Achalasia

Updated: April 26, 2024

Mail Whatsapp PDF Image

Background

Achalasia impacts how food travels from the esophagus to the stomach. The esophagus’s bottom muscle doesn’t fully relax. This causes trouble swallowing and chest pain. Doctors aren’t certain what causes achalasia. Tests like high-resolution manometry and functional luminal imaging probe topography identify achalasia subtypes. Type II seems to respond best to treatments such as per-oral endoscopic myotomy. Even so, diagnosing and managing achalasia remains challenging. It’s uncommon but deciding the right treatment approach and checking if it worked is difficult.

Achalasia is a condition in which food we take inside has a problem going from esophagus towards stomach where the muscle of the bottom is esophagus is not in relaxed condition. The cause or etiology of this is not known to healthcare providers also

Epidemiology

Achalasia is a rare illness, affecting about 1.6 out of 100,000 Americans each year, though some cases likely go undetected. For those with spinal cord injuries, the prevalence is much higher – around 84% suffer esophageal problems. Worldwide rates vary. Even after treatment, like pneumatic dilation or surgery, some patients experience setbacks. This disorder strikes both sexes equally, mostly hitting adults aged 25-60, though children can also develop it. Little inheritance pattern exists, but research continues into causes and cures.

Anatomy

Pathophysiology

Achalasia is an issue with how the esophagus moves food down. Peristalsis, the wave-like motion that pushes food down, also doesn’t work right. This causes difficulty swallowing and chest pain. It happens because of ongoing inflammation and a rise in cancer risk. The exact cause isn’t known. However, studies suggest an autoimmune problem plays a role. There’s a lack of specific nerve cells that control the esophagus muscles. This throws off the balance of chemical messengers—gene changes and tissue findings back up this autoimmune theory. Neural pathways break down, leading to too many sphincter contractions. That causes a blockage and widening of the esophagus. Inflammation and tissue damage happen in advanced cases. Inflammation targets the nerve plexus, and cells start dying off. Sphincter function shows losing inhibitory nerves leads to obstruction, widening, and an enlarged esophagus. Congenital absence of enteric neurons, like in Hirschsprung’s disease, highlights neural problems driving achalasia’s origins.

Etiology

Achalasia is a rare issue affecting the esophagus. It happens when nerves in the lower esophagus deteriorate. This includes the myenteric plexus and vagus nerve fibers. The deterioration causes a loss of inhibitory neurons, leading to malfunction. The exact cause isn’t clear. But potential factors are autoimmune processes, viral infections, genetics, and nerve damage. Most U.S. cases are primary idiopathic achalasia, meaning the cause is unknown. Secondary achalasia can result from other conditions. There’s evidence of an autoimmune component. Genetic studies link achalasia to immune-related DNA and gene variations. Familial cases are less common. They often follow marriages between relatives, suggesting recessive inheritance patterns. Dysfunction of ganglion cells, heredity, and autoimmune disorders likely contribute to achalasia’s causes

Genetics

Prognostic Factors

Clinical History

Achalasia causes problems swallowing food. Patients feel like food is stuck in their throat or chest. They might regurgitate undigested food. Chest pain, heartburn, and weight loss happen too. The main symptom is dysphagia, which starts with solids, then liquids. Other signs include hiccups and burping issues. Over time, night regurgitation leads to coughing and breathing problems. Chest pain occurs during swallowing or randomly. Achalasia usually starts slowly between ages 20-60. Symptoms worsen gradually over months/years. Mild-moderate weight loss is normal due to trouble eating. But rapid, severe weight loss in older adults may signal a tumor blocking food passage. Symptom types vary based on studied groups.

Physical Examination

A physical checkup of people with achalasia may show signs they’re too thin. This means the condition affected their weight and overall health. Doctors use the Eckardt symptom score to rank how bad achalasia symptoms are. It looks at four main things: weight loss, chest pain, trouble swallowing, and food coming back up. Each symptom gets a score from 0 to 3. The total possible score is 12. The scores put patients into different stages. Scores of 0-1 mean the disease is gone for now. Scores of 2-3 suggest treatment didn’t work well. This scoring system helps doctors understand symptoms, disease stages, and if treatments are working for achalasia patients.

Age group

Associated comorbidity

Associated activity

Acuity of presentation

A physical checkup of people with achalasia may show signs they’re too thin. This means the condition affected their weight and overall health. Doctors use the Eckardt symptom score to rank how bad achalasia symptoms are. It looks at four main things: weight loss, chest pain, trouble swallowing, and food coming back up. Each symptom gets a score from 0 to 3. The total possible score is 12. The scores put patients into different stages. Scores of 0-1 mean the disease is gone for now. Scores of 2-3 suggest treatment didn’t work well. This scoring system helps doctors understand symptoms, disease stages, and if treatments are working for achalasia patients.

Differential Diagnoses

  • Diffuse esophageal spasm
  • Paraesophageal hernia
  • Schatzki ring
  • Scleroderma
  • Stricture
  • Hiatal hernia
  • Gastroesophageal reflux disease

Laboratory Studies

Imaging Studies

Procedures

Histologic Findings

Staging

Treatment Paradigm

Achalasia needs diagnostic procedures such as x-rays, endoscopies, and research studies to confirm the specific type of achalasia. Subsequently, various therapy options, both non-surgical and surgical, can be explored. Non-invasive approaches include medications, infusions, or inflatable devices to ease the pressure of sphincter valve, facilitating easier movement of food. Alternatively, surgical procedures are available. A Heller myotomy involves cutting the muscle to expose the valve, while POEM uses an endoscope to cut fibres of muscle from within the esophagus – both aimed at aiding in smooth food transit. Following any treatment, regular follow-ups which are important to monitor potential complications or issues, ensuring efficacy.

by Stage

by Modality

Chemotherapy

Radiation Therapy

Surgical Interventions

Hormone Therapy

Immunotherapy

Hyperthermia

Photodynamic Therapy

Stem Cell Transplant

Targeted Therapy

Palliative Care

lifestyle-modifications-for-achalasia

Lifestyle changes can ease symptoms and better quality of life for people with achalasia. Eat smaller meals more often to prevent food from getting stuck in the esophagus, reducing dysphagia. Chew thoroughly and eat slowly to help to swallow and reduce lodged food risk. Avoid trigger foods like hard or dry foods. Stay upright after eating, and raise bed head to prevent reflux, coughing, and regurgitation at night. Manage stress through breathing exercises and meditation as stress worsens symptoms. Avoid alcohol and tobacco as these relax the lower esophageal sphincter, increasing symptoms. Tailor lifestyle changes to preferences, along with medical/surgical treatment. Consult a healthcare provider for personalized recommendations.

Administration of Pharmaceutical Agents with Drugs

  • Nifedipine: Calcium channel blockers are drugs that lower the pressure in the esophageal sphincter. They sometimes help ease achalasia symptoms. Drugs like nifedipine block calcium from entering muscle cells. This makes the sphincter relax, allowing easier food passage. However, these drugs have limited effectiveness. They are generally used when other treatments like dilatation or surgery seem risky. Nifedipine side effects can include low blood pressure, swollen legs and feet, and headaches. Patients can quickly build up tolerance to the drug’s effects. For some, nifedipine provides temporary achalasia relief. But symptom improvement is often incomplete and brief. So, calcium channel blockers are a secondary option to manage achalasia.

Use of nitrates in the treatment of individuals with achalasia

  • Isosorbide dinitrate: Nitrates aid in the treatment of achalasia. Isosorbide dinitrate is one example. These medications can relax sphincter muscles. They work by impacting smooth muscle fibers in the lower esophagus. Nitrates trigger cyclic GMP inside cells. This process relaxes vascular smooth muscle. As a result, pressure in the esophageal sphincter decreases. Relaxation helps food pass through more easily. However, like calcium channel blockers, nitrates have limits. They may not work well or cause side effects. So, nitrates are typically secondary options. They’re used it if other achalasia therapies weren’t suitable.

Use of Phosphodiesterase 5 (PDE-5) inhibitors in the treatment of individuals with achalasia

  • Sildenafil: Medicines called phosphodiesterase 5 inhibitors, like sildenafil, relax smooth muscles. Doctors tried these for achalasia. These drugs stop the enzyme PDE-5 from breaking down cGMP. Higher cGMP relaxes smooth muscles. In achalasia, the lower esophageal sphincter stays too tight. More relaxation could help open it up. But not enough proof shows sildenafil works well for achalasia symptoms. More studies must check if sildenafil and similar drugs are safe and good for treating this disease.

Nutritional plan in the management of individuals with achalasia

Having a good nutrition plan is essential for managing achalasia. It helps reduce symptoms, stop complications, and make sure you get enough nutrients. The plan means eating soft, moist foods that go down easy, like mashed potatoes, cooked veggies, and smoothies. But you should avoid trigger foods that are tough to swallow, like steak and dry bread. It’s best to have small meals more often since that lowers the chances of food getting stuck in your esophagus. Chewing well also help you swallow better. Staying hydrated by drinking liquids between meals is critical. And eating while sitting up straight can help food go down smoothly without reflux. Foods packed with vitamins, minerals, protein, and healthy fats are good choices. You might need supplements, too, if you’re not getting enough nutrients. Checking your weight and talking to your doctor or dietitian helps ensure your nutritional needs are met while managing achalasia symptoms and feeling your best.

surgical-intervention-in-the-management-of-individuals-with-achalasia

Achalasia is a condition treated with surgery often. The surgery involves laparoscopic Heller myotomy (LHM) and partial fundoplication. LHM with fundoplication works well. It relieves difficulty swallowing symptoms. The fundoplication prevents reflux after surgery. Studies prove adding partial fundoplication helps. It reduces reflux risk compared to myotomy alone. It’s cost-effective long-term too. For fundoplication, partial anterior fundoplication (Dor) is preferred. It’s simple and effective against reflux. Some minimally invasive options exist too. For example, peroral endoscopic myotomy (POEM). But POEM risks reflux without an antireflux procedure. Pneumatic dilation is another choice when surgery expertise lacks. But it often needs repeating and has recurrence risk. The treatment choice depends on factors. Like severity, patient preference, and specialized center availability offering POEM.

phases-of-management-in-the-treatment-of-individuals-with-achalasia

  • Diagnosis: Achalasia requires checking with tests to ensure the right diagnosis and severity.
  • Management: First, doctors try non-surgical paths like lifestyle changes, medicines, or procedures done with an endoscope.
  • Surgical interventions: But if those aren’t preferred or don’t work, surgery options are available. These include laparoscopic Heller myotomy (LHM) or peroral endoscopic myotomy (POEM).
  • Follow-up: After treatment, regular follow-ups help track how well it’s working and catch any issues.

Medication

Media Gallary

Content loading

Latest Posts

Achalasia

Updated : April 26, 2024

Mail Whatsapp PDF Image



Achalasia impacts how food travels from the esophagus to the stomach. The esophagus’s bottom muscle doesn’t fully relax. This causes trouble swallowing and chest pain. Doctors aren’t certain what causes achalasia. Tests like high-resolution manometry and functional luminal imaging probe topography identify achalasia subtypes. Type II seems to respond best to treatments such as per-oral endoscopic myotomy. Even so, diagnosing and managing achalasia remains challenging. It’s uncommon but deciding the right treatment approach and checking if it worked is difficult.

Achalasia is a condition in which food we take inside has a problem going from esophagus towards stomach where the muscle of the bottom is esophagus is not in relaxed condition. The cause or etiology of this is not known to healthcare providers also

Achalasia is a rare illness, affecting about 1.6 out of 100,000 Americans each year, though some cases likely go undetected. For those with spinal cord injuries, the prevalence is much higher – around 84% suffer esophageal problems. Worldwide rates vary. Even after treatment, like pneumatic dilation or surgery, some patients experience setbacks. This disorder strikes both sexes equally, mostly hitting adults aged 25-60, though children can also develop it. Little inheritance pattern exists, but research continues into causes and cures.

Achalasia is an issue with how the esophagus moves food down. Peristalsis, the wave-like motion that pushes food down, also doesn’t work right. This causes difficulty swallowing and chest pain. It happens because of ongoing inflammation and a rise in cancer risk. The exact cause isn’t known. However, studies suggest an autoimmune problem plays a role. There’s a lack of specific nerve cells that control the esophagus muscles. This throws off the balance of chemical messengers—gene changes and tissue findings back up this autoimmune theory. Neural pathways break down, leading to too many sphincter contractions. That causes a blockage and widening of the esophagus. Inflammation and tissue damage happen in advanced cases. Inflammation targets the nerve plexus, and cells start dying off. Sphincter function shows losing inhibitory nerves leads to obstruction, widening, and an enlarged esophagus. Congenital absence of enteric neurons, like in Hirschsprung’s disease, highlights neural problems driving achalasia’s origins.

Achalasia is a rare issue affecting the esophagus. It happens when nerves in the lower esophagus deteriorate. This includes the myenteric plexus and vagus nerve fibers. The deterioration causes a loss of inhibitory neurons, leading to malfunction. The exact cause isn’t clear. But potential factors are autoimmune processes, viral infections, genetics, and nerve damage. Most U.S. cases are primary idiopathic achalasia, meaning the cause is unknown. Secondary achalasia can result from other conditions. There’s evidence of an autoimmune component. Genetic studies link achalasia to immune-related DNA and gene variations. Familial cases are less common. They often follow marriages between relatives, suggesting recessive inheritance patterns. Dysfunction of ganglion cells, heredity, and autoimmune disorders likely contribute to achalasia’s causes

Achalasia causes problems swallowing food. Patients feel like food is stuck in their throat or chest. They might regurgitate undigested food. Chest pain, heartburn, and weight loss happen too. The main symptom is dysphagia, which starts with solids, then liquids. Other signs include hiccups and burping issues. Over time, night regurgitation leads to coughing and breathing problems. Chest pain occurs during swallowing or randomly. Achalasia usually starts slowly between ages 20-60. Symptoms worsen gradually over months/years. Mild-moderate weight loss is normal due to trouble eating. But rapid, severe weight loss in older adults may signal a tumor blocking food passage. Symptom types vary based on studied groups.

A physical checkup of people with achalasia may show signs they’re too thin. This means the condition affected their weight and overall health. Doctors use the Eckardt symptom score to rank how bad achalasia symptoms are. It looks at four main things: weight loss, chest pain, trouble swallowing, and food coming back up. Each symptom gets a score from 0 to 3. The total possible score is 12. The scores put patients into different stages. Scores of 0-1 mean the disease is gone for now. Scores of 2-3 suggest treatment didn’t work well. This scoring system helps doctors understand symptoms, disease stages, and if treatments are working for achalasia patients.

A physical checkup of people with achalasia may show signs they’re too thin. This means the condition affected their weight and overall health. Doctors use the Eckardt symptom score to rank how bad achalasia symptoms are. It looks at four main things: weight loss, chest pain, trouble swallowing, and food coming back up. Each symptom gets a score from 0 to 3. The total possible score is 12. The scores put patients into different stages. Scores of 0-1 mean the disease is gone for now. Scores of 2-3 suggest treatment didn’t work well. This scoring system helps doctors understand symptoms, disease stages, and if treatments are working for achalasia patients.

  • Diffuse esophageal spasm
  • Paraesophageal hernia
  • Schatzki ring
  • Scleroderma
  • Stricture
  • Hiatal hernia
  • Gastroesophageal reflux disease

Achalasia needs diagnostic procedures such as x-rays, endoscopies, and research studies to confirm the specific type of achalasia. Subsequently, various therapy options, both non-surgical and surgical, can be explored. Non-invasive approaches include medications, infusions, or inflatable devices to ease the pressure of sphincter valve, facilitating easier movement of food. Alternatively, surgical procedures are available. A Heller myotomy involves cutting the muscle to expose the valve, while POEM uses an endoscope to cut fibres of muscle from within the esophagus – both aimed at aiding in smooth food transit. Following any treatment, regular follow-ups which are important to monitor potential complications or issues, ensuring efficacy.

Gastroenterology

Lifestyle changes can ease symptoms and better quality of life for people with achalasia. Eat smaller meals more often to prevent food from getting stuck in the esophagus, reducing dysphagia. Chew thoroughly and eat slowly to help to swallow and reduce lodged food risk. Avoid trigger foods like hard or dry foods. Stay upright after eating, and raise bed head to prevent reflux, coughing, and regurgitation at night. Manage stress through breathing exercises and meditation as stress worsens symptoms. Avoid alcohol and tobacco as these relax the lower esophageal sphincter, increasing symptoms. Tailor lifestyle changes to preferences, along with medical/surgical treatment. Consult a healthcare provider for personalized recommendations.

Gastroenterology

  • Nifedipine: Calcium channel blockers are drugs that lower the pressure in the esophageal sphincter. They sometimes help ease achalasia symptoms. Drugs like nifedipine block calcium from entering muscle cells. This makes the sphincter relax, allowing easier food passage. However, these drugs have limited effectiveness. They are generally used when other treatments like dilatation or surgery seem risky. Nifedipine side effects can include low blood pressure, swollen legs and feet, and headaches. Patients can quickly build up tolerance to the drug’s effects. For some, nifedipine provides temporary achalasia relief. But symptom improvement is often incomplete and brief. So, calcium channel blockers are a secondary option to manage achalasia.

Gastroenterology

  • Isosorbide dinitrate: Nitrates aid in the treatment of achalasia. Isosorbide dinitrate is one example. These medications can relax sphincter muscles. They work by impacting smooth muscle fibers in the lower esophagus. Nitrates trigger cyclic GMP inside cells. This process relaxes vascular smooth muscle. As a result, pressure in the esophageal sphincter decreases. Relaxation helps food pass through more easily. However, like calcium channel blockers, nitrates have limits. They may not work well or cause side effects. So, nitrates are typically secondary options. They’re used it if other achalasia therapies weren’t suitable.

  • Sildenafil: Medicines called phosphodiesterase 5 inhibitors, like sildenafil, relax smooth muscles. Doctors tried these for achalasia. These drugs stop the enzyme PDE-5 from breaking down cGMP. Higher cGMP relaxes smooth muscles. In achalasia, the lower esophageal sphincter stays too tight. More relaxation could help open it up. But not enough proof shows sildenafil works well for achalasia symptoms. More studies must check if sildenafil and similar drugs are safe and good for treating this disease.

Gastroenterology

Having a good nutrition plan is essential for managing achalasia. It helps reduce symptoms, stop complications, and make sure you get enough nutrients. The plan means eating soft, moist foods that go down easy, like mashed potatoes, cooked veggies, and smoothies. But you should avoid trigger foods that are tough to swallow, like steak and dry bread. It’s best to have small meals more often since that lowers the chances of food getting stuck in your esophagus. Chewing well also help you swallow better. Staying hydrated by drinking liquids between meals is critical. And eating while sitting up straight can help food go down smoothly without reflux. Foods packed with vitamins, minerals, protein, and healthy fats are good choices. You might need supplements, too, if you’re not getting enough nutrients. Checking your weight and talking to your doctor or dietitian helps ensure your nutritional needs are met while managing achalasia symptoms and feeling your best.

Gastroenterology

Achalasia is a condition treated with surgery often. The surgery involves laparoscopic Heller myotomy (LHM) and partial fundoplication. LHM with fundoplication works well. It relieves difficulty swallowing symptoms. The fundoplication prevents reflux after surgery. Studies prove adding partial fundoplication helps. It reduces reflux risk compared to myotomy alone. It’s cost-effective long-term too. For fundoplication, partial anterior fundoplication (Dor) is preferred. It’s simple and effective against reflux. Some minimally invasive options exist too. For example, peroral endoscopic myotomy (POEM). But POEM risks reflux without an antireflux procedure. Pneumatic dilation is another choice when surgery expertise lacks. But it often needs repeating and has recurrence risk. The treatment choice depends on factors. Like severity, patient preference, and specialized center availability offering POEM.

Gastroenterology

  • Diagnosis: Achalasia requires checking with tests to ensure the right diagnosis and severity.
  • Management: First, doctors try non-surgical paths like lifestyle changes, medicines, or procedures done with an endoscope.
  • Surgical interventions: But if those aren’t preferred or don’t work, surgery options are available. These include laparoscopic Heller myotomy (LHM) or peroral endoscopic myotomy (POEM).
  • Follow-up: After treatment, regular follow-ups help track how well it’s working and catch any issues.

Free CME credits

Both our subscription plans include Free CME/CPD AMA PRA Category 1 credits.

Digital Certificate PDF

On course completion, you will receive a full-sized presentation quality digital certificate.

medtigo Simulation

A dynamic medical simulation platform designed to train healthcare professionals and students to effectively run code situations through an immersive hands-on experience in a live, interactive 3D environment.

medtigo Points

medtigo points is our unique point redemption system created to award users for interacting on our site. These points can be redeemed for special discounts on the medtigo marketplace as well as towards the membership cost itself.
 
  • Registration with medtigo = 10 points
  • 1 visit to medtigo’s website = 1 point
  • Interacting with medtigo posts (through comments/clinical cases etc.) = 5 points
  • Attempting a game = 1 point
  • Community Forum post/reply = 5 points

    *Redemption of points can occur only through the medtigo marketplace, courses, or simulation system. Money will not be credited to your bank account. 10 points = $1.

All Your Certificates in One Place

When you have your licenses, certificates and CMEs in one place, it's easier to track your career growth. You can easily share these with hospitals as well, using your medtigo app.

Our Certificate Courses