Achalasia Cardia Diagnosis, Treatment and Cost

Diagnosis of achalasia cardia depends on a combination of clinical history, physical examination, and specific diagnostic tests, which help the gastroenterologist to reach a conclusive diagnosis. Achalasia is suspected in patients presenting with progressive dysphagia (difficulty swallowing) and regurgitation (backward flow of contents). The gastroenterologist considers the following before selecting the appropriate tests to diagnose achalasia cardia:

• Medical history
• Physical examination
  
  

Medical history

• In diagnosing achalasia cardia, a gastroenterologist first takes a detailed history of the patient’s symptoms. Most people report dysphagia (difficulty swallowing), which often starts with solid foods and later includes liquids. Nearly 70–97% experience problems with both solids and liquids when they first see a doctor. Regurgitation of undigested food is also common. 
• More than half report chest pain, although treatment of the swallowing problem rarely stops the pain completely. As the condition advances, symptoms may include regurgitation with risk of aspiration, night-time coughing, heartburn, and weight loss from difficulty eating, often rapid due to swallowing problems. 
• Less common symptoms include hiccups or the inability to burp. In severe cases, the enlarged esophagus can press on the windpipe, causing noisy breathing or a “bullfrog neck” appearance. Some patients also develop lung problems from repeated aspiration.
• Recording these symptoms and their timeline is a crucial first step in identifying achalasia and planning further diagnostic tests.
  
  

Physical examination

• While achalasia is mainly diagnosed through history and specialized tests, physical examination can still play an important role in assessing disease severity and treatment outcomes. Gastroenterologists often use the Eckardt symptom score during evaluation. 
• This tool rates four key symptoms, such as weight loss, chest pain, dysphagia (difficulty swallowing), and regurgitation. On a scale from 0 to 3, the highest possible score is 12. Lower scores (0–1) suggest remission, while higher scores (4 or more) indicate active disease or treatment failure. 
• Using this scoring system during examination helps in classifying the disease stage, monitoring progress, and guiding further management decisions. Physical exam usually does not confirm achalasia (as findings are minimal), but the score is used as a functional clinical measure.

✅Diagnostic Evaluation of Achalasia Cardia

Based on the above-mentioned information, a gastroenterologist advises specific diagnostic tests to confirm the condition and rule out other causes of swallowing difficulties. The following are the tests that might be recommended to diagnose achalasia cardia:

• Barium swallow
• Upper endoscopy (EGD)
• Esophageal manometry
• 24-hour pH monitoring
• Functional lumen imaging probe (FLIP)
• Endoscopic ultrasound (EUS)
  
  

Barium swallow

• The barium swallow is often the first imaging study performed when achalasia is suspected. In this test, the patient swallows a radiopaque barium solution while X-ray fluoroscopy is used to visualize the esophagus. 
• In achalasia, the classic appearance is a “bird’s-beak” tapering at the gastroesophageal junction (GEJ) due to the non-relaxing lower esophageal sphincter (LES), along with a dilated proximal esophagus that may be straight or tortuous in chronic cases. Delayed passage of barium into the stomach is a hallmark feature. 
• The barium swallow also helps rule out structural causes of dysphagia, such as benign strictures from reflux or caustic injury, Schatzki rings, esophageal webs, diverticula, or external compression by mediastinal masses or vascular anomalies. It provides a functional overview of esophageal emptying and morphology, making it a valuable screening tool before more invasive tests.
  
  

Upper endoscopy (Esophagogastroduodenoscopy- EGD)

• Upper endoscopy is essential in all patients with suspected achalasia to directly visualize the esophageal lumen, mucosa, and gastroesophageal junction, and to rule out malignancy or other obstructive lesions. In achalasia, the endoscopist often encounters a dilated esophagus filled with saliva, retained food, and sometimes liquid. 
• The LES may appear tightly closed but usually opens with gentle pressure of the endoscope, and the mucosa is generally normal unless there is stasis-induced inflammation. Endoscopy plays a crucial role in excluding pseudoachalasia caused by gastroesophageal junction adenocarcinoma or submucosal tumors.
• It also helps rule out benign strictures, esophagitis (including eosinophilic or infectious causes), and varices. If there are “red flag” features such as rapid onset of symptoms, marked weight loss, or mucosal irregularity at the GEJ, targeted biopsies and additional imaging (CT or endoscopic ultrasound) are performed to exclude cancer.
  
  

Esophageal manometry

• High-resolution esophageal manometry is the gold standard for confirming achalasia and subtyping the disorder according to the Chicago Classification. A thin catheter with pressure sensors is passed through the nose into the esophagus to measure the pressure patterns of the LES and the body of the esophagus during swallowing. 
• In achalasia, manometry shows increased integrated relaxation pressure (IRP), indicating incomplete LES relaxation, as well as the absence of normal peristalsis. Achalasia is categorised into three types based on the pattern of contractions. 
• This test not only confirms achalasia but also helps distinguish it from other motility disorders such as distal esophageal spasm, ineffective esophageal motility, and non-achalasia esophagogastric junction outflow obstruction. The subtype information from HRM is important because it influences treatment choice and prognosis.
  
  

24-hour pH monitoring

• This test measures the acidity in the esophagus over a full day to evaluate gastroesophageal reflux disease (GERD) as a potential cause of symptoms. 
• In achalasia, acid exposure is generally normal because the LES is hypertonic and does not allow frequent reflux. This test is most useful when there is diagnostic uncertainty between achalasia and reflux-related motility disorders, or when symptoms overlap (such as chest pain and regurgitation). 
• It helps rule out GERD as a primary diagnosis and prevents misdiagnosis that could lead to inappropriate anti-reflux surgery or medical therapy. In post-treatment follow-up, 24-hour pH monitoring may also be used to detect reflux that can occur after interventions like Heller myotomy or POEM.
• This test is reserved for cases with overlapping reflux-like symptoms or post-treatment reflux surveillance.
  
  

Functional lumen imaging probe (FLIP)

• The functional lumen imaging probe is an advanced diagnostic tool consisting of a catheter with a balloon filled with conductive saline and embedded with multiple impedance planimetry sensors along its length. As the balloon is inflated, the device measures cross-sectional area and intraluminal pressure to calculate the distensibility index (DI) of the esophagogastric junction. 
• In achalasia, the DI is usually reduced (often less than 2 mm²/mmHg at a 60 mL fill), indicating a stiff, non-relaxing LES. FLIP can also generate real-time topographic maps of esophageal contractility, which can help detect abnormal motility patterns when manometry results are inconclusive or unobtainable. 
• Besides confirming reduced EGJ distensibility in achalasia, FLIP can help rule out normal motility, assess other esophageal outflow disorders, and monitor immediate therapeutic response during procedures such as POEM or pneumatic dilation.
  
  

Endoscopic ultrasound (EUS)

Endoscopic ultrasound combines endoscopy with high-frequency ultrasound imaging to provide detailed views of the esophageal wall layers and surrounding mediastinal structures. In the evaluation of achalasia, EUS is particularly useful when there is a suspicion of pseudoachalasia caused by submucosal tumors, infiltrating cancers, or extrinsic compression at the gastroesophageal junction.

The main and valuable role of EUS in achalasia diagnosis is to rule out pseudoachalasia, a condition that mimics achalasia and is often caused by tumors or masses near or involving the EGJ. EUS can detect tumors originating from the muscular layer or outside the EGJ and evaluate inflammation in the esophageal wall. It helps distinguish idiopathic achalasia from secondary causes due to malignancies or other external compressions.

✅Staging of Achalasia Cardia

Achalasia cardia radiology stages, as seen on a barium swallow study, are generally classified based on the diameter and shape of the esophagus, reflecting the progression of the disease:

• Stage I: The esophagus diameter is less than or equal to 4 cm, indicating little dilatation. At this early stage, the esophagus remains generally normal in size with no noticeable structural changes, indicating a mild condition. 
• Stage II: The esophageal diameter ranges between 4 and 6 cm, indicating moderate dilation. The esophagus generally remains straight without significant distortion of shape, reflecting progression, but it is still a less advanced stage.
• Stage III: The esophageal diameter is greater than or equal to 6 cm, with marked dilation present. The esophagus remains straight but is considerably dilated, indicating more advanced disease where muscle tone and peristalsis are severely compromised.
• Stage IV (End-stage disease): The esophageal diameter remains above 6 cm, but the esophagus is now sigmoid-shaped and tortuous, indicating end-stage achalasia. This stage shows severe esophageal remodeling and disruption of normal anatomy, with significant esophageal dilation and twisting.

✅Differential Diagnosis of Achalasia Cardia

When evaluating a patient with symptoms like difficulty swallowing and regurgitation, various other diseases can be mistaken for achalasia cardia. The following are usually considered as differential diagnoses of achalasia cardia:

• Esophageal spasm: This is a motility disorder characterized by uncoordinated or simultaneous esophageal contractions, causing chest pain and intermittent dysphagia. Manometry shows preserved LES relaxation (unlike achalasia).
• Scleroderma: This is a connective tissue disease causing smooth muscle atrophy and fibrosis in the distal esophagus, leading to aperistalsis (absence of normal esophageal contractions) and a hypotensive, incompetent LES (opposite of achalasia). Often associated with severe reflux.
• Gastroesophageal reflux disease (GERD): Chronic acid reflux causing mucosal injury, strictures, or motility changes. LES pressure is low or normal; pH monitoring confirms excess acid exposure.
• Stricture: This is a fixed narrowing of the esophagus from causes such as peptic injury, caustic ingestion, or radiation. It produces progressive dysphagia, especially for solids.
• Schatzki ring: This is a thin mucosal ring at the gastroesophageal junction, causing intermittent solid food dysphagia, often relieved by careful chewing or dilation.
• Hiatal hernia: This is a protrusion of part of the stomach through the diaphragm into the thorax. It may cause reflux symptoms or mechanical obstruction.
• Paraesophageal hernia: This is a type of hiatal hernia where the gastric fundus herniates alongside the esophagus, risking volvulus or strangulation, and may present with dysphagia or postprandial fullness.

✅Considerations for a gastroenterologist for treating achalasia cardia

When treating patients with achalasia cardia, a gastroenterologist considers the following to achieve better outcomes:

• Before starting treatment, it is important to confirm the diagnosis and exclude secondary causes such as malignancy (pseudoachalasia) by performing a combination of barium swallow, upper GI endoscopy, and esophageal manometry. Additionally, if available, the functional lumen imaging probe (FLIP) can provide further insights into esophagogastric junction function.
• The treatment choice - whether pneumatic balloon dilation, laparoscopic Heller myotomy, peroral endoscopic myotomy (POEM), or neurotoxin injection- needs to be tailored to the patient's age, comorbid conditions, achalasia subtype (Type I, II, or III), and personal preferences.
• Patients with comorbidities or those who are poor surgical candidates may benefit from less invasive options like neurotoxin injection or pneumatic dilation, despite the higher likelihood of recurrence.
• For younger patients or those with type III (spastic) achalasia, POEM may be favoured because of its effectiveness in managing spastic esophageal disorders.
• The potential for post-procedure GERD needed to be taken into account, particularly following myotomy procedures. Patients need to be informed about reflux symptoms and may require prolonged acid suppression therapy.
• Ongoing follow-up is important to monitor for symptom recurrence, complications such as reflux or aspiration, and to evaluate the necessity for additional interventions.
• Lastly, educating patients on the chronic nature of achalasia, the possibility of symptom recurrence, and the importance of regular follow-up care is essential for long-term management.

✅Achalasia Cardia Treatment Goals

The key goals of therapy for achalasia cardia are: 

• To reduce or minimize symptoms such as chest pain, heartburn, dysphagia, and regurgitation. 
• To enhance esophageal emptying and restore efficient swallowing. 
• To avoid further dilatation and structural damage to the esophagus.
• To reduce the risk of complications such as esophagitis and aspiration.
• To minimize treatment-related side effects and ensure patient safety.
• To achieve durable, long-term control of the disease.

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