Achalasia: when the oesophagus stops letting food through

Most people have never heard of the oesophageal valve — the lower oesophageal sphincter — until it stops working. In achalasia, this muscular ring at the junction between the oesophagus and stomach fails to relax when food arrives, creating a functional obstruction that neither acid-suppressing medication nor dietary modification can fix. The oesophagus above gradually dilates, filling with retained food and saliva, and patients spend years managing symptoms that look deceptively like reflux.

Achalasia is rare — affecting around 10 per 100,000 Australians — but the diagnostic delay is long: an average of four to five years from first symptoms. Most patients carry a reflux diagnosis and a proton pump inhibitor prescription for much of that time. The key clinical clue that separates achalasia from reflux is dysphagia for both solids and liquids simultaneously from the beginning. Reflux and peptic stricture cause trouble with solids first; with achalasia, liquids are equally obstructed because the problem is not a narrowing but a failure of the valve to open.

A. Core clinical — the AU general-practice framework

Recognising achalasia in general practice

The history is characteristically gradual — symptoms build over months to years. Patients describe food sitting behind the breastbone, the need to drink liquid with meals to push food through, and sometimes regurgitation of undigested food hours later or on lying flat. Nocturnal regurgitation causes coughing, aspiration, and recurrent chest infections in some patients.

Chest pain — particularly in type III (spastic) achalasia — can be the dominant symptom. Weight loss is slow and cumulative, reflecting reduced intake due to the difficulty of every meal. Halitosis from retained food in a dilated oesophagus is often reported.

The pattern that should prompt consideration of achalasia in general practice:

• Dysphagia for solids and liquids from the beginning (not progressive solids-to-liquids)
• Symptoms that do not clearly improve on proton pump inhibitors
• Regurgitation of undigested food (not acidic digested material)
• Retrosternal chest pain or discomfort, particularly postprandially
• Gradual, unexplained weight loss over months to years
• Recurrent aspiration pneumonia without other cause

The diagnostic workup

Achalasia diagnosis is a three-step process coordinated through gastroenterology. No single investigation is sufficient on its own.

Step 1 — Gastroscopy (MBS item 30473) is mandatory first. The primary purpose is not to diagnose achalasia but to exclude pseudoachalasia — malignancy at the gastro-oesophageal junction that produces identical symptoms. Pseudoachalasia accounts for up to 5% of suspected cases, particularly in patients over 55 with rapid symptom onset and disproportionate weight loss. Biopsies are taken from the oesophagus and the gastro-oesophageal junction. In established achalasia, the gastroscopy typically shows a dilated oesophagus with retained saliva and food despite fasting, normal mucosa, and a valve that offers resistance but “pops” through — these findings suggest the diagnosis but do not confirm it.

Step 2 — Barium swallow provides functional imaging: the classic “bird-beak” tapering at the lower oesophageal sphincter with proximal dilation and poor emptying. A timed barium oesophagram — measuring how much contrast remains at 1 and 5 minutes — also quantifies emptying and establishes a baseline for monitoring treatment response.

Step 3 — High-resolution manometry is the gold standard (Chicago Classification version 4.0, Yadlapati 2021). It measures pressure along the oesophagus during swallowing and diagnoses achalasia by finding impaired lower oesophageal sphincter relaxation (integrated relaxation pressure >15 mmHg) plus absent oesophageal peristalsis. Three subtypes are identified:

• Type I — classical; quiescent oesophageal body
• Type II — panoesophageal pressurisation; best treatment outcomes (~95% response)
• Type III (spastic) — premature contractions; most challenging to treat (30–70% response with standard therapies; POEM preferred)

High-resolution manometry is available at Australian tertiary motility centres: Westmead and Royal Prince Alfred (Sydney), Royal Melbourne, Royal Brisbane and Women’s, and Royal Adelaide hospitals.

General practice management before specialist review

While awaiting gastroenterology review, general practice can support patients with:

• Dietary advice — eat slowly, chew thoroughly, eat soft foods during severe phases, avoid lying flat for 2–3 hours after meals
• Head-of-bed elevation (10–15 cm blocks or a wedge pillow) to reduce nocturnal regurgitation and aspiration risk
• Proton pump inhibitors — though they do not address the motility problem, many achalasia patients have reflux symptoms (especially post-treatment) where they provide genuine symptom relief
• Nutritional support and dietitian referral if weight loss is significant

Short-term bridging pharmacotherapy with sublingual nifedipine (10–30 mg before meals) or isosorbide dinitrate can partially relax the lower oesophageal sphincter, providing modest symptomatic relief while awaiting definitive treatment — though the evidence is limited and side effects (headache, hypotension) constrain their use.

B. Treatment — options and outcomes

There is no cure for achalasia; all treatments work by disrupting or weakening the lower oesophageal sphincter to allow passive gravity-assisted oesophageal emptying. The ACG 2020 guideline and SAGES 2024 update provide the current evidence base.

Types I and II achalasia — three equally effective options

Pneumatic dilation uses an endoscopic balloon to forcibly disrupt the sphincter muscle fibres. It is performed as a day-procedure under sedation, often in staged dilations of increasing diameter. Five-year efficacy of ~70–85% is comparable to surgery; the main risk is perforation (~1–4%), which may require emergency surgical repair. It can be repeated and is a reasonable first choice for patients who prefer to avoid surgery.

Laparoscopic Heller myotomy surgically divides the lower oesophageal sphincter muscle through keyhole incisions and adds a partial anti-reflux wrap (Dor or Toupet fundoplication) to reduce postoperative reflux. Five-year efficacy approaches 85–90%. The anti-reflux wrap is the reason post-Heller reflux rates (~25–30%) are lower than post-POEM (~50%).

POEM — peroral endoscopic myotomy — is a technically demanding endoscopic procedure in which the oesophageal muscle is cut from the inside via a submucosal tunnel, without any external incisions. Five-year efficacy is comparable to Heller myotomy at 85–95%, with faster recovery (Werner NEJM 2019). The trade-off is a higher post-procedure reflux rate (~50%) requiring long-term proton pump inhibitors. POEM is available at specialist centres across Australia.

For Types I and II, choice between these three options is individualised based on patient preference, surgical risk, centre expertise, and whether the patient accepts a higher reflux burden (POEM) versus a longer recovery (Heller myotomy).

Type III achalasia — POEM preferred

The SAGES 2024 guideline update recommends POEM for type III because the myotomy can be extended into the spastic body of the oesophagus — something not technically achievable with standard Heller myotomy. This produces better symptom relief in spastic disease than dilation or surgery alone.

When definitive treatment is not possible

Botulinum toxin injection at endoscopy temporarily relaxes the sphincter for 6–12 months. It is appropriate for patients who are too unwell for definitive treatment, as a palliative option, or as a bridge while awaiting surgery. However, prior botulinum toxin injection can cause scarring that complicates subsequent Heller myotomy, so it should be used selectively.

Pharmacotherapy (nifedipine, nitrates) is a last resort for patients unable to have any procedure.

Post-treatment surveillance

All achalasia patients require long-term follow-up. Reflux management — proton pump inhibitors, often lifelong — is standard after any procedure. Oesophageal emptying is monitored with repeat barium swallow at 3 and 12 months after treatment. Symptom assessment using the Eckardt score (0–12, covering dysphagia, regurgitation, chest pain, and weight loss; ≤3 = treatment success) guides ongoing management.

From ten years after diagnosis, surveillance gastroscopy every three years is recommended by most specialist centres to detect oesophageal squamous cell carcinoma, which carries a roughly threefold higher risk in long-standing achalasia compared with the general population. Whether surveillance improves outcomes is not yet proven by randomised trial, but it is considered reasonable by the treating specialist community.

C. Pseudoachalasia — why gastroscopy comes first

The most important diagnosis not to miss in suspected achalasia is pseudoachalasia — a malignancy at the gastro-oesophageal junction that compresses or infiltrates the sphincter and produces identical symptoms. It accounts for around 5% of cases, and the proportion rises in patients over 55 with rapid onset (over weeks rather than months), disproportionate weight loss, and less dilated oesophagus than typical achalasia.

Gastric adenocarcinoma is the most common cause, but oesophageal cancer, lymphoma, and metastatic disease can all produce the same picture. Manometry cannot distinguish pseudoachalasia from true achalasia — the pressure traces are identical. Endoscopy with biopsies and, where uncertain, CT imaging and endoscopic ultrasound are needed to make the distinction.

The implication for general practice: every patient referred with suspected achalasia requires gastroscopy before manometry, not as a confirmatory test for achalasia, but as a cancer exclusion.

D. Australian operations

Achalasia is managed in tertiary centres. The general practice role is recognition, initial investigation, supportive care, and referral. High-resolution manometry services are concentrated at Westmead, Royal Prince Alfred, Royal Melbourne, Royal Brisbane and Women’s, and Royal Adelaide hospitals.

MBS access for the diagnostic pathway includes gastroscopy item 30473 for the initial investigation; therapeutic gastroscopy items for dilation and botulinum injection; oesophageal manometry under item 11820; and specialist gastroenterology consult under standard specialist items. POEM and Heller myotomy are performed under hospital surgical items at tertiary centres and are accessible through the public hospital system.

PBS coverage for post-treatment management: proton pump inhibitors (pantoprazole, esomeprazole) are available on the PBS General Schedule for documented reflux. Sublingual nifedipine and isosorbide dinitrate — used for palliative bridging — are available on the PBS General Schedule for cardiovascular indications, though their use in achalasia is off-label.

Dietitian referral under GPCCMP (MBS item 965) is appropriate for patients with significant malnutrition or cachexia before and after treatment. Speech pathology under the same plan can assist with swallow strategies to reduce aspiration risk.

The Gastroenterological Society of Australia provides patient resources. An informal patient support community is accessible through the Achalasia Australia Facebook group.

E. Special populations

Older adults (over 70). Pneumatic dilation is often preferred over surgery in older patients with higher operative risk. POEM avoids an anaesthetic of the same duration as Heller myotomy and has no external wound, but the post-procedure reflux burden still requires long-term medication. Botulinum toxin is a reasonable palliative option in frail older adults where definitive treatment carries unacceptable risk.

Pregnancy. Achalasia is rare in pregnancy but can worsen due to progesterone-mediated relaxation of gastrointestinal smooth muscle and increased intra-abdominal pressure. Conservative management — dietary modification, head-of-bed elevation, proton pump inhibitors — is preferred during pregnancy. Elective definitive treatment is deferred until after delivery.

Paediatric achalasia. Rare, but presentations in children and adolescents do occur. Paediatric gastroenterology input is essential. POEM is increasingly used in paediatric centres with appropriate expertise.

Patients from Latin American backgrounds. Chagas disease — caused by the parasite Trypanosoma cruzi, endemic in Central and South America — can produce an identical clinical picture by destroying the myenteric plexus, mimicking primary achalasia. Serology for Chagas disease is appropriate in any patient from an endemic region presenting with achalasia-like symptoms.

When to escalate

All confirmed or strongly suspected achalasia requires specialist gastroenterology review — there is no general practice treatment for the condition itself. Escalate urgently when:

• Complete inability to swallow or acute food bolus impaction — Emergency Department
• Suspected aspiration pneumonia — assessment and treatment, then prompt specialist referral
• Unexplained weight loss or gastroscopy findings raising concern for pseudoachalasia — urgent oncology or upper-GI surgical review
• Post-procedure haematemesis, severe chest pain, or fever — consider oesophageal perforation — Emergency Department immediately

What this article is and is not

This is general health information drawn from the Therapeutic Guidelines (eTG), Australian Medicines Handbook, ACG 2020 achalasia guideline, Chicago Classification v4.0, and SAGES 2024. It is not personal medical advice and does not establish a doctor–patient relationship. Decisions about investigation and treatment are made with your own GP and gastroenterologist.

Consumer resources: HealthDirect — achalasia, GESA patient resources, Better Health Channel.

---

Sources cited

1. Therapeutic Guidelines (eTG) — Gastrointestinal
2. Australian Medicines Handbook (AMH)
3. NPS MedicineWise
4. Gastroenterological Society of Australia (GESA)
5. HealthDirect — Achalasia
6. Better Health Channel
7. Vaezi MF et al — ACG Clinical Guidelines: Achalasia (AJG 2020)
8. Yadlapati R et al — Chicago Classification v4.0 (Neurogastroenterol Motil 2021)
9. Stefanidis D et al — SAGES 2024 POEM guideline update
10. Werner YB et al — POEM vs Heller myotomy (NEJM 2019)
11. Boeckxstaens GE et al — Pneumatic dilation vs Heller myotomy (NEJM 2011)