World Gastroenterology Organisation Global Guidelines

FigureDYSPHAGIA WGO Cascades—Global Guidelines Cascades—A Resource-sensitive Approach A gold standard approach is only feasible if the full range of diagnostic tests and medical treatment options are available. Such resources for the diagnosis and management of dysphagia may not be sufficiently available in every country. The World Gastroenterology Organisation (WGO) guidelines provide a resource-sensitive approach in the form of diagnostic and treatment cascades. A WGO cascade is a hierarchical set of diagnostic, therapeutic, and management options for dealing with risk and disease, ranked by the resources available. Introduction Dysphagia refers either to the difficulty someone may have with the initial phases of a swallow (usually described as “oropharyngeal dysphagia”) or to the sensation that foods and or liquids are somehow being obstructed in their passage from the mouth to the stomach (usually described as “esophageal dysphagia”). Dysphagia is thus the perception that there is an impediment to the normal passage of swallowed material. Food impaction1 is a special symptom that can occur intermittently in these patients. A key decision is whether the dysphagia is oropharyngeal or esophageal. This distinction can be made confidently on the basis of a very careful history, which provides an accurate assessment of the type of dysphagia (oropharyngeal vs. esophageal) in about 80% to 85% of cases.2 More precise localization is not reliable. Causes of Dysphagia When one is trying to establish the etiology of dysphagia, it is useful to follow the same classification adopted for symptom assessment—that is, to make a distinction between causes that mostly affect the pharynx and proximal esophagus (oropharyngeal or “high” dysphagia), on the one hand, and causes that mostly affect the esophageal body and esophagogastric junction (esophageal or “low” dysphagia), on the other. However, it is true that many disorders overlap and can produce both oropharyngeal and esophageal dysphagia. Thorough history-taking, including medication use, is very important, as drugs may be involved in the pathogenesis of dysphagia. In young patients, oropharyngeal dysphagia is most often caused by muscle diseases, webs, or rings. In older people, it is usually caused by central nervous system disorders, including stroke, Parkinson disease, and dementia. Normal aging may cause mild (rarely symptomatic3) esophageal motility abnormalities. Dysphagia in the elderly patient should not be attributed automatically to the normal aging process (Tables 1 and 2).TABLE 1: Causes of Oropharyngeal DysphagiaTABLE 2: Most Common Causes of Esophageal DysphagiaCLINICAL DIAGNOSIS An accurate history covering the key diagnostic elements is useful and can often establish a diagnosis with certainty. It is important to carefully establish the location of the perceived swallowing problem: oropharyngeal versus esophageal dysphagia. Oropharyngeal Dysphagia Clinical History Oropharyngeal dysphagia can also be called “high” dysphagia, referring to oral or pharyngeal locations. Patients have difficulty in initiating a swallow, and they usually identify the cervical area as the area presenting a problem. In neurological patients, oropharyngeal dysphagia is a highly prevalent comorbid condition associated with adverse health outcomes including dehydration, malnutrition, pneumonia, and death. Impaired swallowing can cause increased anxiety and fear, which may lead to patients avoiding oral intake—resulting in malnutrition, depression, and isolation. Frequent accompanying symptoms: Difficulty initiating a swallow, repetitive swallowing. Nasal regurgitation. Coughing. Nasal speech. Drooling. Diminished cough reflex. Choking (note that laryngeal penetration and aspiration may occur without concurrent choking or coughing). Dysarthria and diplopia (may accompany neurological conditions that cause oropharyngeal dysphagia). Halitosis in patients with a large, residue-containing Zenker diverticulum or in patients with advanced achalasia or long-term obstruction, with luminal accumulation of decomposing residue. Recurrent pneumonia. Precise diagnosis is possible when there is a definite neurological condition accompanying the oropharyngeal dysphagia, such as: Hemiparesis following an earlier cerebrovascular accident. Ptosis of the eyelids and fatigability, suggesting myasthenia gravis. Stiffness, tremors, and dysautonomia, suggesting Parkinson disease. Other neurological diseases, including cervical dystonia and compression of the cranial nerves, such as hyperostosis or Arnold-Chiari deformity (hindbrain herniations). Specific deficits of the cranial nerves involved in swallowing may also help pinpoint the origin of the oropharyngeal disturbance, establishing a diagnosis. Testing Tests for evaluating dysphagia can be chosen depending on the patient’s characteristics, the severity of the problem, and the available expertise. Stroke patients should be screened for dysphagia within the first 24 hours after the stroke and before oral intake, as this leads to a 3-fold reduction in the risk of complications resulting from dysphagia. Patients with persistent weight loss and recurrent chest infections should be urgently reviewed.5 A bedside swallow evaluation protocol has been developed by the American Speech-Language-Hearing Association (ASHA); a template is available at: http://www.speakingofspeech.info/medical/BedsideSwallowingEval.pdf. This inexpensive bedside tool provides a detailed and structured approach to the mechanisms of oropharyngeal dysphagia and its management, and it may be useful in areas with constrained resources. Major tests for evaluating oropharyngeal dysphagia are: Video fluoroscopy, also known as the “modified barium swallow” This is the gold standard for evaluating oropharyngeal dysphagia.6–8 Swallowing is recorded on video during fluoroscopy, providing details of the patient’s swallowing mechanics. It may also help predict the risk of aspiration pneumonia.9 Video-fluoroscopic techniques can be viewed at slower speeds or frame by frame and can also be transmitted via the Internet, facilitating interpretative readings at remote sites.10 Upper endoscopy Nasoendoscopy is the gold standard for evaluating structural causes of dysphagia6–8—for example, lesions in the oropharynx—and inspection of pooled secretions or food material. This is not a sensitive means of detecting abnormal swallowing function. It fails to identify aspiration in 20% to 40% of cases when followed up with video fluoroscopy, due to the absence of a cough reflex. Fiberoptic endoscopic evaluation of swallowing (FEES) FEES is a modified endoscopic approach that involves visualizing the laryngeal and pharyngeal structures through a transnasal flexible fiberoptic endoscope while food and liquid boluses are given to the patient. Pharyngoesophageal high-resolution manometry (HRM) This is a quantitative evaluation of the pressure and timing of pharyngeal contraction and upper esophageal relaxation. It can be used in conjunction with video fluoroscopy to allow a better appreciation of the movement and pressures involved. It may have some value in patients with oropharyngeal dysphagia despite a negative conventional barium study. It may be useful when surgical myotomy is being considered. Automated impedance manometry11 This is a combination of impedance and HRM. Pressure-flow variables derived from automated analysis of combined manometric/impedance measurements provide valuable diagnostic information. When they are combined to provide a score on the swallow risk index, these measurements are a robust predictor of aspiration. Water swallow test This is inexpensive and is a potentially useful basic screening test alongside the evidence obtained from the clinical history and physical examination. It involves the patient drinking 150 mL of water from a glass as quickly as possible, with the examiner recording the time taken and number of swallows. The speed of swallowing and the average volume per swallow can be calculated from these data. It is reported to have a predictive sensitivity of >95% for identifying the presence of dysphagia, and it may be complemented by a “food test” using a small amount of pudding placed on the dorsum of the tongue.12 The algorithm shown in Figure 1 provides an indication of more sophisticated tests and procedures that are needed to pursue a diagnostic investigation leading to specific therapies.FIGURE 1: Evaluation and management of oropharyngeal dysphagia. CNS indicates central nervous system.Esophageal Dysphagia Differential Diagnosis The most common conditions associated with esophageal dysphagia are: Peptic stricture—occurs in up to 10% of patients with gastroesophageal reflux disease,13,14 but the incidence decreases with proton-pump inhibitor use. Esophageal neoplasia—including cardia neoplasia and pseudoachalasia. Esophageal webs and rings. Achalasia, including other primary and secondary esophageal motility disorders. Scleroderma. Spastic motility disorders. Functional dysphagia. Radiation injury. Rare causes: Lymphocytic esophagitis. Cardiovascular abnormalities. Esophageal Crohn’s involvement. Caustic injury. Clinical History Esophageal dysphagia can also be called “low” dysphagia, referring to a probable location in the distal esophagus—although it should be noted that some patients with forms of esophageal dysphagia such as achalasia may perceive it as being located in the cervical region, mimicking oropharyngeal dysphagia. Dysphagia that occurs equally with solids and liquids often involves an esophageal motility problem. This suspicion is reinforced when intermittent dysphagia for solids and liquids is associated with chest pain. Dysphagia that occurs only with solids but never with liquids suggests the possibility of mechanical obstruction, with luminal stenosis to a diameter of <15 mm. If the dysphagia is progressive, peptic stricture or carcinoma should be considered in particular. It is also worth noting that patients with peptic strictures usually have a long history of heartburn and regurgitation, but no weight loss. Conversely, patients with esophageal cancer tend to be older men with marked weight loss. In case of intermittent dysphagia with food impaction, especially in young men, eosinophilic esophagitis should be suspected. The physical examination of patients with esophageal dysphagia is usually of limited value, although cervical/supraclavicular lymphadenopathy may be palpable in patients with esophageal cancer. Some patients with scleroderma and secondary peptic strictures may also present with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal involvement, sclerodactyly, and telangiectasia). Halitosis is a very nonspecific sign that may suggest advanced achalasia or long-term obstruction, with accumulation of slowly decomposing residues in the esophageal lumen. The clinical history is the cornerstone of evaluation and should be considered first. A major concern with esophageal dysphagia is to exclude malignancy. The patient’s history may provide clues. Malignancy is likely if there is: A short duration—<4 months. Disease progression. Dysphagia more for solids than for liquids. Weight loss. In contrast, achalasia is more likely if: There is dysphagia for both solids and liquids. Dysphagia for liquids strongly suggests the diagnosis. There is passive nocturnal regurgitation of mucus or food. There is a problem that has existed for several months or years. The patient takes additional measures to promote the passage of food, such as drinking or changing body position. Eosinophilic esophagitis is more likely if there is: Intermittent dysphagia associated with occasional food impaction. Testing The medical history is the basis for initial testing. Patients usually require early referral, as most will need an endoscopy. The algorithm shown in Figure 2 outlines management decision making on whether endoscopy or a barium swallow should be the initial test employed.FIGURE 2: Evaluation and management of esophageal dysphagia. GERD indicates gastroesophageal reflux disease. Endoscopic evaluation: A video endoscope (fiberoptic endoscopes have largely been replaced by electronic or video endoscopes) is passed through the mouth into the stomach, with detailed visualization of the upper gastrointestinal tract. If available, high-resolution video endoscopy can be used to detect subtle changes, such as the typical whitish islands in eosinophilic esophagitis. Introducing the endoscope into the gastric cavity is very important to exclude pseudoachalasia due to a tumor of the esophagogastric junction. Endoscopy makes it possible to obtain tissue samples and carry out therapeutic interventions. Endoscopic ultrasound is useful in some cases of outlet obstruction. Barium-contrast esophagram (barium swallow): Barium esophagrams taken with the patient supine and upright can outline irregularities in the esophageal lumen and identify most cases of obstruction, webs, and rings. A barium examination of the oropharynx and esophagus during swallowing is the most useful initial test in patients with a history or clinical features suggesting a proximal esophageal lesion. In expert hands, this may be a more sensitive and safer test than upper endoscopy. It can also be helpful for detecting achalasia and diffuse esophageal spasm, although these conditions are more definitively diagnosed using manometry. It is useful to include a barium tablet to identify subtle strictures. A barium swallow may also be helpful in dysphagic patients with negative endoscopic findings if the tablet is added. A full-column radiographic evaluation15 is helpful if a subtle mechanical impediment is suspected despite a negative upper endoscopic evaluation. A timed barium esophagram is very useful for evaluating achalasia before and after treatment. Esophageal manometry: This diagnostic method is based on recording pressure in the esophageal lumen using either solid-state or perfusion techniques. Manometry is indicated when an esophageal cause of dysphagia is suspected after an inconclusive barium swallow and endoscopy, and following adequate antireflux therapy, when healing of the esophagitis has been confirmed endoscopically. The 3 main causes of dysphagia that can be diagnosed using esophageal manometry are achalasia, scleroderma, and esophageal spasm. Esophageal HRM with esophageal pressure topography: Is used to evaluate esophageal motility disorders. Is based on simultaneous pressure readings with catheters with up to 36 sensors distributed longitudinally and radially for readings within sphincters and in the esophageal body, with a 3-dimensional plotting format for depicting the study results (esophageal pressure topography). The Chicago Classification (CC) diagnostic algorithmic scheme allows hierarchical categorization of esophageal motility disorders. CC has clarified the diagnosis of achalasia and of distal esophageal spasm. Radionuclide esophageal transit scintigraphy: The patient swallows a radiolabeled liquid (eg, water mixed with technetium Tc 99m sulfur colloid or radiolabeled food), and the radioactivity in the esophagus is measured. Patients with esophageal motility disorders typically have delayed passage of the radiolabel from the esophagus. Motility abnormalities should therefore be suspected in patients with negative endoscopy and an abnormal transit time. When barium tests and HRM impedance testing are used, there is little additional value for esophageal scintigraphy. Diagnostic Cascades Tables 3 and 4 provide alternative diagnostic options for situations with limited resources, medium resources, or “state-of-the-art” resources.TABLE 3: Cascade: Diagnostic Options for Oropharyngeal DysphagiaTABLE 4: Cascade: Diagnostic Options for Esophageal DysphagiaTREATMENT OPTIONS Oropharyngeal Dysphagia The goals of treatment are to improve the movement of food and drink and to prevent aspiration. The cause of the dysphagia is an important factor in the approach chosen (Table 5).TABLE 5: Oropharyngeal Dysphagia: Causes and Treatment ApproachThe management of complications is of paramount importance. In this regard, identifying the risk of aspiration is a key element when treatment options are being considered. For patients who are undergoing active stroke rehabilitation, therapy for dysphagia should be provided to the extent tolerated. Simple remedies may be important—for example, prosthetic teeth to fix dental problems, modifications to the texture of liquids16 and foodstuffs,17 or a change in the bolus volume. Swallowing rehabilitation and reeducation: Appropriate postural, nutritional, and behavioral modifications can be suggested. Relatively simple maneuvers during swallowing may reduce oropharyngeal dysphagia. Specific swallowing training by a specialist in swallowing disorders. Various swallowing therapy techniques have been developed to improve impaired swallowing. These include strengthening exercises and biofeedback. Nutrition and dietary modifications: Softer foods, possibly in combination with postural measures, are helpful. Oral feeding is best whenever possible. Modifying the consistency of food to thicken fluids and providing soft foods can make an important difference.18 be taken to and of the risk of to food swallowing possibly due to the increased and provided by treatment with an inhibitor to the cough may also be A soft feeding passed should be considered if there is a risk of or when oral not provide adequate feeding after stroke the and the in with endoscopic involves a into the stomach through a from an and if available this is usually to surgical The that feeding may be is in patients who are have a stroke, or who during the initial feeding should be used in the and or feeding in the at the causes of dysphagia, such as have been in up to of but their In contrast, and endoscopic myotomy in patients with Zenker diverticulum is a Esophageal Dysphagia dysphagia evaluation and In the most common cause is food impaction. There may be an of mechanical obstruction. is after of the food should be taken to the risk of by the A of management options for esophageal dysphagia that may be taken into is provided in Options for Esophageal Esophageal Peptic strictures are usually the of gastroesophageal reflux disease, but strictures can also be caused by The diagnosis has to Caustic strictures after of strictures. strictures. strictures. Eosinophilic esophagitis. When the stricture has been confirmed with a is the treatment of is an alternative but it may be antireflux therapy with proton-pump as dysphagia and decreases the need for esophageal in patients with peptic esophageal strictures. may be in some patients. 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Guidelines American of dysphagia. American of at: and for and aspiration in older at: Guidelines of Patients and of A Clinical Guidelines at: Association of Clinical at: