasia. This test shows pressure changes in your oesophagus during swallowing. 

Other diagnostic tests:

  • Barium Oesophagram - X-rays capture detailed images of your swallowing process while you drink liquid barium

  • Upper Endoscopy - A camera-equipped flexible tube provides a direct view of your oesophagus to rule out tumours or strictures.

  • Chest X-ray - Shows oesophagus's abnormal dilation.

Treatment Options

Doctors treat achalasia by relieving symptoms since no cure exists yet. Several treatment options are available each with distinct benefits.

  • Medications: Calcium channel blockers and nitrates help reduce LES pressure. 

  • Botulinum toxin injections target the LES to block acetylcholine release and temporarily paralyse the sphincter muscles. 

  • Pneumatic dilation uses an inflated balloon at the LES to break muscle fibres. This non-surgical option is the most effective non-surgical treatment. 

  • Surgical approaches include:

  • Laparoscopic Heller Myotomy (LHM): The gold standard treatment with 90% success rates

  • Peroral Endoscopic Myotomy (POEM): A newer, minimally invasive method shows great results

Complications

Achalasia patients face several serious health issues that can damage their overall health and quality of life if left untreated. Common complications are:

  • Weight loss and malnutrition 

  • Nutritional deficiencies 

  • Aspiration pneumonia (food and liquid that back up in the oesophagus might enter the lungs and cause life-threatening inflammation)

  • Oesophageal perforation 

  • Megaesophagus (progressive dilation) 

  • GERD-like symptoms 

  • Food gets stuck and requires medical help

  • Patients feel bloated and uncomfortable after meals

  • Cancer 

  • Return of the disease even after successful treatment

Living with Achalasia

People with achalasia face drastic changes in their daily lives. Simple changes in eating habits can make a big difference in managing discomfort.

Food choices play a crucial role. Soft, moist options work best for most people:

  • Smoothies and protein shakes help during low appetite periods

  • Mashed potatoes, yoghurt, and tender meats are easier to handle

  • Sauces and gravies make swallowing more manageable

  • Room temperature or warm drinks feel better than ice-cold ones

The right eating patterns make a difference too. Small bites, really thorough chewing, and slow eating reduce the risk of food getting stuck. Food moves downward better when you sit upright at a table. Waiting about three hours after meals before lying down helps prevent regurgitation.

Smaller, more frequent meals throughout the day often work better than three large ones. This approach helps minimise discomfort and improve nutritional intake.

A raised head position during sleep, using bed risers or a wedge pillow, prevents undigested food from flowing toward the throat. This simple change helps avoid nighttime coughing and choking episodes.

Achalasia affects each person differently. Something that triggers problems for one person might not affect another. A food diary helps identify your personal triggers. Working with doctors helps develop individual-specific strategies to maintain proper nutrition.


FAQs

  1. What is achalasia (cardiospasm)?

Achalasia (also called cardiospasm) is a rare neurogenic disorder that affects the oesophagus. The condition creates two problems at the same time: the oesophageal muscles don't contract properly, and the lower oesophageal sphincter (LES) can't relax normally during swallowing. The condition makes it hard to move food from the oesophagus to the stomach.

  1. What are the main symptoms of achalasia?

People find it harder to swallow both solids and liquids as time goes on. Other symptoms are:

  • Undigested food coming back up

  • Chest pain that's severe enough to wake them up

  • Burning sensations in the chest

  • Coughing, especially at night

  • Weight loss

  1. What causes achalasia?

Doctors haven't found the exact cause yet. Research shows that nerve cells in the oesophageal muscles die off, which seems to be the mechanism. Studies point to viral infections, autoimmune reactions, and genetic factors as possible triggers.

  1. How is achalasia diagnosed?

Doctors use high-resolution manometry as the gold standard test. They start with an endoscopy to rule out other conditions. A barium swallow test reveals the characteristic "bird's beak" appearance at the lower oesophageal sphincter.

  1. Are there different types of achalasia?

The condition has three distinct types based on manometric patterns:

  • Type I (classic): minimal oesophageal contractility (20-40% of cases)

  • Type II: panesophageal pressurisation (50-70% of cases)

  • Type III (spastic): premature or spastic contractions (5% of cases)

  1. Can achalasia cause difficulty swallowing liquids and solids?

Of course. Most swallowing disorders only affect solids, but achalasia makes it hard to swallow both liquids and solids from the beginning.

  1. What treatments are available for achalasia?

Doctors can treat achalasia with medications like calcium channel blockers and nitrates, botulinum toxin injections, pneumatic dilation, or surgical procedures such as laparoscopic Heller myotomy and POEM (Peroral Endoscopic Myotomy). These treatments help manage symptoms, but they can't restore normal peristalsis permanently.

  1. Is surgery required for achalasia?

Not everyone with achalasia needs surgery. Doctors recommend procedures like Heller myotomy or POEM (Peroral Endoscopic Myotomy) when other treatments don't work. These surgical options offer the most lasting relief from symptoms. Most patients feel better right after surgery, and the benefits can last ten years or more. The removal of the lower oesophagus, called esophagectomy, is an option for patients with end-stage achalasia who don't respond to other treatments.

  1. Can achalasia be managed with medications?

Medications don't work very well for achalasia patients. Calcium channel blockers and nitrates can help food pass more easily by relaxing the lower oesophageal sphincter temporarily. Patients should take these drugs 10-30 minutes before meals. The medicine's effectiveness tends to decrease over time, and side effects like headaches, low blood pressure, and swollen ankles are common. Doctors usually prescribe these medications as short-term solutions or for patients who can't handle more definitive treatments.

10.What lifestyle changes help with achalasia symptoms?

Simple daily adjustments can make life more comfortable. These are:

  • Eat smaller meals slowly and chew thoroughly

  • Add water to meals to help food go down easier

  • Keep a 3-4 hour gap between solid food and bedtime

  • Lift your head during sleep using bed risers or wedge pillows

  • Switch to liquid or powder medications instead of pills

Dr. Sarvjeet Kumar
Gastrosciences
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