Dysphagia

Question 1

Mrs Sweeney is a 76-year-old retired greengrocer who presents to her general practitioner (GP) because she is having difficulty swallowing.
Different patients mean different things when they say ‘swallowing difficulty’. What could Mrs Sweeney mean?

Answer 1

When a patient says ‘swallowing difficulty’, they could mean:
• Dysphagia: difficulty swallowing. If they really mean dysphagia, try to understand when exactly it feels as though the food ‘gets stuck’. Those with high dysphagia (oro-pharyngeal and upper oesophageal) describe difficul- ty initiating a swallow or immediately upon swallowing. Those with low dysphagia (lower oesophageal) feel the food getting stuck a few seconds after swallowing.
• Odynophagia: painful swallowing. Odynophagia may be due to malignancy, but is more commonly a feature of infection such as candidiasis.
• Globus: the common sensation of having a lump in the throat without true dysphagia. Globus is very common and its aetiology is poorly understood – however, only a small proportion of affected patients will seek medical help and it is an entirely benign condition.

Question 2

Summarise the main anatomical types of dysphagia

Answer 2

Dysphagia is an impairment of swallowing and thus can involve any structure between the mouth and the lower oesophageal sphincter. Try to organize your differential anatomically into ‘high dysphagia’ (oropharyngeal and upper oesophageal) and ‘low dysphagia’ (lower oesophageal), and think about whether the underlying mechanism is structural or functional (Table 6.1).

Question 3

Which diagnoses are the most common?

Answer 3

Broadly speaking, high dysphagia is more likely to be due to generalized/systemic neuromuscular disease, whereas low dysphagia is more likely to be due to a local obstructing lesion.

Question 4

Give examples of functional high dysplasia

Answer 4

Stroke Parkinson’s
disease Myasthenia gravis Multiple sclerosis Myotonic dystrophy
Motor neuron disease

Question 5

Give examples of a structural high dysphagia

Answer 5

Cancer
Pharyngeal pouch
Cricopharyngeal bar

All mural

Question 6

Give examples of a functional low dysphagia

Answer 6

Achalasia Chagas disease
Nutcracker oesophagus
Diffuse oesophageal spasm
Limited cutaneous scleroderma (CREST)

Question 7

Give examples of a structural low dysphagia

Answer 7

Luminal:
Foreign Body

Mural:
Cancer
Stricture (caustic
or inflammatory)
Plummer–Vinson syndrome
Schatzki ring
Congenital atresia†
Post- fundoplication

Extrinsic:
Mediastinal mass
Retrosternal goitre
Bronchial carcinoma
Thoracic aortic aneurysm
Pericardial effusion
Ortner’s syndrome
Dysphagia lusoria†

Question 8

In a patient of Mrs Sweeney’s age, is there any particular diagnosis that you must rule out?

Answer 8

New-onset dysphagia in middle-aged to elderly patients is carcinoma until proven otherwise.

Question 9

What questions would you like to ask specifically about the swallowing?

Answer 9

What is the duration of the symptoms?
Is it progressive or intermittent?
Is the dysphagia to solids, fluids, or both?

Question 10

Why is the duration of symptoms important

Answer 10

What is the duration of the symptoms? This is a key question – cancer typi- cally presents with a short history of days to weeks, whereas chronic motility disorders such as achalasia present with symptoms lasting months to years.

Question 11

Describe the importance of asking whether the dysphagia is progressive or intermittent

Answer 11

• Is the dysphagia progressive or intermittent? Progressive dysphagia is high- ly suggestive of a stricture (benign or malignant), whereas intermittent symp- toms are more characteristic of motility disorders.

Question 12

Describe the importance of asking which types of food the dysphagia occurs with

Answer 12

Is the dysphagia to solids, fluids, or both? If the patient is able to swallow fluid as per normal but has difficulty with solid food items (which feel as if they are sticking†) this points towards a mechanical obstruction, i.e. a stric- ture (benign or malignant). Of course, as the stricture becomes more severe then the dysphagia may start to involve fluids as well. Equally it is possible for oesophageal cancer to present as a sudden ‘absolute’ dysphagia if a morsel of food lodges above a critically narrowed lumen – in which case the patient cannot even swallow saliva. If the dysphagia is initially more pronounced for fluids over solids then this suggests a motility disorder (e.g. achalasia or a neu- romuscular condition).

Question 13

Describe the importance of asking about coughing in the history

Answer 13

• Is there any coughing? Coughing that occurs immediately after swallowing suggests that there is a problem with the coordination of swallowing events and thus points towards disorders such as stroke and Parkinson’s disease. By con- trast, if coughing occurs some time after a meal, this implies regurgitation of food retained within a pharyngeal pouch, or gastro-oesophageal reflux disease

Question 14

Describe the importance of asking about choking in the history

Answer 14

• Is there any choking? This too suggests a functional problem with the oro- pharyngeal phase of swallowing.

Question 15

Describe the importance of asking about gurgling or dysphonia

Answer 15

• Is there any gurgling or dysphonia? Patients with a pharyngeal pouch can often be heard to make gurgling noises if they attempt to speak soon after eat- ing or drinking. It may also be possible to see a visible bulging of the neck.

Question 16

Describe the importance of asking about heartburn or water brash

Answer 16

• Is there heartburn or waterbrash? The presence of these two symptoms is highly suggestive that the dysphagia is related to reflux disease, with or without a stricture.

Question 17

Describe the importance of asking about weight loss

Answer 17

Weight loss: this is the cardinal ‘red flag’ for oesophageal cancer, although of course any cause of dysphagia will ultimately result in weight loss if the dys- phagia is sufficiently severe.

Question 18

Describe the importance of asking about nocturnal cough/wheeze

Answer 18

Nocturnal cough/wheeze: while these symptoms are more commonly due to asthma, gastro-oesophageal reflux, or post-nasal drip, they can also be a fea- ture of achalasia because stasis of food and saliva in the oesophagus can result in aspiration.

Question 19

What neurological symptoms are relevant

Answer 19

• Neurological symptoms should be enquired about in any patient who has features suggestive of a functional dysphagia, e.g. difficulty coordinating swal- lowing, slow eating, extra effort required to eat/chew, tiredness after eating, and early dysphagia for liquids.

Question 20

What Rheumatological symptoms are relevant

Answer 20

Rheumatological symptoms may be relevant in the context of limited cutane- ous scleroderma (previously known as CREST syndrome), in which patients may suffer from a combination of Calcinosis, Raynaud’s, (o)Esophageal dys- motility (the E in CREST refers to the American spelling of oesophagus), Scle- rodactyly, and Telangiectasia.

Question 21

Why is past medical/surgical history relevant?

Answer 21

The two key medical conditions to enquire about are gastro-oesophageal reflux disease (GORD) and peptic ulcers. However, the patient may not have a proven diagnosis of either of these conditions and may simply complain of a combination

dyspepsia and/or waterbrash. GORD and peptic ulcers are directly implicated in
the aetiology of two of the most common causes of dysphagia:
• GORD predisposes to oesophageal adenocarcinoma and non-malignant strictures of the oesophagus. A history of GORD due to a sliding hiatus hernia is also significant if the patient has had a fundoplication operation to tighten the lower oesophageal sphincter. Post-operative dysphagia is a potential com- plication of such a procedure if the wraps are made too tight.
• Peptic ulcers can also lead to scarring and strictures around the gastric cardia and lower oesophagus.
There may also be a history of a progressive neurological disease such as multiple sclerosis or Parkinson’s disease.

Question 22

Why is a detailed drug history of particular importance when investigating dysphagia?

Answer 22

Drugs can contribute towards dysphagia in two main ways:

1) Firstly, drugs such as calcium-channel blockers and nitrates, which relax smooth muscle, can cause or exacerbate reflux symptoms by decreasing oesophageal tone.
2) Secondly, drugs such as non-steroidal anti-inflammatory drugs (NSAIDs), steroids, and bisphosphonates predispose to peptic ulceration.

Question 23

On further questioning, Mrs Sweeney describes how she has had progressive difficulty swallowing solid foods – particularly meat – over the past 3 weeks. She says that the chunks of food feel as if they are getting stuck midway down her throat, but she has no difficulty with the actual chewing movements.
She has not suffered from any coughing or choking after eating. She has not noticed any changes in her voice or any gurgling after eating or drinking.
When asked directly about any weight loss, she says that she has probably lost a few kilos as her clothes feel somewhat loose, but says that she is not surprised at this because she feels that she is able to eat so little.
Her past medical history is significant for GORD, for which she takes regular omeprazole, and polymy- algia rheumatica, for which she takes daily prednisolone. Her GP has also prescribed calcium, vitamin D, and a bisphosphonate to be taken as bone-protecting therapy while she is taking regular steroids.
The GP examines Mrs Sweeney. What are the relevant aspects of the physical examination?

Answer 23

There is relatively little that can be gained from the examination of a patient with dysphagia, but there are five features that are particularly relevant:
1) Cranial nerve pathology: this is particularly important if there are features in the history that suggest a functional dysphagia as the patient may have bulbar palsy for example.
2) Signs of gastrointestinal (GI) malignancy: patients with oesophageal carci- noma may be markedly cachectic, and may have a palpable Virchow’s node. If there is a carcinoma extending into the cardia of the stomach this may be palpable in thin patients.
3) Neck mass: It may be possible to palpate a large pharyngeal pouch in thin patients, and this may even gurgle on palpation. A goitre may also be palpable, causing the dysphagia by extrinsic compression
† 4) Features of CREST syndrome (described above).
5) Koilonychia: suggests severe iron-deficiency anaemia which can cause Plummer–Vinson syndrome (oesophageal webs).

Question 24

Mrs Sweeney has a cachectic appearance. Abdominal examination reveals a palpable lymph node in the left supraclavicular fossa but is otherwise unremarkable.
What is the most likely diagnosis to account for Mrs Sweeney’s dysphagia?

Answer 24

The short history of a progressive dysphagia that is more pronounced for sol- ids than fluids, occurring on a background of GORD in an elderly patient, and coupled with examination findings of cachexia and lymphadenopathy, is high- ly suggestive of oesophageal carcinoma. This is statistically more likely to be adenocarcinoma given that this subtype accounts for about 65% of oesophageal carcinoma in the UK.

Question 25

The GP refers Mrs Sweeney urgently to an upper GI surgeon.

What are the different types of investigation used for dysphagia, and what are the indications for each?

Answer 25

Barium Swallow
Endoscopy
Videofluroscopy
Manometry

Question 26

Describe the indication for the barium swallow

Answer 26

Barium swallow:
− This is a cineradiographic study which monitors the passage of a bolus of
barium contrast medium from the upper to the lower oesophageal sphinc- ter of a supine patient. Various lesions have characteristic appearances on barium swallow (see Figs 6.1–6.4). Patients may also be asked to swallow an effervescent agent to produce a double-contrast study that is better at visualizing mucosal lesions. Note that a barium swallow is not the same thing as a barium meal – the latter follows the passage of barium through the stomach and into the duodenum.
− Barium swallow is useful in investigating patients who may have a high lesion. During endoscopy this region is intubated blindly, and in the presence of a pharyngeal pouch or high oesophageal cancer there is a risk of injury or perforation. It is also indicated in patients with features suggesting achalasia (e.g. intermittent symptoms, difficulties with fluids and solids).

Question 27

Describe the indication for endoscopy

Answer 27

Allows visualization of luminal and mural lesions, as well as the oppor-
tunity to biopsy and treat lesions. Various procedures, such as stricture dilatation, stent insertion, laser coagulation, and Botox injections can all be carried out endoscopically.
Endoscopy is a more sensitive and specific test than double-contrast bari- um swallow and is often the first-line investigation for low dysphagia.

Question 28

Describe two potential diagnoses made with the barium swallow

Answer 28

Figure 6.1 Barium swallow showing a mid-oesophageal stricture consistent with oesophageal cancer.

Figure 6.2 Barium swallow showing extrinsic compression of the lower oesophagus by an extraluminal mass.

Question 29

Describe the indication for video fluoroscopy

Answer 29

Essentially a modified form of barium swallow in which upright patients
are given barium in liquid, solid, or semi-solid form. A speech therapist
modifies the swallowing technique throughout the study.
− Mostsuitedtopatientswithafunctionalhighdysphagia.

Question 30

Describe the indication for manometry

Answer 30

− Assesses the pressures in the lower oesophageal sphincter and the peri-
staltic wave in the rest of the oesophagus.
− Manometry is the key investigation for diagnosing a motility disorder
and distinguishing between the different types of motility disorders (e.g. achalasia and nutcracker oesophagus). It is indicated when barium swal- low and/or endoscopy are unremarkable, suggesting a cause other than mechanical obstruction.

Question 31

Mrs Sweeney was seen by an upper GI surgeon within a fortnight. An outpatient oesophagogastroscopy was performed and an irregular ulcerative lesion on a background of ‘velvety’ epithelium was seen in the distal 5 cm of oesophagus. Multiple biopsy samples were taken. Histology revealed high-grade dysplas- tic columnar epithelium, with penetration of the basement membrane.
What is the diagnosis and how should Mrs Sweeney be managed?

Answer 31

Mrs Sweeney has oesophageal adenocarcinoma on a background of Barrett’s oesophagus (hence the velvety epithelium in the distal oesophagus). She should be referred to a specialist multidisciplinary cancer team comprising surgeons, patholo- gists, anaesthetists, radiologists, oncologists, and specialist nursing support.
The first thing to do with a cancer patient is to grade and stage the malignancy. Remember that you grade the tumour histopathologically (the more the cells devi- ate from normality, the higher the grade) and stage the patient by determining how far the cancer has spread anatomically.
The team will assess the stage of Mrs Sweeney’s cancer to establish whether the tumour is operable and whether she is fit for surgery. Oesophagectomy is associated
with the highest mortality of any elective surgical procedure, and only about a third of patients are deemed suitable for surgery due to advanced disease or comorbidity. Various imaging modalities may be required for staging:

Question 32

Describe how the tumour can then be staged

Answer 32

1) Endoscopic ultrasound (EUS): useful for assessing intramural versus trans- mural disease, and for detecting local lymph node involvement.
2) Spiral computed tomography (CT) chest/abdomen: is the principle modality for staging the tumour. If the scan reveals inoperable or metastatic disease then there may be no advantage in further assessment of the primary. Some centres also use positron emission tomography (PET) scanning for tumour staging.
3) Laparoscopy: may be used to exclude peritoneal deposits if scanning indicates a significant burden of infradiaphragmatic disease but radical treatment is still planned.

Question 33

Describe the potential treatment options for the tumour

Answer 33

If the tumour is judged to be suitable for radical treatment, it is necessary to also perform a fitness assessment for surgery with a combination of lung function tests, arterial blood gas (ABG), electrocardiogram (ECG), exercise tolerance test ± echocardiogram.
Surgery is the definite treatment modality for early stage oesophageal adenocar- cinoma. Adjuvant chemotherapy (after the surgery) is poorly tolerated, but there is a role for neo-adjuvant chemotherapy (before the surgery) to shrink the tumour pre-operatively. The majority of patients diagnosed with oesophageal cancer are unsuitable for surgery (either because of the advanced tumour stage or patient comorbidities) and multidisciplinary palliative care is therefore required.

Question 34

What is the prognosis

Answer 34

The overall prognosis for oesophageal cancer is usually poor. As with other can- cers, prognosis is stage-dependent. For the minority of cancers that are caught at stage 1 (no invasion of muscularis propria and no nodal involvement) the 5-year survival is about 80%. Unfortunately 70% of patients present with lymph node involvement, and in these patients 5-year survival is about 15% even with modern treatments.

Question 35

Miss Aggrawal is a 27-year-old lawyer who presents with a 2-year history of mild dysphagia to both sol- ids and liquids. She says that she has no problems with coordinating swallowing in her mouth, but feels that food and drink are sticking on their way down to her stomach. She has not noticed any choking or gurgling related to eating and is not aware of any weight loss.
She has visited her GP twice in the last year, complaining of heartburn and a nocturnal cough. These were attributed to GORD and asthma, respectively, despite no assessment of her peak flow, and she was offered acid-suppressing medication and a bronchodilator inhaler. Neither of these therapies has eased her symptoms, however. She takes no other medications and is otherwise fit and well.
Given Miss Aggrawal’s dysphagia to liquids, her GP performs a thorough examination of her peripheral nervous system and cranial nerves, but nothing abnormal is noted. Abdominal and neck examinations are also unremarkable.
The GP refers Miss Aggrawal for a barium swallow. This shows a dilated oesophagus with a beak-like terminal narrowing. Manometry is also performed and shows an elevated lower oesophageal sphincter pressure with incomplete relaxation of the sphincter and aperistalsis in the body of the oesophagus.
What is the most likely diagnosis?

Answer 35

The history, examination, and investigation findings suggest achalasia. Compare this presentation of a young patient, with no loss of weight, and a long history of symptoms, with the classical cancer presentation of an older patient with weight loss and rapidly progressive dysphagia. The bird’s beak appearance on barium swallow is classical for achalasia, but it is necessary to perform manometry to distinguish acha lasia from other motility disorders such as nutcracker (hypertensive) oesophagus. It is likely that both Miss Aggrawal’s ‘GORD’ and ‘asthma’ were symptoms of her achalasia, hence the lack of response to medications.

Question 36

Describe the treatment options for achalasia

Answer 36

There are a number of treatment options available to Miss Aggrawal, although
it is important to appreciate that no treatment can restore peristaltic function to her oesophagus and thus her swallowing will never completely return to normal. Treat- ments are aimed at loosening the lower oesophageal sphincter:
• Pneumatic balloon dilatation achieves good results in the majority of patients, and about 60% are free of dysphagia at 5 years. Most patients will require repeat treatments. The main risk is of oesophageal perforation (5%).
• Surgical (Heller’s) myotomy (longitudinal incision of the muscle fibres of the dis- tal oesophagus) tends to produce better short- and long-term relief of dysphagia, but risks the development of GORD in about 10% of patients. For this reason, there is debate as to whether to perform simultaneous Nissen fundoplication (wrapping the stomach around the lower oesophagus to prevent reflux).
• Botox injections produce good results that last up to 1 year, and may be favoured in patients unsuited to interventional therapy.

Drugs such as calcium-channel blockers and/or nitrates may relax the lower oesophageal sphincter sufficiently to produce relief of symptoms, but are not first-line therapies and are primarily reserved for elderly patients with con- traindications to surgery or dilatation.

Question 37

What conditions present similarly to achalasia

Answer 37

Note that there are two other small-print diagnoses which may produce identical investigation find- ings to achalasia: Chagas disease (endemic to Central and South America and due to Trypanosoma cruzi infection) and ‘pseudo-achalasia’ due to an oesophageal carcinoma which infiltrates the myenteric plexus. Endoscopy is required to identify the latter, and should be suspected if there is a <6-month history in an older patient with weight loss.

Question 38

Mr Giles is a 60-year-old farmer who is brought to hospital by ambulance. He was at a local cattle mar- ket when he developed chest pain and his fellow farmers called an ambulance. He was given glycer- yl trinitrate (GTN) en route to hospital, which relieved his pain, and an early ECG revealed no signs of ischaemia.
On arrival at A&E he is pain free and repeat ECGs are unremarkable. He describes how he has had inter- mittent central crushing chest pain for the last year. He has been hospitalized twice with the pain, and had an angiogram during his last admission which revealed patent coronary arteries. On further ques- tioning he describes getting episodes of chest pain at least once a week, but that they are responsive to GTN. The pain does not radiate, and it is not induced by exercise.
A functional enquiry reveals that Mr Giles has been troubled by heartburn for ‘some years’ and takes antacid tablets whenever he feels the need. When specifically asked whether his chest pain ever coin- cides with a difficulty swallowing food he says ‘yes’.
Mr Giles’s past medical history is significant for hypertension, for which he takes bendroflumethiazide and ramipril. He is a cigarette smoker with a 12-pack-year history (20 cigarettes a day for 12 years), and enjoys about 10 pints of ale a week.
The A&E registrar examines Mr Giles but does not find anything remarkable. He requests a blood troponin, which is negative, and discharges Mr Giles with a diagnosis of ‘non-cardiac chest pain’.
Can you suggest a more refined diagnosis? How would you like to investigate Mr Giles? How would you treat him if your suggested diagnosis is correct?

Answer 38

It is likely that Mr Giles is suffering from oesophageal spasm. This is suggested by the fact that he has chest pain that bears no relation to exertion and does not radi- ate (hence being atypical of cardiac chest pain) and is relieved by GTN spray (the smooth muscle of the oesophagus responds to nitrates). The fact that his coronary angiogram revealed no lesions adds further weight to this diagnosis. The association of the chest pain with intermittent spells of dysphagia clinches the diagnosis – but do not expect all patients with oesophageal spasm to present with dysphagia. Most patients will have a symptom complex involving some or all of chest pain, heart- burn, regurgitation, globus, and dysphagia.
The diagnosis could be confirmed by performing manometry. There are two main types of spasm that may be seen, although the treatment for both is the same. Diffuse oesophageal spasm produces high-amplitude, simultaneous, prolonged contractions of the oesophageal body, and is sometimes referred to as ‘corkscrew oesophagus’. By contrast, nutcracker (or hypertensive) oesophagus produces an abnormally strong peristaltic pressure wave in the oesophageal body, and may be associated with a hypertensive lower oesophageal sphincter.
If Mr Giles does have oesophageal spasm it is likely that his symptoms would be relieved with a calcium-channel blocker such as sublingual nifedipine. Alterna- tively he could continue to use GTN for symptomatic relief. If he is able to identify any foods (such as very hot or cold food) which precipitate the spasm he should be advised to consider avoiding them.

Question 39

Mr Gan is a 64-year-old retired restaurateur who presents to his GP with a 5-week history of progressive dysphagia to solids. He describes how it feels as if food items are getting stuck halfway down his throat. He denies any choking, gurgling, heartburn, or waterbrash. He has, however, suffered from increasing dyspnoea over the past 2 months, and has been coughing for a couple of weeks. The coughing occurs both day and night, and has recently been associated with episodes of haemoptysis. Mr Gan thinks that he has lost about 4 kilos in the past couple of months and describes feeling increasingly lethargic.
He has no significant past medical history and takes no regular medications. He does not drink, but has a 40-pack-year history of cigarette smoking.
On examination, Mr Gan is cachectic. There is hepatomegaly and palpable lymph nodes in his left supraclavicular fossa. Percussion and auscultation of Mr Gan’s chest is unremarkable, as is a neurologi- cal exam.
In light of Mr Gan’s haemoptysis and prolonged dyspnoea, his GP refers him for an urgent chest radio- graph. This shows multiple coin lesions and hilar lymphadenopathy. A subsequent CT of his chest and abdomen indicates prominent subcarinal lymph nodes and multiple liver lesions consistent with meta- static lung cancer.
What is the mechanism of Mr Gan’s dysphagia?

Answer 39

Mr Gan’s dysphagia is caused by extrinsic compression of the oesophagus by the prominent subcarinal deposits of secondary tumour. He would be offered palliative care to try and minimize the degree of dysphagia suffered but his prognosis, sadly, is poor.
6

Question 40

What is Barrett’s oesophagus? How common and worrying is this condition?

Answer 40

Barrett’s is metaplasia of the squamous epithelium of the lower oesophagus into columnar epithelium (also known as CLO or columnar lined oesophagus). It is usually associated with inflammation and ulceration of the distal oesophagus, and may be endoscopically visible as an area of ‘velvety’ epithelium. Barrett’s is caused by persistent irritation of the squamous epithelium by GORD; both stomach acid and bile are implicated in the aetiology.
The significance of Barrett’s is that it is a precursor lesion to adenocarcinoma, increasing the relative risk 30–40-fold. About 1% of the UK population has Barrett’s (although only about 5% of these patients are diag- nosed). The incidence of adenocarcinoma developing in these Barrett’s patients is approximately 1 per 100 person years. Regular endoscopic surveillance is recommended for patients with known Barrett’s; the role of lifelong acid-suppressing therapy (proton-pump inhibitors), antireflux surgery, and endoscopic mucosal ablation is controversial.

Question 41

What are the risk factors for oesophageal cancer?

Answer 41

There are two main types of oesophageal cancer – squamous cell carcinoma and adenocarcinoma (carcinoid, small cell carcinoma, and leiomyoma are very rare). In recent years there has been a relative decrease in the incidence of squamous cell carcinoma and a corresponding increase in the incidence of adenocarcinoma. Risk factors for these cancers are:
• Squamous cell carcinoma:
− Alcohol
− Smoking
− Dietary nitrosamines (found in pickled/mouldy foods) and nitrates (found in Chinese, Iranian, and
South African diets)
− Aflatoxins
− Achalasia
− Plummer–Vinson syndrome (see below)
− Hereditary tylosis†
− Coeliac disease
• Adenocarcinoma:
− Barrett’s oesophagus (hence any factor which predisposes to reflux oesophagitis)
− Smoking and alcohol intake are not as important as they are for squamous cell carcinoma

Question 42

What is hereditary tylosis

Answer 42

Hereditary tylosis is a very rare autosomal dominant condition, also known as focal non-epidermolytic palmoplantar keratoderma. It manifests as symmetrical keratosis of the palms and soles of the feet.

Question 43

What is Plummer-Vinson Syndrome

Answer 43

This is a rare collection of features including atrophic glossitis (smooth tongue), cheilosis (cracks at the cor- ner of the mouth), koilonychia, and dysphagia that is associated with iron-deficiency anaemia. The dysphagia is due to the development of a post-cricoid web of hyperkeratinization. Correction of the iron deficiency may lead to a dramatic improvement in the degree of dysphagia, but persistent dysphagia may require balloon dilatation. The syndrome is most common in middle-aged to elderly women and is pre-malignant for cricopha- ryngeal carcinoma.
The condition is also known as Patterson–Brown-Kelly syndrome (Plummer and Vinson described the condi- tion in America, while Patterson and Brown-Kelly described it independently in the UK).

Question 44

What is the pathophysiology of achalasia

Answer 44

Achalasia is caused by an absence of ganglion cells in the myenteric plexus (Auerbach’s plexus) of the oesophagus. The consequence is a failure of relaxation of the lower oesophageal sphincter, and aperistalsis in the oesophageal body. The underlying cause of these changes in achalasia is unknown. Chagas disease results in an identical pathophysiology, and infiltrating carcinoma can also produce a ‘pseudo-achalasia’ by invasion of the myenteric plexus. A congenital absence of myenteric plexus ganglion cells also underlies Hirschprung’s disease (megacolon) of the intestine.

Question 45

A dysphagic patient presents with a hoarse voice and bovine cough. Can you think of any pathologies that may account for all these symptoms?

Answer 45

A hoarse voice and bovine cough are characteristic of recurrent laryngeal nerve pathology. There are two main ways in which dysphagia may be connected with such a palsy:

1) The nerve may be infiltrated by a primary malignancy of the oesophagus or a mediastinal malignancy that is causing dysphagia by extrinsic compression.
2) Ortner’s syndrome describes the situation in which the recurrent laryngeal nerve is compressed by the cardiovascular system – most commonly left atrial dilatation secondary to mitral stenosis. Sufficiently severe left atrial enlargement can also cause dysphagia by extrinsic compression.

Question 46

A patient with oesophageal adenocarcinoma is judged to be an unsuitable candidate for surgery. What palliative treatments may be available for such a patient?

Answer 46

There are various treatment modalities which may be used to provide symptomatic relief from malignant dysphagia, including:
• Intubation with a rigid silastic endoprosthesis or expandable metal stent
• Ablation with laser or argon beam plasma coagulation
• Chemotherapy
• Radiotherapy
• Psychological and social support.

Question 47

How can oesophageal cancer present? What are the most common symptoms?

Answer 47

Oesophageal cancer, whilst still relatively rare, is now the eighth most common cancer in the UK with around 7000 new cases a year. The most common symptoms are:
• Dysphagia (~75%)
• Weight loss (~60%)
• GI reflux (~20%)
• Odynophagia (~20%)
• Dyspnoea (~10%)
Less common symptoms include GI bleeding, fatigue due to anaemia, hoarseness (due to involvement of
the recurrent laryngeal nerve), cough, facial flushing due to superior vena cava (SVC) obstruction, and breath- lessness due to malignant pleural effusion.

Question 48

What are the common complications after an oesophagectomy?

Answer 48

Removal of the oesophagus and refashioning of a gullet using the stomach is a major operation. The major complications for the junior house officer to be aware of are:
• Breakdown of the anastomosis due to poor blood supply (e.g. tight sutures, tension on the anastomo- sis), infection, and patient factors (e.g. smoking, diabetes mellitus, old age).
• Pneumonia due to poor ventilation secondary to pain, this can be minimized using specialist anal- gesia (e.g. high thoracic epidural blocks by specialized anaesthetists) and encouraging ventilation (physiotherapy).
• Cardiac arrhythmia. Manipulation of the tissue near to the heart predisposes patients to intraoperative and post-operative cardiac arrhythmias.

Question 49

What is the pharynx

Answer 49

The pharynx consists of the nasal, oral and laryngeal sections. The latter is the part of the throat that connects to the oesophagus.

Question 50

What is the oesophagus

Answer 50

The oesophagus is a muscular tube approximately 20 cm long that connects the pharynx to the stomach just below the diaphragm. Its only function is to transport food from the mouth to the stomach. In the upper portion of the oesophagus, both the outer longitudinal layer and inner circular muscle layers are striated. In the lower two-thirds of the oesophagus, including the thoracic and abdominal parts containing the lower oesophageal sphincter, both layers are composed of smooth muscle.

Question 51

Describe the lining of the oesophagus

Answer 51

The oesophagus is lined by stratified squamous epithelium, which extends distally to the squamocolumnar junction where the oesophagus joins the stomach, recognized endoscopically by a zigzag (‘Z’) line, just above the most proximal gastric folds.

Question 52

Describe the UOS and the LOS

Answer 52

The oesophagus is separated from the pharynx by the upper oesophageal sphincter (UOS), which is normally closed due to tonic activity of the nerves supplying the cricopharyngeus. The lower oesophageal sphincter (LOS) consists of a 2–4 cm zone in the distal end of the oesophagus that has a high resting tone and, assisted by the diaphragmatic sphincter, is largely responsible for the prevention of gastric reflux.

Question 53

Describe swallowing

Answer 53

During swallowing, the bolus of food is voluntarily moved from the mouth to the pharynx. This process is mediated by a complex reflex involving a swallowing centre in the dorsal motor nucleus of the vagus in the brainstem. Once activated, the swallowing centre neurones send pre-programmed discharges of inhibition followed by excitation to the motor nuclei of the cranial nerves. This results in initial relaxation, followed by distally progressive activation of neurones to the oesophageal smooth muscle and LOS. Pharyngeal and oesophageal peristalsis mediated by this swallowing reflex causes primary peristalsis. Secondary peristalsis arises as a result of stimulation by a food bolus in the lumen, mediated by a local intra-oesophageal reflex. Tertiary contractions indicate pathological non-propulsive contractions resulting from aberrant activation of local reflexes within the myenteric plexus.
The smooth muscle of the thoracic oesophagus and LOS is supplied by vagal autonomic motor nerves consisting of extrinsic preganglionic fibres and intramural postganglionic

neurones in the myenteric plexus (Fig. 13.6). There are parallel excitatory and inhibitory pathways.

Question 54

Summarise the innervation of the oesophagus

Answer 54

Innervation of the oesophagus. The excitatory pathway consists of vagal preganglionic neurones releasing acetylcholine (ACh), connecting to postganglionic neurones that release ACh and substance P (SP). The inhibitory pathway consists of vagal preganglionic neurones releasing ACh, connecting to postganglionic neurones that release nitric oxide (NO), vasoactive intestinal peptide (VIP), adenosine triphosphate (ATP) and SP.

Question 55

Classify the different causes of dysphagia

Answer 55

Oesophageal motility disorders
• Achalasia
• Scleroderma
• Diffuse oesophageal spasm • Presbyoesophagus
• Diabetes mellitus
• Chagas' disease
Extrinsic pressure
• Mediastinal glands • Goitre
• Enlarged left atrium
Intrinsic lesion
• Foreign body • Stricture:
– Benign – peptic, corrosive
– Malignant – carcinoma • Lower oesophageal ring
• Oesophageal web

Disease of mouth and tongue
• e.g. Candidiasis
Neuromuscular disorders
• Pharyngeal disorders
• Bulbar palsy (e.g. motor neurone disease, stroke) • Myasthenia gravis

Question 56

Summarise dysphagia

Answer 56

Major oesophageal symptoms are:
• Dysphagia. Difficulty in swallowing is defined as a sensation of obstruction during the

passage of liquid or solid through the pharynx or oesophagus: that is, within 15 s of food leaving the mouth. The characteristics of the progression of dysphagia to solids can be helpful. For example, intermittent slow progression with a history of heartburn suggests a benign peptic stricture; relentless progression over a few weeks suggests a malignant stricture. The slow onset of dysphagia for solids and liquids at the same time suggests a motility disorder, such as achalasia

Question 57

What are the main signs of oesophageal disease

Answer 57

The main sign of oesophageal disease is weight loss due to reduced food intake. Cervical lymphadenopathy with cancer is uncommon. Very rarely, a pharyngeal pouch may be seen to swell the neck during drinking.