DYSPHAGIA

Taking the dysphagia history

A good history will elucidate the site and the general pathophysiological process in 80% of cases, and is vital before embarking on a focussed and cost effective utilisation of specific diagnostic techniques. Frequently, patients will describe food sticking or holding up either retrosternally or in the neck. However, more atypical symptoms can include regurgitation, a sense of fullness retrosternally and hiccup. Dysphagia is distinguished from odynophagia (pain on swallowing) by the perception of actual bolus hold up. The aims of the history are to:

• determine whether dysphagia is actually present (i.e. distinct from globus sensation and xerostomia)

• establish whether the site of the problem is oesophageal or pharyngeal

• distinguish between a structural and a motor abnormality.

Where does the food stick?

Retrosternal hold-up suggests that the disorder lies within the oesophagus. If the site of hold-up is in the neck, the pathology can lie either in the oesophagus or in the pharynx (Figure 2.1). Due to referred sensation, the site of perceived hold-up is above the suprasternal notch in 30% of cases where the actual hold-up is within the oesophageal body. Therefore, the next batch of questions aims to distinguish pharyngeal from oesophageal dysfunction.

FIGURE 2.1 Differentiating oesophageal disorders.

Is one or more of the four cardinal symptoms of pharyngeal dysfunction present?

The four cardinal symptoms of pharyngeal dysfunction are:

1. Delayed or absent oropharyngeal swallow initiation

2. Deglutitive post-nasal regurgitation or egress of fluid through the nose during swallowing

3. Deglutitive cough indicative of aspiration

4. The need to swallow repetitively to achieve satisfactory clearance of swallowed material from the hypopharynx.

Supportive but less specific symptoms of oropharyngeal dysphagia include: bolus hold-up in the neck, piecemeal swallows, oral spill or drooling, dysphonia, throat clearing, garbled voice and weight loss.

If one or more of these symptoms is/are present the dysphagia is probably oropharyngeal, either structural or neuromyogenic, and further history and investigation should proceed accordingly (see below).

If oesophageal dysphagia is suspected, the next step is to establish whether it is a structural or motor disorder (Tables 2.1 and 2.2).

TABLE 2.1 Structural oesophageal disorders causing dysphagia

• Peptic stricture • Mucosal rings – Lower (e.g. Schatzki’s ring) – Multiple rings (e.g. eosinophilic oesophagitis) – Cervical webs

• Carcinoma

• Corrosive strictures

– Caustic, pill-induced

• Oesophageal diverticula

• Benign tumours

• Extrinsic (vascular compression, tumour)

TABLE 2.2 Aetiology of pharyngeal dysphagia^*

Structural	Neuromyogenic
• Tumour • Stenosis – Post-surgical – Radiation – Idiopathic • Zenker’s diverticulum • Cricopharyngeal bar • Web • Extrinsic compression

* Common causes. List not intended to be comprehensive.

Is the dysphagia for solids or liquids?

Typically the patient with a motor disorder (e.g. achalasia, diffuse spasm) will describe dysphagia for liquid and solids while structural disorders will cause dysphagia for solids only. As the calibre of the oesophagus narrows, the size of the bolus required to cause obstruction becomes progressively smaller.

If the problem is likely to be a structural oesophageal disorder, the following enquiry will define the likely cause.

How long has dysphagia been present; is it intermittent, is it progressive?

Longstanding dysphagia is compatible with a benign condition. Slowly progressive, longstanding dysphagia, particularly if reflux is or has been experienced, is highly suggestive of a peptic stricture. A short history of dysphagia particularly with rapid progression (weeks or months) and associated weight loss is highly suggestive of oesophageal cancer. Longstanding, intermittent, non-progressive dysphagia purely for solids is indicative of a fixed structural lesion such as a distal oesophageal ring or proximal oesophageal mucosal web.

What does the patient do when the bolus sticks?

Sipping water will frequently relieve obstruction due to a structural lesion causing solid bolus dysphagia. However, if the patient sips water in the context of a solid bolus impaction, this will result in immediate regurgitation of the water indicative of complete bolus obstruction. If the bolus is catching very high (e.g. at the cricopharyngeus) patients learn very quickly not to sip water as it results in immediate coughing and spluttering due to laryngeal penetration.

Does the patient regurgitate?

While regurgitation can be associated with solid bolus hold-up during a meal, spontaneous regurgitation between meals (generally fluid and ‘frothy’ saliva) is highly suggestive of dysmotility (see below).

If the problem is likely to be an oesophageal motility disorder, the following enquiry will define the likely cause.

The three cardinal features of oesophageal dysmotility are dysphagia (for solids and liquids), chest pain and regurgitation.

Regurgitation during the meal as well as spontaneous regurgitation between meals or at night (generally fluid and saliva) is highly suggestive of dysmotility. The regurgitated fluid/food is generally not noxious to taste (unlike reflux-related regurgitation).

Does the patient experience chest pain or discomfort?

Oesophageal spasm and achalasia typically cause chest pain. While frequently described as heavy or crushing, it can be indistinguishable from the typical ‘heartburn’ of reflux. The pain frequently occurs during the meal but can be quite unpredictable and sporadic or nocturnal. Sipping antacids or even water can relieve the related dysmotility, thus further confusing distinction from reflux pain.

If oesophageal dysmotility is strongly suspected, distinction between achalasia and oesophageal spasm can be difficult at times. Achalasia is very much more common than spasm. In achalasia, chest pain is more prominent early in the disease, but over the years tends to diminish and may disappear as dysphagia and regurgitation worsen. In contrast, the chest pain of spasm is the predominant symptom and can be quite severe. Due to poor oesophageal clearance, regurgitation is frequently more impressive in achalasia than it is in the case of spasm. The oesophagus generally dilates over time in the context of achalasia, but this is less prevalent with spasm. Finally, there can be significant overlap between the two syndromes and spasm can evolve into more typical achalasia over time as they both share a similar underlying inhibitory neuropathic process. Oesophageal motility disorders can be classified as either primary (e.g. achalasia or diffuse oesophageal spasm) or secondary (e.g. scleroderma) (Table 2.3).

TABLE 2.3 Classification of oesophageal motility disorders

Examination of the patient with dysphagia

In the context of oesophageal dysphagia, the physical examination is generally unremarkable. However one should examine the skin for features compatible with connective tissue disorders particularly scleroderma and CREST syndrome. Muscle weakness or wasting might be evident if myositis is present, and this can overlap with other connective tissue disorders affecting the oesophagus. Signs of malnutrition, weight loss and pulmonary complications from possible aspiration should be looked for. If pharyngeal dysphagia is suspected, careful evaluation for neuromuscular disorders is important (see below).

Diagnostic tests for oesophageal dysphagia

The most valuable investigations include a barium swallow, endoscopy and oesophageal manometry. Endoscopy will frequently obviate the need for barium radiology. However, the barium swallow, particularly with the addition of video sequences of the oesophageal body, can be very useful when the endoscopy fails to identify an abnormality and/or when oesophageal motility studies are atypical or equivocal. For example, one might identify so-called ‘corkscrew’ oesophagus indicative of diffuse oesophageal spasm. The hallmark of achalasia is oesophageal dilatation, a ‘birdbeak’ tapering at the cardio-oesophageal junction and the oesophagus typically supports a column of barium, often mixed with food and mucus. However, more subtle radiographic features in the early stages of achalasia might include loss of mid and distal oesophageal peristalsis and delayed clearance of barium from the distal oesophagus. Barium radiology will identify many structural abnormalities such as diverticula, strictures, rings and webs, tumours and ulcerative pathology.

Endoscopy is virtually always indicated in the dysphagic patient. It is necessary to identify and biopsy ulcerative pathology, malignancy and infective oesophagitis. A normal endoscopy, however, does not rule out a structural abnormality. Consider taking mid-oesophageal biopsies to rule out eosinophilic oesophagitis (abnormal mucosal eosinophilia in the oesophagus on histology [>24 eosinophils/ high power field]) in the setting of any case of unexplained dysphagia or food impaction. The ringed oesophagus is not always apparent unless adequate distension or insufflation of the oesophagus is achievable. The so-called ‘multi-ringed’ oesophagus, characteristic of eosinophilic oesophagitis, may have very subtle features such as longitudinal furrows, the ‘feline’ oesophagus with typical corrugation compatible with longitudinal shortening as well as the development of mucosal rings. Reflux oesophagitis and ulcerative oesophagitis (e.g. herpes simplex, cytomegalovirus [CMV], Candida) have typical appearances. Strictures can be biopsied and dilated at the time of endoscopy. The finding of food, fluid or salivary residue within the oesophagus is highly suggestive of dysmotility, particularly achalasia.

If endoscopy is normal or the history is suggestive of dysmotility, oesophageal manometry is the best way to confirm this. The characteristic features of achalasia are failure of lower oesophageal sphincter relaxation and aperistalsis. Typical although not always present is a hypertensive lower oesophageal sphincter. Oesophageal spasm is diagnosed in the context of normal sphincter relaxation, synchronous oesophageal pressure waves (>20% of wet swallows) and intermittent, normally progressive oesophageal peristalsis. Variably present, but not necessary for diagnosis, are high amplitude oesophageal pressure waves (>180 mmHg) and prolonged (>6 seconds) oesophageal pressure waves. The features of scleroderma oesophagus are complete absence of peristalsis and absence of lower oesophageal sphincter tone. Despite the virtual absence of propulsive motor activity in the scleroderma oesophagus, dysphagia in this syndrome is generally related to reflux-induced strictures. While manometry is the gold standard for diagnosis of motor disorders, in equivocal or atypical cases (approximately 5%) this must be interpreted in the context of the clinical and radiological features.

Diagnostic approach in suspected oropharyngeal dysphagia

The aetiology of pharyngeal dysphagia can be considered in 2 broad categories; structural disorders or neuromyogenic causes (Table 2.2). The range of potential neuromyogenic causes of pharyngeal dysphagia is broad but the most common is stroke. At least 50% of stroke patients experience pharyngeal dysphagia. While considering the likely underlying cause in a patient, there are four fundamental issues to consider in the work-up of these patients as listed in Table 2.4.

TABLE 2.4 Approach to oropharyngeal dysphagia

PAIN ON SWALLOWING (ODYNOPHAGIA)

Odynophagia is the symptom of pain on swallowing, generally arising from irritation of an inflamed or ulcerated mucosa by the swallowed bolus during its passage through the pharynx or oesophagus. The aetiology is generally infective, inflammatory or corrosive (Table 2.5). Infective causes are most often encountered in the immunocompromised host. The symptom of odynophagia nearly always warrants endoscopic investigation.

TABLE 2.5 Aetiology of odynophagia

SUMMARY

A good history will elucidate the site and the general pathophysiological process in 80% of cases. The aims of the history are to:

The most important investigations for oesophageal dysphagia include a barium swallow, endoscopy and oesophageal manometry. The aetiology of suspected oropharyngeal dysphagia can be considered in two broad categories: structural disorders or neuromyogenic causes. A multidisciplinary approach to the management of oropharyngeal dysphagia is essential.

The aetiology of odynophagia is usually infective, inflammatory or corrosive. Odynophagia in reflux disease almost always occurs in the context of oesophagitis. Other causes of odynophagia include; oesophageal candidiasis, herpes simplex virus (type 1), cytomegalovirus, drug-induced oesophageal ulceration and caustic oesophageal injury.