Case Study

A patient with dysphagia

Prepared for BMJ Learning (Nov 2006) in association with Gut, an international journal of Gastroenterology and Hepatology

Learning outcomes

On completion of this module you should know :

  1. the common causes of dysphagia
  2. the safest and best way to make a diagnosis in such a patient and
  3. the best way to manage the patient when you have made a diagnosis.

About the author 

Dr Kang has published widely on acid-peptic disorders and the epidemiology of gastrointestinal diseases.  Previously an academic gastroenterologist with the National University of Singapore, he is now Lead Consultant Gastroenterologist at St George’s Hospital, London.  

Why I wrote this module

Achalasia of the cardia is an uncommon yet eminently treatable cause of dysphagia which is often not recognised because of lack of awareness about this condition. The correct diagnosis is usually suggested by the history and able to be confirmed by appropriate investigations.

Case history

A 63 year old year woman presented to the gastroenterology clinic with vomiting and weight loss. This had been going on for 4 months. She had a history of Hodgkin’s disease which had been treated 17 years previously. She had a staging splenectomy at this time. She had also suffered two previous strokes leaving her with left-sided weakness.  Abdominal examination was normal.  Upper endoscopy was normal and she received a trial of anti-emetics. A few weeks later she was admitted as an emergency with continuing vomiting.

MCQ1: Which of the following conditions can generally be considered to have been excluded by a normal upper endoscopy?

  1. Pharyngeal pouch
  2. Oesophageal web
  3. Peptic oesophageal stricture
  4. Achalasia of the cardia
Correct Answer: 3
  1. Endoscopy is not the diagnostic modality of choice if a pharyngeal pouch is suspected.  The endoscope may go into the pouch and there may be a risk of perforation.  Alternatively the diagnosis may be missed if the pouch is bypassed during intubation of the oesophagus.
  2. An oesophageal web is an uncommon cause of dysphagia.  It usually occurs in the upper or middle oesophagus and can be associated with iron deficiency anaemia.  It is best seen on barium swallow.   Often upper endoscopy is apparently normal but the web is ruptured by the passage of the endoscope: the procedure can then be therapeutic.
  3. A benign peptic oesophageal stricture is easily diagnosed at endoscopy and its differentiation from oesophageal carcinoma is normally straightforward. 
  4. Upper endoscopy is often reported to be normal in early achalasia of the cardia.  But an experienced observer can frequently notice a subtle degree of resistance at the oesophageal-cardiac junction, which suddenly gives way as the sphincter pressure is overcome.  In more advanced disease there is also dilatation of the oesophagus, with food and fluid residue, and tertiary contractions may be evident.

Learning bite

It is important to remember that dysphagia can occasionally be misinterpreted as vomiting, both by the patient and by the doctor. 

Given the patient’s past medical history, a full neurological assessment was performed.
The patient was unsteady on her feet but there was no evidence of raised intra-cranial pressure nor of other new signs (apart from her mild left sided weakness). CT scan of the head showed the old infarct but was otherwise normal. 

A barium meal was performed and showed possible coarsening of the duodenal mucosa (subsequently thought to be an artefact from delayed filling). Upper gastrointestinal endoscopy was therefore performed to obtain duodenal histology.  On this occasion there was obstruction at the gastro-oesophageal junction with a mild degree of dilatation.  Solid residue was present in the oesophagus and a degree of resistance was felt at the cardio-oesophageal junction.  

MCQ2: Which of the following is the LEAST likely diagnosis?

  1. Achalasia of the cardia
  2. Pseudo-achalasia
  3. Recurrence of lymphoma
  4. Peptic stricture

Correct Answer: 4

  1. Even with a history of a normal endoscopy only several weeks ago, achalasia or pseudo-achalasia would seem to be a possibility. 
  2. Even with a history of a normal endoscopy only several weeks ago, achalasia or pseudo-achalasia would seem to be a possibility. 
  3. Extrinsic compression from recurrence of lymphoma would also be a possibility. 
  4. Peptic stricture would be most unlikely since there was no suggestion of reflux oesophagitis

Biopsies of the duodenum and gastro-oesophageal junction were normal. The patient was unable to tolerate oesophageal manometry, but barium swallow showed aperistalsis and hold-up of contrast at the cardio-oesophageal junction (Figure 1). 

Figure 1: Barium swallow in achalasia

The oesophagus is dilated, with a fluid level and tapers
down smoothly below to a bird's beak appearance.

An oesophageal manometry tracing from another patient with achalasia is shown in figure 2.

The patient’s hospital records established that the patient had not undergone radiotherapy at the time of treatment for her Hodgkin’s disease. A few small axillary lymph nodes were found and biopsied, and bone marrow biopsy was also performed.  These were all normal. CT scan of the chest and abdomen showed no abnormal masses or lymph nodes.  The oesophageal wall was however diffusely thickened (Figure 3) and this was confirmed on endoscopic ultrasonography.  During the course of the investigations she was fed via an enteral tube.

MCQ3: What is now the most likely diagnosis?

  1. probable achalasia
  2. probable pseudo-achalasia
  3. probable intramural oesophageal lymphoma
  4. insufficient information for a conclusion to be drawn

Correct answer 1

  1. Unfortunately oesophageal manometry was not able to be performed in this case, but the history and the barium swallow were ultimately diagnostic.
  2. Pseudo-achalasia is unlikely in view of her normal investigations.
  3. Intramural oesophageal lymphoma is very rare and also unlikely.
  4. There is sufficient information to come to a diagnosis of probable achalasia.

MCQ4: In achalasia there is typically

  1. loss of the submucosal ganglion cells
  2. a mild neutrophilic infiltration just above the oesophago-gastric junction
  3. an increase in the latency gradient in the oesophageal body
  4. onset of symptoms in 60s
  5. a steadily increasing incidence in Caucasian populations

Correct Answer 4

  1. Achalasia is the best-defined primary oesophageal motility disorder.  There is loss of ganglion cells in the myenteric (Auerbach’s) plexus.
  2. The loss of ganglion cells is accompanied by an inflammatory response consisting of T lymphocytes, eosinophils and mast cells and neural fibrosis.  The end result of these changes is a selective loss of post-ganglionic inhibitory neurons containing nitric oxide and vasoactive intestinal polypeptide.  Stimulatory cholinergic neurons remain intact, resulting in a high basal lower oesophageal sphincter pressure and insufficient relaxation. 
  3. Aperistalsis is caused by loss of the latency gradient that permits sequential contractions along the oesophageal body, a process which is mediated by nitric oxide.  Possible aetiological factors leading to these inflammatory changes include auto-immunity, infection, degenerative changes and genetic influences. (1;2).
  4. Achalasia predominantly affects people over the age of 60 to 70 years.  Several series have a female predominance, but this could be due to there being more older women than older men.  It is uncommon, with a prevalence rate of approximately 10 cases per 100,000 people among white populations. 
  5. Its incidence seems static over the last 50 years.  The condition appears less common among Orientals and Africans but case ascertainment can be a factor(3).

MCQ5: In the clinical presentation of achalasia:

  1. dysphagia is commonly for solids only
  2. postural manoeuvres may help to aid swallowing
  3. heartburn does not normally occur
  4. the symptoms are usually of sudden onset

Correct Answer 2

  1. Dysphagia for both solids and liquids is the most consistent symptom in achalasia, occurring in almost all patients(1;4;5). 
  2. Individual patients often develop a range of strategies to live with their dysphagia, such as arching the neck and shoulders, raising the arms and standing or sitting up straight during the meal, and walking around afterwards(4).  Many patients report that they eat much more slowly than other family members. 
  3. Other symptoms include chest pain, which is reported by 17-95% of patients and is more common in individuals with mild disease.  Regurgitation occurs in 59-81% of patients, typically later in the course of the disease when the oesophagus starts to dilate.  Bland, undigested food or saliva retained in the oesophagus regurgitates in the recumbent position and wakes the patient from sleep with coughing and choking, sometimes leading to a chest infection. Heartburn is a frequent complaint despite the absence of acid reflux from the stomach.  Weight loss to some degree occurs in most patients.  Halitosis is sometimes reported(1;4;5). Retro-sternal chest pain in achalasia typically affects younger patients and is usually crampy in nature, often waking the patient from sleep and sometimes relieved by ingestion of cold drinks.  In many but not all instances the pain is thought to arise from high amplitude, repetitive simultaneous contractions in the oesophagus.  The chest pain of achalasia often persists even when dysphagia has improved with successful dilatation(5).  The pathogenesis of heartburn in achalasia is obscure.  Although gastro-oesophageal reflux may occur after successful pneumatic dilatation of myotomy, and a low pH can result from fermentation of stagnant oesophageal contents, heartburn can occur in untreated patients who would not be expected to have gastro-oesophageal reflux(5).   It is therefore important to confirm gastro-oesophageal reflux and exclude achalasia by manometry before subjecting patients to fundo-plication, especially in non-erosive gastro-oesophageal reflux disease.
  4. Symptoms are often slowly progressive over months or years, during which time many patients may adapt to significant symptoms by slowly altering their diet or eating habits.  Furthermore, barium swallow or endoscopy can be normal in patients with early disease.  The mean delay in the diagnosis of achalasia, said to be about five years, has not changed in recent decades(5).

MCQ 6:Which of the following statements is correct about the diagnosis of achalasia?

  1. it is usually obvious with barium swallow or endoscopy
  2. there are often simultaneous contractions of high amplitude on manometry
  3. the lower oesophageal sphincter pressure is always raised
  4. lower oesophageal sphincter relaxation is always impaired

Correct answer 4

  1. In patients with early disease the barium swallow may be reported as normal.  The oesophagus is not dilated at this stage but there is a loss of peristalsis.  The distal oesophagus is characterised by a smooth tapering resembling a bird’s beak.  With progressive disease the oesophagus dilates and retains food and saliva, producing an air-fluid level which may be visible on a plain X-ray(1). Upper gastrointestinal endoscopy is also often reported to be normal in patients with early disease, but there may be resistance at the cardio-oesophageal junction, which suddenly gives way to increasing pressure from the endoscope. Oesophageal mucosa obscured by retained saliva with a frothy appearance, even after an overnight fast, can be a give-away.  With advanced disease the oesophagus is dilated and tortuous with large amounts of food and fluid residue, the so-called ‘sigmoid oesophagus’.  Endoscopy can be challenging in such patients and sometimes a clear fluid diet for several days or oesophageal lavage is required before endoscopy to avoid aspiration and to allow adequate views to be obtained.
  2. Oesophageal manometry is the key test for the diagnosis of achalasia (Fig 2)(1). There is aperistalsis, with all swallows followed by simultaneous contractions, typically of low amplitude, although the term ‘vigorous achalasia’ is used when normal or high amplitude contractions are seen. 
  3. The lower oesophageal sphincter pressure is usually raised, but may be within normal limits. 
  4. Lower oesophageal sphincter relaxation is always abnormal in achalasia.  The majority of patients have absent or incomplete relaxation with wet swallows.  In 20-30% of patients, the relaxation is complete to the gastric baseline but are of abnormally short duration. 

Pseudo-achalasia

Some tumours of the gastro-oesophageal junction can produce pseudo-achalasia.  This is an uncommon condition: only five patients were identified among a large series of 206 patients with achalasia diagnosed on manometry. (14).  It is usually due to submucosal infiltration by gastric or oesophageal cancer. 

Compared to patients with true achalasia, those with pseudo-achalasia are likely

  1. to be older
  2. to have a shorter duration of dysphagia
  3. to have substantial weight loss and
  4. to have lost the weight more quickly (14-16). 

These two conditions cannot usually be differentiated by barium swallow or manometry.  Endoscopy should always be performed in achalasia to examine the gastro-oesophageal junction and to obtain histology but sometimes the diagnosis of pseudo-achalasia can still be missed, so repeated examinations may be necessary.  Computed tomography (CT) scans can be helpful in some cases(16).  Oesophageal masses, asymmetric or asymmetric wall thickening may be demonstrable on CT scan in some patients with pseudo-achalasia but in other patients, CT may be normal or show non-diagnostic features.  Conversely, symmetric wall thickening can be demonstrated in the gastro-oesophageal junction in some patients with achalasia(16), such as the one described above (Fig 3).

A firm clinical diagnosis of achalasia was thus made. The clinicians then considered the therapeutic options, keeping in mind the co-morbid illnesses in this particular patient.  Her symptoms had continued unabated, and although she was getting nutrition via nasogastric feeding, she had not gained weight during the period of investigation.

MCQ7: Which of the following statements is correct?

  1. you should continue with supportive care only
  2. a calcium channel clocker to relax the lower oesophageal sphincter is very likely to be beneficial
  3. pneumatic balloon dilatation of the lower oesophagus and cardia is likely to help
  4. laparoscopic disruption of the lower oesophageal sphincter (Heller’s
    procedure) is now indicated
  5. injection of Botulinum toxin to the sphincter zone would not help   

Correct answer 3

  1. You cannot just continue supportive care only – she cannot continue nasogastric feeding indefinitely.
  2. Smooth muscle relaxants including glyceryl trinitrate, long acting nitrates or calcium channel blockers have been used to relieve pain or dysphagia but benefit only a minority of patients.
  3. Pneumatic dilatation is the most effective non-surgical treatment for achalasia.  A balloon is placed across the lower oesophageal sphincter and is inflated to a pressure sufficient to rupture the muscle fibres.  A combined endoscopic-fluoroscopic approach is required with the Rigiflex balloon whereas fluoroscopy is not necessary with the ‘over-the-scope’ Witzel balloon.  The Rigiflex balloon comes in sizes of 3 cm, 3.5 cm and 4 cm.  A sequential, graded approach can therefore be used, starting with the smallest balloon and going on to the next size after 4-8 weeks if the patient does not respond.  Head-to head comparisons of the two balloons have not been reported but some authors feel that the perforation rate with this graded approach (0-8.3% for 12 studies, mean 2.5%) is less than that using the Witzel balloon with a standard 4 cm diameter (0-13% for 6 studies, mean 6%)(6).  The proportions of patients experiencing a good response averaged 66% with the Witzel balloon and 82% with the Rigiflex balloon.  However, the remission rate after pneumatic dilatation decreases with increasing length of follow-up, to 40% after 5 years and only 36-40% after 10 –15 years(7;8).  Pneumatic dilatation is usually performed under sedation on an outpatient basis (6).  Older individuals often respond better than younger ones(7) but a repeat procedure is sometimes necessary. 
  4. Heller’s operation involves a myotomy across the lower oesophageal sphincter, and relieves dysphagia in 90% of patients(9).  The main complication is uncontrolled gastro-oesophageal reflux in about 10% of patients, so an anti-reflux procedure is often performed at the time of the myotomy. Traditionally surgical myotomy is advocated when there is a poor response to pneumatic dilatation, or when symptoms recur but this approach may change with increasing use of minimally invasive surgery. Surgical myotomy is now usually carried out laparoscopically, and the reduced morbidity and shorter stay has made surgery a more attractive option for otherwise healthy patients with achalasia, although long-term results are unknown.
  5. Botulinum toxin inhibits the release of acetylcholine from nerve terminals and counters the effect of the selective loss of inhibitory neurotransmitters.  Injection of botulinum toxin into the lower oesophageal sphincter improves dysphagia in about 85% of patients(10) with older patients and those with vigorous achalasia especially likely to respond.  Symptoms invariably recur.  Although repeat injections can be given the efficacy wanes over time because of the development of antibodies against the botulinum toxin(10).  Botulinum toxin injections cause severe inflammation and scarring of the gastro-oesophageal junction, which is said to increase the technical difficulties and risks of myotomy(9).

Treatment of achalasia is palliative because oesophageal aperistalsis and impaired lower oesophageal sphincter relaxation is rarely, if ever, completely reversed by any mode of therapy.  The aim of treatment is to improve swallowing by reducing the gradient across the lower oesophageal sphincter, thus facilitating oesophageal emptying by gravity and hopefully preventing further oesophageal dilatation. 

Traditionally, symptomatic improvement is used to assess the results of pneumatic dilatation or surgical myotomy.  Objective assessment of lower oesophageal pressure or a timed barium swallow can help to define objective improvement and assess the need for further treatment.

Many authorities advocate pneumatic dilatation in the first instance, progressing to surgical myotomy if the response is unsatisfactory(11).  Botulinum toxin injection is normally reserved for older unfit individuals and those with concomitant morbidity who are unfit for surgery and in whom oesophageal perforation would be catastrophic. A recent head-to-head study compared the use of pneumatic dilatation and laparoscopic myotomy and reported similar long-term efficacy for both procedures(12).  Another recently reported randomised controlled trial compared laparoscopic myotomy with botulinum toxin injection.  Symptomatic response to botulinum toxin injection was inferior to that of surgery, especially with increasing length of follow-up(13).

Patients with advanced mega-oesophagus can present a difficult therapeutic challenge and in some patients, oesophagectomy may be required.

Clinical case

Subsequently pneumatic dilatation of the cardia was performed using a Rigiflex balloon.  The swallowing returned to normal.  The patient put on weight, and continued to swallow well three years later.

MCQ 8: Patients with achalasia are at risk of aspiration and impaired nutrition and also at risk of side effects resulting from therapeutic intervention.  Which of the following additional longer-term problems has been identified in patients with achalasia?

  1. squamous carcinoma of the middle or lower oesophagus
  2. adenocarcinoma of the lower oesophagus
  3. benign stricture of the lower oesophagus

Correct answer 1

  1. Patients with achalasia are at increased risk of oesophageal carcinoma – especially squamous cell carcinoma (17). Typically tumours occur in patients with a history of achalasia for more than 15 years, in the context of a mega-oesophagus.  Because a large tumour is required to produce symptoms, the tumours tend to be advanced at the time of presentation.  Nonetheless the role of surveillance endoscopy remains unclear.
  2. Patients with achalasia are not at increased risk of adenocarcinoma.
  3. Achalasia in itself is not a risk factor for benign peptic strictures but pneumatic dilatation and cardiomyotomy do increase the risk of gastro-oesophageal reflux disease.

Key points

In patients with achalasia there is aperistalsis with insufficient relaxation of the lower oesophageal sphincter.  The resting lower oesophageal pressure is typically elevated.

The most common symptom is dysphagia, usually for both solids and liquids.  This is in contrast to patients with peptic stricture or oesophageal carcinoma, in whom dysphagia is generally for solids only.  Other typical symptoms of achalasia include chest pain and regurgitation.

Barium swallow is often more helpful than endoscopy in the diagnosis of achalasia, especially in the early stages.  There is loss of peristalsis and poor emptying with retained saliva and food.  Oesophageal dilatation occurs with more chronic disease.

There is no cure for achalasia and the aim of treatment is to reduce symptoms by improving oesophageal emptying.  The two most effective treatments are pneumatic dilatation and surgical myotomy.  Traditionally surgery is reserved for patients in whom pneumatic dilatation is unsuccessful or whose symptoms recur following satisfactory initial response to dilatation. 

However, this approach may change with the advent of laparoscopic myotomy.

Endoscopic injection of botulinum toxin is an alternative strategy but its effect tends to be transient.  It is normally reserved for older and unfit individuals who are at high surgical risk and in whom perforation following pneumatic dilatation would be especially dangerous.

Pseudo-achalasia should be suspected in older individuals especially those with profound weight loss.  Endoscopy with biopsy, CT scan and endoscopic ultrasound may be helpful but the diagnosis of pseudo-achalasia can be extremely difficult even with the use of all these modalities.

There is an increased risk of squamous cell carcinoma, most commonly of the mid-oesophagus.  Presentation is usually late, but the place of surveillance has not been established.

Acknowledgement:

The author would like to thank Professor David Evans for providing the manometry tracing for this case study.

Reference List

  1. Richter JE. Oesophageal motility disorders. Lancet 2001;358(9284):823-8.
  2. Paterson WG. Etiology and pathogenesis of achalasia. Gastrointest Endosc Clin N Am 2001;11(2):249-66, vi.
  3. Mayberry JF. Epidemiology and demographics of achalasia. Gastrointest Endosc Clin N Am 2001;11(2):235-48, v.
  4. Blam ME, Delfyett W, Levine MS et al. Achalasia: a disease of varied and subtle symptoms that do not correlate with radiographic findings. Am J Gastroenterol 2002;97(8):1916-23.
  5. Eckardt VF. Clinical presentations and complications of achalasia. Gastrointest Endosc Clin N Am 2001;11(2):281-92, vi.
  6. Kadakia SC, Wong RK. Pneumatic balloon dilation for esophageal achalasia. Gastrointest Endosc Clin N Am 2001;11(2):325-46, vii.
  7. Eckardt VF, Gockel I, Bernhard G. Pneumatic dilation for achalasia: late results of a prospective follow up investigation. Gut 2004;53(5):629-33.
  8. West RL, Hirsch DP, Bartelsman JF et al. Long term results of pneumatic dilation in achalasia followed for more than 5 years. Am J Gastroenterol 2002;97(6):1346-51.
  9. Ali A, Pellegrini CA. Laparoscopic myotomy: technique and efficacy in treating achalasia. Gastrointest Endosc Clin N Am 2001;11(2):347-58, vii.
  10. Hoogerwerf WA, Pasricha PJ. Pharmacologic therapy in treating achalasia. Gastrointest Endosc Clin N Am 2001;11(2):311-24, vii.
  11. Vaezi MF, Richter JE. Diagnosis and management of achalasia. American College of Gastroenterology Practice Parameter Committee. Am J Gastroenterol 1999;94(12):3406-12.
  12. Vela MF, Richter JE, Wachsberger D et al. Complexities of managing achalasia at a tertiary referral center: use of pneumatic dilatation, Heller myotomy, and botulinum toxin injection. Am J Gastroenterol 2004;99(6):1029-36.
  13. Zaninotto G, Annese V, Costantini M et al. Randomized controlled trial of botulinum toxin versus laparoscopic heller myotomy for esophageal achalasia. Ann Surg 2004;239(3):364-70.
  14. Tracey JP, Traube M. Difficulties in the diagnosis of pseudoachalasia. Am J Gastroenterol 1994;89(11):2014-8.
  15. Achkar E. Diseases associated with or mimicking achalasia. Gastrointest Endosc Clin N Am 2001;11(2):267-80, vi.
  16. Carter M, Deckmann RC, Smith RC et al. Differentiation of achalasia from pseudoachalasia by computed tomography. Am J Gastroenterol 1997;92(4):624-8.
  17. Dunaway PM, Wong RK. Risk and surveillance intervals for squamous cell carcinoma in achalasia. Gastrointest Endosc Clin N Am 2001;11(2):425-34, ix.

Postest:

1. Which of the following conditions can be reliably excluded by a normal endoscopy?

  1. Pharyngeal pouch
  2. Oesophageal web
  3. Achalasia
  4. Peptic stricture
  5. Corkscrew oesophagus

Answer: 4
A normal endoscopy excludes a peptic stricture. The other conditions can be missed at endoscopy.

2. Regarding the epidemiology of achalasia, which of the following statements is correct?

  1. Most cases occur after the sixth decade
  2. There is a male predominance with a ratio of 2:1
  3. Its incidence appears to have increased since the second world war
  4. It is especially prevalent in oriental countries

Answer: 1
Most patients with achalasia present after the sixth decade.

3. All of the following are recognised manometric features of achalasia except for:

  1. Elevated lower oesophageal sphincter pressure
  2. Impaired or absent relaxation of the lower oesophageal sphincter
  3. Simultaneous non-peristaltic contractions of the body of the oesophagus
  4. High amplitude peristaltic waves with wet swallows

Answer: 4
Aperistalsis is the rule in achalasia.

4. Which of the following statements regarding pneumatic dilatation in achalasia is correct?

  1. Pneumatic dilatation is more efficacious among younger individuals than in older patients
  2. Oesophageal peristalsis returns in 50% of patients following successful dilatation
  3. Pneumatic dilatation can be safely performed without fluoroscopy
  4. Patients should be admitted overnight
  5. The perforation rate should be under 2%

Answer: 3
Pneumatic dilatation can be safely performed without fluoroscopy.

5. Which of the following statements regarding botulinum toxin injection treatment for achalasia is correct?

  1. Because it is non-invasive, botulinum toxin injection treatment is the first line treatment in most cases of achalasia
  2. The results of botulinum toxin injection is comparable to that of pneumatic dilatation at 6 months
  3. The results of botulinum toxin injection is superior to that of surgical myotomy at 3 years.
  4. Repeat injection is contraindicated because of the risk of anaphylaxis

Answer: 2
The results of botulinum toxin injection is comparable to that of pneumatic dilatation at 6
months.

6. Regarding surgical myotomy for achalasia:

  1. Dysphagia is relieved in more than 95% of patients
  2. Peristalsis returns after successful surgical myotomy in 25% of patients
  3. The chances of a successful outcome is reduced when there is gross dilatation of the oesophagus
  4. Without concomitant fundoplication, severe gastro-oesophageal reflux occurs in 50% of patients
  5. With or without fundoplication, severe reflux occurs in more than 20% of patients

Answer: 3
The chance of a successful outcome is reduced when there is gross dilatation of the oesophagus.

7. Which of the following is NOT a recognised symptom of achalasia:

  1.  Heartburn
  2.  Chest pain
  3.  Dysphagia
  4.  Regurgitation
  5.  Chronic laryngitis

Answer: 5
Recognised symptoms of achalasia include:

  1. Heartburn
  2. Chest pain
  3. Dysphagia
  4. Regurgitation

8. Regarding the risk of malignancy in achalasia, which of the following statements is correct?

  1. Squamous cell carcinoma is the usual histological subtype
  2. Tumours are most common in the lower third of the oesophagus
  3. 30% of carcinomas are prevalent cancers, already present at the time of diagnosis of achalasia
  4. Early diagnosis can be achieved with regular surveillance endoscopy
  5. Studies indicate that the risk is probably reduced by proton pump inhibitors

Answer: 1
Squamous cell carcinoma is the usual histological subtype.

9. Which of the following statements relating to the pathology of achalasia is INCORRECT?

  1. The primary event is a non-inflammatory degeneration of cholinergic neurons in the myenteric (Auerbach’s) plexus
  2. There is a prominent but patchy inflammatory response in the myenteric (Auerbach’s) plexus
  3. There is a selective loss of inhibitory neurons containing nitric oxide and vasoactive intestinal polypeptide
  4. Aperistalsis is caused by damage to the stimulatory cholinergic neurons

Answer: 1
In patients with achalasia there is loss of ganglion cells in the myenteric (Auerbach’s) plexus, associated with an inflammatory response.

Competing interests

None