Postgraduate Medicine
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Dysphagia Russell D. Yang MD, PhD & Jorge E. Valenzuela MD To cite this article: Russell D. Yang MD, PhD & Jorge E. Valenzuela MD (1992) Dysphagia, Postgraduate Medicine, 92:7, 129-146, DOI: 10.1080/00325481.1992.11701537 To link to this article: http://dx.doi.org/10.1080/00325481.1992.11701537
Published online: 17 May 2016.
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Date: 03 July 2016, At: 11:15
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Dysphagia A practical approach to diagnosis
Russell D. Yang, MD, PhD
Jorge E. Valenzuela, MD
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Preview Impairment of swallowing may be due either to a motor disorder or to a structural lesion. In this article, Drs Yang and Valenzuela provide a simple algorithm to use in differential diagnosis of esophageal dysphagia and explain the appropriate use of radiographic, endoscopic, and manometric studies in pinpointing the cause.
Difficulty in swallowing, or dysphagia, is rarely factitious or psychogenic and should always be evaluated systematically. It may be due to a motor disorder or to a lesion affecting the oropharynx or esophageal lumen. In general, motor disturbances produce episodic dysphagia, which either does not progress or does so slowly. Liquids are as difficult to swallow as solid foods. In contrast, structural lesions, which are more common than motor disturbances, produce relentless dysphagia that is initially worse with solid foods. As a lesion evolves, semisolids and liquids also become difficult to swallow.
Classification by location Dysphagia can be classified by the location of impairment, which may be oropharyngeal, esophageal, or esophagogastric. OROPHARYNGEAL DYSPHAGIA-
With oropharyngeal (transfer) dysphagia, patients have trouble
initiating a swallow or moving a food bolus from the mouth to the upper esophagus. Abnormalities of the jaw, lips, tongue, velum, larynx, or pharynx may cause (1) poor oral control and insufficient mastication of solidfood boluses, (2) delayed initiation of the swallowing reflex, and (3) poor clearance of food residue in the pharynx, which may lead to aspiration into the trachea before, during, or after swallowing. Patients and/or their relatives should be carefully questioned about such common complaints as coughing, aspiration, nasal regurgitation, sneezing, and weight loss from avoiding food. Neuromuscular disorders cause 75% to 80% of cases, and other manifestations of a generalized process may be present. Rarely, a local structural lesion of the oropharynx is implicated. A small number of patients have no associated systemic or local disease. This group is believed to have abnormal function
of the upper esophageal sphincter. The term "cricopharyngeal achalasia" is used to describe this entity, although it is often difficult to document a true abnormality of the sphincter. Histologic examination of the cricopharyngeal muscle in these patients has shown fibrosis, and the syndrome may represent a disease of the muscle. An esophagogram may demonstrate a posterior indentation, or "bar," at the level of the pharyngoesophageal sphincter (figure 1), and manometric studies may confirm a lack of relaxation in this area. Some patients with Zenker's diverticulum have been shown to have cricopharyngeal achalasia, but the role of motor dysfunction in the pathogenesis of this syndrome is as yet unclear. Patients who have had neck surgery, radiation therapy, or a tracheostomy may also have oropharyngeal dysphagia. Motility defects in these patients result from local scar formation or from disruption of neural and muscular structures at the level of the pharynx and upper esophageal sphincter. Table 1 lists the various causes of oropharyngeal dysphagia. FSOPHAGEAL DYSPHAGIA-
With esophageal (transport) dysphagia, patients have difficulty
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Patients with esophageal dysphagia often can point to the level of obstruction.
Table 1. Causes of oropharyngeal dysphagia
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Central nervous system disease Cerebrovascular accident Parkinson's disease Multiple sclerosis Amyotrophic lateral sclerosis Brainstem tumors Huntington's chorea Tabes dorsalis
Peripheral nervous system disorders Bulbar poliomyelitis Peripheral neuropathy from diabetes or mononeuritis multiplex
Motor end-plate disease Myasthenia gravis
Skeletal muscle disorders Inflammatory disease (polymyositis, dermatomyositis, mixed connective tissue disease) Muscular dystrophies (myotonic, oculopharyngeal) Metabolic myopathies (thyrotoxic, myxedematous)
Local structural lesions Cancer Disruption from surgical resection Zenker's diverticulum Cervical spurs Thyromegaly Esophageal webs
Figure 1. Esophagogram in 78-year-old woman with oropharyngeal dysphagia showing indentation, or "bar" (arrow), in upper esophagus.
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moving a food bolus through the esophageal body; often they can point to the level of obstruction. The spectrum of the disorder ranges from mild, intermittent difficulties with solid foods to complete obstruction. Up to 85% of cases of esoph-
ageal dysphagia are associated with structural or mechanical abnormalities of the esophagus. The most common abnormalities are strictures (figure 2), adenocarcinoma or squamous cell carcinoma (figure 3), and lower esophageal (Schatzki's) rings.
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Most cases of dysphagia can be diagnosed on the basis of history alone.
Most patients with structural abnormalities have difficulty swallowing solid food when the lumen size is less than 13 mm. Clinically, this may present as the "steakhouse syndrome" if a piece of meat becomes lodged in the narrowed area. Symptoms are considered to be progressive if swallowing difficulties start with solid foods and gradually evolve to liquids as a result of continued narrowing by stricture or an enlarging carcinoma. The remaining 15% of patients with esophageal dysphagia have a motility disorder, which usually causes difficulty in swallowing both solid foods and liquids (particularly cold liquids). Achalasia is the most common motility abnormality, followed by nonspecific esophageal motility disorder and diffuse esophageal spasm. When esophageal manometry studies are done, diffuse esophageal spasm is defined as the presence of simultaneous, repetitive contractions with intermittent normal peristalsis. Achalasia, however, is manifested by aperistalsis of the esophageal body with a hypertensive lower esophageal sphincter that relaxes incompletely after a swallow. Table 2 lists disorders to be considered in differential diagnosis of esophageal dysphagia.
Figure 3. Endoscopic appearance of constricting esophageal lesion in 58-yearold man who was heavy smoker. Brush cytology and biopsies confirmed presence of squamous cell carcinoma. Figure 2. Esophagogram in 70-year-old man with long history of heartburn and regurgitation. Stricture (arrow) is seen near junction of upper two thirds and lower one third of esophageal body.
FSOPHAGOGASfRIC DYSPHAGIA-With
this condition, the patient has difficulty passing a food bolus through the region of the lower esophageal sphincter into the stomach. Features are similar to those of esophageal dysphagia.
History taking Most cases of dysphagia can be diagnosed on the basis of history alone. In patients with oropharyngeal dysphagia, it is important
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to establish that the symptoms occur only during the act of swallowing. If the disorder is psychogenic (ie, globus hystericus), symptoms are present all the time. In patients with esophageal dysphagia, three questions help establish the cause: ( 1) Is dysphagia associated with liquids, solids, or both? (2) Is dysphagia progressive, or are symptoms intermittent? (3) Are there associated symptoms? Figure 4 is a schema to assist continued
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Regurgitation in patients with dysphagia is distinguished from vomiting by its passive nature and the lack of an acid or bitter taste.
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Table 2. Causes of esophageal dysphagia Mechanical disorders Intrinsic • Esophageal carcinoma • Benign tumor • Peptic stricture • Strictures caused by ingestion of caustic material • Lower esophageal (Schatzki's) ring • Esophageal webs Extrinsic • Vascular compression (eg, dysphagia lusoria, dysphagia aortica) • Mediastinal abnormalities (enlarged lymph nodes, substernal thyroid gland)
Motor disorders Achalasia Chagas' disease Diffuse esophageal spasm Scleroderma and other connective tissue diseases Nonspecific esophageal motor dysfunction Amyloidosis Diabetic and alcoholic neuropathy
in differential diagnosis of esophageal dysphagia.
Associated symptoms A number of manifestations associated with dysphagia may be helpful in determining the cause. These include heartburn, odynophagia, chest pain, regurgitation, aspiration, weight loss, anorexia, and anemia. HEAIITBURN-Heartburn is an important historical clue. Reflux of gastric acid from the stomach
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onto esophageal mucosa is manifested by a substernal burning sensation that may radiate toward the mouth. Symptoms are worse after meals and when the patient lies down or bends over. Heartburn associated with solid-food dysphagia is usually a manifestation of chronic gastroesophageal reflux and peptic stricture. ODYNOPHAGIA-This refers to pain on swallowing and is a feature of inflammatory lesions of the esophagus caused by, for
example, thrush (candidiasis) or ingestion of caustic material. Odynophagia should not be confused with dysphagia. Although patients may have both when the esophagus is inflamed, dysphagia is usually painless. NONCARDIAC CHFST PAINThe character and distribution of chest discomfort with esophageal dysphagia can mimic that of cardiac pain, but it is usually possible to distinguish the two. In our experience, chest pain is associated more often with reflux esophagitis than with spastic motor disorders. REGURGITATION-Swallowed food may be returned from stomach to esophagus or from esophagus to pharynx and oropharynx. This process is distinguished from vomiting by its passive nature and by the lack of an acid or birter taste. In contrast, vomiting is usually accompanied by nausea and retching with abdominal contractions. Occasionally, regurgitation is associated with dysphagia when emptying of the esophagus is incomplete (ie, in cases of achalasia). ASPIRATION-This is caused by spillage of esophageal or gastric contents into the tracheobronchial tree. Aspiration is usually related to an obstructive lesion of the esophagus, achalasia,
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Lymphadenopathy in a patient with structural dysphagia is highly indicative of carcinoma.
or gastroesophageal reflux. With an obstructive lesion, aspiration may lead to pneumonia and even death. In cases of reflux, symptoms of bronchial asthma may occur. WEIGHT LOss--In a patient with dysphagia, weight loss is due to inability to maintain adequate caloric intake. Weight loss due to anorexia is an ominous sign of esophageal carcinoma. Alternatively, elderly patients who have subclinical embolic phenomena with oropharyngeal dysphagia may refuse to eat. 01HER SYSTEMIC SIGNS AND
SYMP'IOMS--Also important in determining the cause of dysphagia are anemia, which may be due to carcinoma or esophageal webs; melena due to carcinoma; Raynaud's phenomenon with connective tissue disease; constipation; megacolon from chronic Chagas' disease; hypothyroidism; diabetes; and generalized neuromuscular disease.
Physical examination The physical examination in patients with dysphagia is usually unremarkable. However, if dysphagia is chronic or progressive, the patient may appear malnourished or show evidence of weight loss. Examination of the oropharynx may reveal thrush or
Dysphagia
~
Type of food involved
I Solids and liquids (motor dysphagia)
Solids initially (structural dysphagia)
I
I
~
Intermittent
~
Chest pain
~
Diffuse esophageal spasm
~
l
n
Progressive
Heartburn
l
Intermittent
~
No heartburn
Esophageal ring
l
n
Progressive
Heartburn
l
~
Scleroderma Achalasia
Peptic stricture
No heartburn
~
Carcinoma
Figure 4. Differential diagnosis of esophageal dysphagia.
leukoplakia of the labial mucosa (Plummer-Vinson syndrome). Dermatologic examination may show telangiectasias, subcutaneous calcifications, or sclerodactyly suggestive of scleroderma. Tylosis (hyperkeratosis of the hands and feet) is associated with esophageal carcinoma. Dystrophic skin changes characterize epidermolysis bullosa, which is associated with esophageal narrowing. Lymphadenopathy in a patient with structural dysphagia is highly indicative of carcinoma.
A prolonged swallowing time (normal, 6 to 10 seconds) with absence of fluid passing into the stomach is suggestive of achalasia. Abnormal neurologic findings may be due to an underlying motility problem.
Diagnostic evaluation Acute onset of dysphagia while eating often demands an immediate esophagoscopic examination to identifY and remove an impacted food bolus. For chronic symptoms of dys-
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All mechanical obstructions of the esophagus should be evaluated by endoscopy so that tissue samples can be obtained.
Figure 5. Chest film showing considerable dilation of the esophageal body. Lumen of esophagus is partially filled with barium, mucus, food, and secretions. Aspirated contrast medium is visible in airways. Lower esophageal sphincter appears closed. Esophagoscopy demonstrated a smooth appearance of the sphincter, which opened intermittently.
phagia, evaluation usually includes either a barium swallow and cine-esophagogram (video esophagogram) or upper gastrointestinal endoscopy. Esophagography delineates both structural and motor abnormalities. However, special care may be needed in distending the esophagus to define lower esophageal rings or early strictures. If liquid barium fails to reveal a cause for dysphagia, a solid, 12.5-mm barium tablet or hamburger may be given. The barium tablet will be stopped at the point of obstruction, which can be shown radiographically. Luminal narrowing shown when a tablet larger than
12.5 mm is used is rarely of clini-
cal significance. All mechanical obstructions should be evaluated by endoscopy so that tissue samples can be obtained. The diagnostic yield is greatest when both biopsy and brush cytology are done. This is especially true for strictures and masses. In a patient with odynophagia and dysphagia, endoscopy is also useful to obtain inflamed tissue for culture. All patients with achalasia should have endoscopy because it is often possible to distinguish primary from secondary causes by direct visualization and biopsy of tissue. Endoscopy can also be
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used as a safe means of throughthe-scope balloon dilation for patients with strictures. A chest film is usually normal in patients with dysphagia. Occasionally, a hugely dilated esophagus with air-fluid level may be seen in cases of long-standing stricture or achalasia (figure 5). A gastric air bubble often is absent in patients with achalasia or esophagogastric carcinoma. If the diagnosis is unclear after radiographic or endoscopic studies have been done to eliminate structural causes of dysphagia, esophageal manometry is useful to evaluate motor disorders. Endoscopic diagnosis of motility disorders is inaccurate and may be misleading. As mentioned earlier, manometric findings are specific with diffuse esophageal spasm and achalasia. Furthermore, in diseases such as scleroderma, an absence of peristalsis is associated with an incompetent lower esophageal sphincter. Patients with polymyositis show impaired peristalsis of the upper esophagus.
Summary Our diagnostic approach to a patient with dysphagia begins with detailed history taking
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Russell D. Yang, MD, PhD Jorge E. Valenzuela, MD Dr Yang (left) is assistant professor of medicine and codirector of clinical gastroenterology training and Dr Valenzuela (right) is professor of medicine, division of gastrointestinal and liver diseases, University of Southern California School of Medicine, Los Angeles.
and physical examination. On the basis of fmdings, a radiographic and/or endoscopic study of the esophagus is done. We usually have bariumswallow radiography done initially, and if the radiographs are equivocal, upper gastrointestinal endoscopy is performed. Manometric studies are reserved for patients with suspected motility disorders. PCIWI
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Address for correspondence: Jorge E. Valenzuela, MD, Division of Gastrointestinal and Liver Diseases, Department of Medicine, Los Angeles County-University of Southern California Medical Center, 2025 Zonal Ave, LAC 12-100, Los Angeles, CA 90033.
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