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CLINICAL UPDATE

American Society for Gastrointestinal Endoscopy


Editor: Grace Elta, MD ISSN 1070-7212 Vol. 11, No. 4 April 2004

Commentary
Dysphagia is a common esophageal symptom with nearly 1 in 5 subjects older than 50 years describing this symptom in epidemiologic surveys. It is the second most common indication for upper endoscopy in the United States. There is a myriad list of possible etiologies for dysphagia. Consequently, a systematic approach to the differential diagnosis resulting in a well planned investigation is important. As described by Drs. Katzka and Kochman, a carefully obtained medical history is the initial step. Upper endoscopy is almost always indicated as part of the evaluation and treatment.
Grace Elta, MD Editor

DIAGNOSTIC APPROACH FOR DYSPHAGIA


David A. Katzka, MD Michael L. Kochman, MD, FACP
University of Pennsylvania Health System Hospital of the University of Pennsylvania Philadelphia, Pennsylvania
Dysphagia is defined as difficulty with swallowing or a sensation of the ingested food or liquid sticking or pooling at some point above the stomach. This is in contrast to odynophagia, or painful swallowing. Characterization of dysphagia is accomplished by noting the location of the sensation of food sticking, duration of sticking sensation, occurrence with liquids (thin versus thick) and solids (soft versus hard), association with pain or coughing, presence or absence of regurgitation, need for liquids to wash down the food, and the presence of difficulty clearing saliva.1 Depending on the etiology, dysphagia may also be accompanied by nonesophageal symptoms. For example, patients with myasthenia gravis who present with dysphagia may note greater difficulty at dinner time than with breakfast or have other symptoms such as muscle weakness or ptosis. Patients with a reflux-induced stricture may have a history of heartburn or chest pain before effective proton pump inhibitor therapy.2 Patients with a proximal esophageal stricture may have a history of pemphigus vulgaris or lichen planus. Thus, a careful history in patients with dysphagia involves not only a detailed characterization of the cardinal symptom but also the potential associated symptoms. Finally, compensatory symptoms must also be elicited.3 Patients with dysphagia commonly eat slowly and chew carefully, eat small amounts in the form of snacking or frequent small meals, may refuse to go out for meals to avoid demonstrating potential symptoms in public, and may follow a diet that is calorically adequate but markedly restricted from a societal point of view.

CHARACTERIZATION, LOCALIZATION, AND ETIOLOGY OF DYSPHAGIA


The differential diagnosis of dysphagia is broad and includes such varied etiologies as mechanical obstruction, neoplasm, neuromuscular diseases, and connective tissue disorders (Table 1). Two general categories are suggested in the evaluation of dysphagia. The first is an anatomic categorization into diseases of the oropharynx and proximal esophagus and those of the esophageal body and lower esophageal sphincter (LES). The second categorization is into motility or anatomic causes of dysphagia. Thus, four subgroups of dysphagia are described: anatomic disorders of the upper esophagus, motility disorders of the upper esophagus, anatomic disorders of the lower esophagus, and motility disorders of the lower esophagus. Generally dysphagia to solids implicates a mechanical cause, whereas dysphagia to both liquids and solids suggests dysmotility. Almost all chronic causes of dysphagia result in accommodation symptoms including slow eating, alteration of diet, avoidance of social eating situations, and learned maneu-

Table 1. Differential diagnosis of dysphagia


Mechanical obstruction Reflux-induced strictures Schatzki ring Drug- and caustic-induced strictures Dysphagia lusoria or aortica Gastrointestinal stromal tumors Zenkers diverticulum Inflammatory Eosinophilic esophagitis Neoplasm Esophageal squamous cell carcinoma Esophageal adenocarcinoma Gastric carcinoma Lung cancer Breast cancer Neuromuscular disease Cerebrovascular accident Amyotrophic lateral sclerosis Myasthenia gravis Parkinsons disease Polymyositis Cricopharyngeal bar Hypothyroidism Amyloidosis Botulism Polio Shy-Drager syndrome Connective tissue disorders Scleroderma Dermatologic disease Epidermolysis bullosa Lichen planus Pemphigus vulgaris Pseudoxanthoma elasticum

vers to aid bolus passage. Acute causes of dysphagia, particularly those associated odynophagia, implicate processes associated with mucosal ulceration or inflammation. Location of the complaints is of variable value; symptoms felt in the upper chest or base of the neck may reflect pathology in that location but may also indicate referred pain from a distal source. On the other hand, dysphagia felt in the lower chest tends to be more accurate in pointing to the site of dysfunction. The symptom of dysphagia may also reflect a sensory abnormality within the normal parameters of bolus transport and transit such as in functional globus. Oropharyngeal dysphagia due to motility problems may result in regurgitation or spitting of food. This results from the remarkably complex mechanisms involved in moving the bolus from mouth entry to the esophagus. Three oropharyngeal phases of swallowing are present: formation of the bolus through mastication and tongue motion, initiation of the swallow with transfer of the bolus to the oropharynx, and coordinated passage of the bolus through the upper esophageal sphincter (UES). Abnormalities of the first phase may be associated with drooling, complaints of inability to move the bolus to the back of the throat, and dysarthria due to poor tongue movement.

Abnormalities of initiation and transfer include attempts to make a swallow without success, multiple swallows for a single bolus, nasal regurgitation, and fatigue when eating. Problems with transfer and airway protection are associated with coughing while eating, aspiration pneumonia, multiple swallows to clear secretions, and hoarseness. Patients with oropharyngeal dysfunction have their greatest trouble with thin liquids because of the ability to pool and penetrate the larynx more easily than solids. Causes of oropharyngeal dysmotility are divided into neurologic and myogenic causes. Associated symptoms indicating more global cranial nerve or muscle involvement such as facial weakness, ptosis, dysarthria, nasal or hoarse voice, or neck and upper-extremity weakness may be present. Neurologic diseases of the cortex affect initiation and bolus preparation (eg, cerebrovascular accident, tumor). Diseases that affect the brain stem and cranial nerves (eg, tumor, polio, Parkinsons, ShyDrager, botulism) will affect virtually all aspects of oropharyngeal motility. Diseases of the spinal cord and peripheral nerves (eg, amyotrophic lateral sclerosis) will interfere with pharyngeal and UES function and abnormalities of striated muscle function (eg, polymyositis, myasthenia gravis) will cause pharyngeal weakness. Dysmotility of the oropharynx, as in cricopharyngeal bars or poor UES compliance and opening, may result in the formation of a Zenkers diverticulum.4 A Zenkers is unique in that it may cause dysphagia from both poor UES function and mechanical compression of the proximal esophagus by the diverticulum itself. Pure mechanical causes of oropharyngeal dysphagia include primary malignancies of the head and neck. Therapy for the malignancy may lead to stricture formation, which may be complicated by marked dysmotility of this area from secondary nerve and muscle injury due to dissection and radiation.5 Extrinsic tumors may also compress the pharynx, particularly in the case of thyroid cancers. Benign etiologies of pharyngeal, UES, and proximal esophageal stricture formation include webs, vascular compression from an aberrant congenital right subclavian artery (dysphagia lusoria), and stricture formation. Strictures of the proximal esophagus are due to caustic ingestion, reflux, eosinophilic esophagitis, pill-induced injury, and various skin diseases such as pemphigus vulgaris, lichen planus, epidermolysis bullosa dystrophica, and pseudoxanthoma elasticum.6 A history of skin disease is usually present before stricture formation, though proximal esophageal stricture formation may be the initial manifestation of these dermatologic conditions. Dysmotility causes of dysphagia in the esophageal body and LES are less frequent than in the proximal esophagus due to the reliance on local control mechanisms for distal esophageal function. As a result, there is only a short list of primary and secondary diseases that cause distal esophageal dysmotility; the prototypical disease is achalasia. Achalasia is classically defined as a complete absence of esophageal peristalsis in association with a hypertensive LES that incompletely relaxes; recent research has painted a more diverse definition. It has become clear that many patients with achalasia will not have all the classic criteria but the diagnosis may be based on a compilation of clinical, radiographic, and manometric findings. Achalasia is a disease for which accommodation is the rule and patients often describe the compensatory mechanisms such as

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slow eating, avoidance of social eating, or getting up and walking around during a meal.7 Development of esophageal diverticuli may be associated with LES dysfunction. Although diffuse esophageal spasm (DES) has been viewed as a distinct entity from achalasia, some investigators feel that it may be within the spectrum. This appears true in certain patients and explains their response to achalasia therapies. In contrast to DES and achalasia, patients may develop dysphagia in association with LES and esophageal body hypomotility, which is aperistalsis with a wide open LES. This may be seen both in gastroesophageal reflux disease and diseases that systemically affect esophageal motility through smooth muscle and/or enteric nervous system injury such as scleroderma, hypothyroidism, and amyloidosis. Mechanical causes of dysphagia in the distal esophagus and LES include both malignant and benign causes. Benign causes of stricture formation are common, with gastroesophageal reflux being the most common cause. These patients may present with solid food dysphagia with or without an antecedent history of heartburn or other typical reflux symptoms. Another common cause of dysphagia is Schatzki rings, thin membranous excursions of squamous mucosa into the esophageal lumen at the gastroesophageal junction. Eosinophilic esophagitis, an emerging disease most likely secondary to food allergy, caustic ingestion, or medication-induced injury (eg, potassium, tetracycline), may also account for benign strictures. Extrinsic processes may result in dysphagia including aortic or right atrial compression, mediastinal adenopathy, and lung cancer. Malignant causes of mechanical distal esophageal dysphagia are most likely adenocarcinoma or squamous cell carcinoma of the esophagus.8,9

Table 2. Diagnostic evaluation


1. History to elicit nature of dysphagia and accompanying symptoms. 2. Physical examination to exclude neuromuscular, dermatologic, and connective tissue disease. 3. Consideration for referral to gastroenterologist dependent upon suspected etiology and care plan. 4. Barium swallow and possible video study with speech pathologist for cases of suspected oropharyngeal disorders. 5. Endoscopy may be performed as first diagnostic and therapeutic step in those with suspected mid- or distalesophageal lesions or after barium study.

DIAGNOSTIC EVALUATION
After the history, a detailed neurologic examination, particularly of the cranial nerves and upper extremities, should be performed, particularly in patients with suspected oropharyngeal causes of dysphagia (Table 2).10 The neck should be examined for thyromegaly and adenopathy. Chest auscultation is essential for evaluation of signs of pneumonia or asthma associated with aspiration. One of the neglected aspects of the physical examination is asking the patient to eat or drink during the examination. What is often described by the patient as normal eating may be an amazingly abnormal process when witnessed personally by the physician. The next step of the evaluation is often a barium study, though this is increasingly replaced by endoscopy in selected cases. Often a simple barium esophagram may suffice, but frequently a video barium esophagram should be ordered, particularly if there is concern for oropharyngeal dysphagia. This allows for an anatomic and motility evaluation of the oropharynx and esophagus. The video swallow may also be performed in the presence of a speech therapist to better plan potential treatment options as therapeutic maneuvers may be performed during the time of the study. It needs emphasis that in this era of magnetic resonance imaging and computerized tomography, performance of excellent-quality barium radiography is becoming a lost art. As a result, the ordering physician must be aware of the technical expertise of the radiologist performing the examination and potentially alter ones evaluation. Often referral to a gastroenterologist should be pursued early in patients with suspected mechanical causes or non-neu-

rologic causes of dysphagia, both to aid in diagnosis and management and to exclude malignancy. Many experts support a role for esophagogastroduodenoscopy (EGD) as the first test for evaluating dysphagia. In some situations, this is valid, such as in patients with a suspected Schatzki ring or peptic stricture. Other indications for EGD as the first diagnostic test include acute pill-induced esophagitis and suspected gastroesophageal malignancy. Nevertheless, an endoscopy as the initial study in some cases of dysphagia may not be the best choice for the following reasons: 1) motility is poorly assessed; 2) the oropharynx and proximal esophagus are not well seen; 3) inadvertent perforation of a diverticulum, particularly a Zenkers diverticulum, may occur; and 4) therapeutic procedures may be planned in advance rather than repeating the procedure, such as endoscopic ultrasound for malignancy or pneumatic dilation for achalasia.11,12 Nevertheless, endoscopy is typically an essential part of the diagnostic evaluation and therapy, especially for disorders of the esophageal body and LES.13 Esophageal manometry has been used to evaluate dysphagia for several decades, but its clinical utility is declining. It is a primary diagnostic test for those patients with normal-appearing barium or endoscopic studies in the context of potential DES, achalasia, or marked esophageal hypomotility. Commonly, it is used in conjunction with endoscopy and barium esophagram to confirm achalasia or UES and pharyngeal dysfunction. Manometry is also operator dependent with accuracy improving with number of studies performed on a regular basis; when technicians or physicians perform esophageal manometry less than several times per week, its reliability and reproducibility may be questioned.14 Fiberoptic endoscopic evaluation of swallowing has become a valuable tool in the evaluation of oropharyngeal causes of dysphagia. Through this technique, a nasopharyngeal laryngoscopy is performed and a video obtained while the patient is swallowing various foods and dyes. Esophageal scintigraphy by radionuclide tracer may also add supportive information to primary studies. It is most useful when corroborating symptoms objectively, since food rather than barium is administered. It is also used in many studies as an objective measure of improvement, specifically achalasia. An emerging tool for dysphagia is esophageal impedance measurement.15 By measurement of the conductance time between electrodes on a transnasal esophageal catheter, esophageal transit time may be measured and the occurrence of retrograde bolus movement may be seen. Although it is still primarily used as a research

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Statements and opinions expressed in the articles and communications are those of the author(s) and not necessarily those of the Editor or Publisher. The Editor and Publisher disclaim any responsibility or liability for such material.

tool, it may replace many of the indications for esophageal manometry in the evaluation of dysphagia. Other complementary studies include chest radiography, computed tomography of the chest and upper abdomen, and endoscopic ultrasound, particularly for extrinsic or intramural lesions. If a neurologic cause is suspected, electromyography and magnetic resonance imaging of the brain may be needed. In selected circumstances, specialized testing such as anticholinesterase antibodies and single-fiber electromyogram for myasthenia gravis may be helpful. Specifics of the endoscopic therapies and medical management of the myriad disorders is beyond the scope of this monograph.16,17

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6. 7. 8. 9.

SUMMARY
Although the differential diagnosis of dysphagia is large, simple classification into mechanical versus dysmotility causes and oropharyngeal versus esophageal locations will make diagnosis easier. A careful history with attention to the food consistencies that provoke dysphagia, the chronicity of the complaint, and the association with respiratory or neurologic symptoms helps to focus the differential. Objective evaluation may start with a video barium swallow with or without a speech therapist. Endoscopy may be primarily performed in certain clinical situations, but it is almost always performed, often for confirmation or characterization of the diagnosis and for therapy. Other complementary testing including manometry, cross-sectional imaging, and neurologic evaluation will be dictated by the specific details of the case.
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