Medicine chapters
Dysphagia, achalasia and carcinoma of the oesophagus
A clear approach to difficulty swallowing, with the classic features, tests and treatment of achalasia, other motility disorders, webs and rings, and carcinoma of the oesophagus.
- Dysphagia is an alarm symptom: progressive dysphagia for solids then liquids with weight loss in an older patient is oesophageal cancer until proven otherwise, and endoscopy with biopsy comes first.
- Achalasia causes dysphagia to solids and liquids from the start, with a bird's beak on barium and failed LOS relaxation plus aperistalsis on manometry.
- Squamous cancer (upper/middle third; smoking, alcohol, achalasia, Plummer–Vinson) differs from adenocarcinoma (lower third; GORD, Barrett, obesity).
On this page
Overview
Dysphagia means difficulty swallowing: food sticks on its way down. Odynophagia means pain on swallowing and suggests inflammation (infection, reflux, pills) or cancer.
Dysphagia is always taken seriously because it is an alarm feature for cancer. A careful history usually tells you where the problem is and whether it is a blockage or a muscle/nerve problem before any test is done.
This chapter covers the approach to dysphagia, achalasia and other motility disorders, webs and rings, and carcinoma of the oesophagus. Reflux disease and Barrett oesophagus have their own chapter.
Approach to dysphagia
Step 1: oropharyngeal or oesophageal?
| Feature | Oropharyngeal (transfer) dysphagia | Oesophageal dysphagia |
|---|---|---|
| Problem | Starting the swallow | Food sticks after swallowing |
| Symptoms | Choking, coughing, nasal regurgitation, drooling | Food sticks behind the sternum |
| Causes | Stroke, motor neurone disease (bulbar palsy), myasthenia, Parkinson disease, muscular dystrophy, pharyngeal pouch | Stricture, cancer, achalasia, spasm, rings, webs |
Step 2: mechanical or motility?
| Feature | Mechanical obstruction | Motility disorder |
|---|---|---|
| Food type | Solids first, later liquids | Solids and liquids from the onset |
| Course | Often progressive | Often intermittent, long history |
| Examples | Peptic stricture, carcinoma, rings, webs, external compression | Achalasia, diffuse spasm, systemic sclerosis |
"Solid then liquid = something in the lumen; both at once = the muscle is the problem."
Step 3: pattern clues
| Clue | Think of |
|---|---|
| Progressive, weeks to months, weight loss, older smoker | Carcinoma |
| Long history of heartburn, slowly worsening solids | Peptic stricture |
| Long history, both solids and liquids, night regurgitation, little weight loss | Achalasia |
| Intermittent food bolus sticking | Schatzki (lower oesophageal) ring; eosinophilic oesophagitis |
| Gurgling neck lump, regurgitation of undigested food, bad breath | Pharyngeal pouch (Zenker diverticulum) |
| Middle-aged woman with iron deficiency, glossitis | Plummer–Vinson syndrome |
| Raynaud phenomenon, tight skin, heartburn | Systemic sclerosis |
| Chest pain with dysphagia, corkscrew barium | Diffuse oesophageal spasm |
| Painful swallowing in HIV, chemotherapy, steroids | Candida, herpes simplex, CMV oesophagitis |
| Young man with atopy, food impaction | Eosinophilic oesophagitis |
Step 4: investigations
- Upper GI endoscopy (OGD) with biopsy: the first test in most adults, to exclude cancer and take biopsies.
- Barium swallow: useful for motility problems, pouches and webs; done before endoscopy if a pharyngeal pouch is suspected (risk of perforation).
- High-resolution oesophageal manometry: confirms motility disorders such as achalasia.
- CT chest and abdomen, endoscopic ultrasound and PET-CT: staging of cancer.
- Blood count for iron deficiency.
Do not give a PPI and wait in a patient with new dysphagia. Dysphagia is an alarm symptom and needs urgent endoscopy.
Achalasia
Definition and cause
Achalasia ("failure to relax") has two defects:
- The lower oesophageal sphincter (LOS) fails to relax on swallowing, and its resting pressure is often raised.
- The body of the oesophagus loses peristalsis (aperistalsis).
The cause is unknown (autoimmune, viral and degenerative theories). There is inflammation of the myenteric (Auerbach) plexus with loss of ganglion cells, mainly the inhibitory neurons that release nitric oxide and VIP. Cholinergic (excitatory) supply is largely preserved.
A similar picture is seen in Chagas disease (Trypanosoma cruzi, South America).
It is uncommon, has no sex preference, and may start at almost any age, though it is rare in children.
Clinical features
- Dysphagia for both solids and liquids from the start, usually over a long period.
- Regurgitation of undigested food, especially at night; this may cause cough and aspiration pneumonia.
- Chest pain (sometimes mistaken for cardiac pain), particularly early in the disease.
- Weight loss is usually mild.
Investigations
| Test | Finding |
|---|---|
| Chest X-ray | Widened mediastinum, air-fluid level behind the heart, absent gastric fundal air bubble |
| Barium swallow | Dilated oesophagus tapering smoothly to a "bird's beak" at the lower end; no peristalsis |
| Manometry (gold standard) | Aperistalsis and incomplete LOS relaxation, often high LOS pressure |
| Endoscopy | Food residue; tight cardia but the scope passes with little resistance; done to exclude cancer |
| CT | Excludes a tumour at the cardia |
Manometry confirms achalasia, but endoscopy must always come first to rule out a tumour at the cardia.
High-resolution manometry (Chicago classification) divides achalasia into type I (classic, no contractions), type II (pressurisation of the whole oesophagus) and type III (spastic).
Pseudoachalasia: a tumour at the gastro-oesophageal junction (usually adenocarcinoma of the cardia) can copy achalasia on barium and even manometry. Suspect it in an older patient with a short history and marked weight loss, and always do endoscopy and CT first.
Management
All treatments are palliative: they open the sphincter but do not restore peristalsis.
| Option | Notes |
|---|---|
| Drugs (nitrates, calcium-channel blockers, sildenafil) | Weak, short-lived effect; used while waiting or in unfit patients |
| Botulinum toxin injection into the LOS | Safe and simple; effect wears off within months; good for frail, elderly patients |
| Pneumatic balloon dilatation | Works initially in about 80%; many need repeat dilatation; risk of perforation |
| Laparoscopic Heller myotomy | Surgical treatment of choice; usually combined with a partial fundoplication to limit reflux |
| Peroral endoscopic myotomy (POEM) | Myotomy done through an endoscope; good early results |
After any treatment, reflux is common because the sphincter barrier is weakened.
Complications
- Aspiration pneumonia.
- Squamous cell carcinoma of the oesophagus (risk persists even after treatment; about 7% after 25 years in one series), probably from chronic stasis and irritation.
- Epiphrenic diverticulum.
Achalasia vs systemic sclerosis: both have absent peristalsis, but achalasia has a tight, high-pressure LOS that will not relax, whereas scleroderma has a weak, low-pressure LOS, so it causes severe reflux and strictures.
Other motility disorders
- Diffuse oesophageal spasm: uncoordinated, simultaneous contractions with normal LOS relaxation; chest pain and dysphagia; "corkscrew" oesophagus on barium. Treat with PPI if reflux, nitrates, calcium-channel blockers.
- Nutcracker (hypercontractile) oesophagus: very high-pressure peristalsis (over 200 mmHg); chest pain more than dysphagia.
- Systemic sclerosis: smooth muscle replaced by fibrous tissue; weak peristalsis and low LOS pressure; reflux and stricture. Treat as reflux.
- Neurological and muscle diseases: stroke, MND, myasthenia gravis, myotonic and oculopharyngeal dystrophy, diabetes.
Webs, rings, pouches and strictures
- Plummer–Vinson (Paterson–Brown-Kelly) syndrome: a postcricoid web with iron deficiency anaemia, glossitis and angular stomatitis; mainly women. Treat iron deficiency; the web often breaks during endoscopy. It is a risk factor for postcricoid and oesophageal squamous cancer.
- Schatzki ring (lower oesophageal mucosal "B" ring): at the squamocolumnar junction; intermittent bolus obstruction, typically with a large mouthful of meat or bread. Dilatation if needed.
- Pharyngeal pouch (Zenker diverticulum): above the upper sphincter, through Killian dehiscence; elderly; regurgitation of old food, halitosis, gurgling neck swelling. Diagnose by barium swallow. Treat surgically or endoscopically.
- Benign stricture: commonest cause is peptic (reflux); others are corrosives, radiotherapy, variceal sclerotherapy and long-term nasogastric tubes. Treated by endoscopic dilatation and long-term PPI.
- Eosinophilic oesophagitis: atopic young men; food impaction; rings, furrows and white specks at endoscopy; biopsies show many eosinophils. Treat with swallowed topical steroids (fluticasone, budesonide), PPI or elimination diets.
Oesophageal rupture and tears
| Condition | Mechanism | Key facts |
|---|---|---|
| Mallory–Weiss tear | Mucosal tear at the gastro-oesophageal junction after retching | Small haematemesis; usually stops by itself |
| Boerhaave syndrome | Full-thickness rupture after violent vomiting, often after alcohol | Severe chest pain, collapse, surgical emphysema; diagnose with water-soluble contrast swallow or CT; urgent surgery; high mortality |
Carcinoma of the oesophagus
Types and sites
| Feature | Squamous cell carcinoma (SCC) | Adenocarcinoma |
|---|---|---|
| Site | Upper and middle third | Lower third and cardia |
| Precursor | Squamous dysplasia | Barrett oesophagus (intestinal metaplasia) |
| Risk factors | Smoking, heavy alcohol, achalasia, Plummer–Vinson, corrosive stricture, tylosis, very hot drinks, poor diet, coeliac disease | Long-standing GORD, Barrett, obesity, smoking |
| Epidemiology | Most common type worldwide and in Asia, including Pakistan | Rising fast and now the main type in Western countries |
Tylosis is a rare autosomal dominant thickening of the palms and soles with a very high risk of oesophageal SCC.
The most common oesophageal cancer worldwide is squamous cell carcinoma; the most common type in the West today is adenocarcinoma, driven by reflux and obesity.
Clinical features
- Usually aged 60–70; men more than women.
- Progressive, relentless dysphagia: solids first, then liquids within weeks.
- Weight loss and anorexia.
- Pain from food impaction or invasion of nearby structures.
- Late: hoarse voice (recurrent laryngeal nerve), cough on swallowing (fistula into the airway), aspiration, supraclavicular node, liver metastases.
Investigations
- Endoscopy with biopsy: confirms the diagnosis.
- CT of chest and abdomen: local spread, nodes and metastases.
- Endoscopic ultrasound: best for depth of invasion (T stage) and nearby nodes.
- PET-CT: detects distant spread; laparoscopy for tumours at the cardia.
- Barium swallow shows an irregular narrowing with shouldered edges; it is useful when a motility disorder is also in question.
Management
Treatment depends on stage and fitness, through a multidisciplinary team.
- Early (mucosal) cancer: endoscopic mucosal resection.
- Localised, operable disease: oesophagectomy, usually after neoadjuvant chemotherapy or chemoradiotherapy. Less than half of patients are operable at presentation.
- Definitive chemoradiotherapy is an option, particularly for SCC.
- Palliation (most patients): self-expanding metal stent to restore swallowing; brachytherapy, laser or radiotherapy; nutritional support; palliative care.
Prognosis is poor because most patients present late: overall 5-year survival is around 15%.
Expect: history features that separate mechanical from motility dysphagia, chest X-ray and barium signs of achalasia (absent gastric bubble, fluid level, bird's beak), manometry as the diagnostic test, loss of nitric oxide neurons, treatment options (botulinum, pneumatic dilatation, Heller myotomy, POEM), pseudoachalasia, Plummer–Vinson and pharyngeal pouch, risk factors and sites of SCC versus adenocarcinoma, the first investigation for suspected cancer, endoscopic ultrasound for staging, and stenting for palliation.
Quick revision
- Dysphagia is an alarm symptom: endoscopy first.
- Solids then liquids, progressive, weight loss: cancer or stricture.
- Solids and liquids from onset: achalasia.
- Achalasia: absent gastric bubble, bird's beak, aperistalsis with non-relaxing LOS on manometry.
- Achalasia treatment: botulinum (frail), balloon dilatation, Heller myotomy (choice), POEM.
- Elderly, short history, "achalasia": exclude cardia cancer.
- Scleroderma: weak LOS and reflux; achalasia: tight LOS.
- Plummer–Vinson: web + iron deficiency + glossitis; SCC risk.
- SCC upper/middle third (smoking, alcohol, achalasia); adenocarcinoma lower third (GORD, Barrett, obesity).
Written by The House Job from standard references (Kumar and Clark's Clinical Medicine; Bailey & Love's Short Practice of Surgery).
FAQ
Why is the gastric air bubble absent in achalasia?
Swallowed air cannot pass the tight lower oesophageal sphincter, so little gas reaches the stomach, while the dilated oesophagus may show an air-fluid level in the chest.
What is the best test to confirm achalasia?
Oesophageal manometry, which shows absent peristalsis and failure of the lower oesophageal sphincter to relax; endoscopy is still needed to exclude cancer.
Which oesophageal cancer is linked to Barrett oesophagus?
Adenocarcinoma of the lower third of the oesophagus, which develops through intestinal metaplasia and dysplasia caused by long-standing reflux.
How is swallowing restored in inoperable oesophageal cancer?
Most often with a self-expanding metal stent placed endoscopically, sometimes combined with brachytherapy or radiotherapy.