• Accepted as the most accurate method for diagnosing reflux. It is, however, uncomfor-table and expensive investigation. Therefore, indicated especially:
If barium swallow or EGD studies are negative,
In patients being considered for antire-flux surgery, and
‡‡ This technique has been revolutionized by a catheter-less, wireless pH system.
101
In the evaluation of patients with chest pain of undetermined origin.
• An abnormal result is indicated by a pH of
<4 for more than 3% of the time when the patients are supine, and for > 8% of the time when they are erect.
Videofluoroscopic Swallowing Function Study (VSFS)‡‡
• The VSFS—performed jointly by a physician (typically a radiologist) and a speech-language pathologist—is the gold standard for evaluating the mechanism of swallo-wing. For this study, the patient is seated comfortably and given foods mixed with barium to make them radiopaque. The patient eats and drinks these foods while radiographic images are observed on a video monitor and recorded on videotape.
• VSFS permits close observation of all structures involved in swallowing process, i.e. lips, tongue, palate, pharynx, larynx, and proximal esophagus; indicated especially in patients at risk for silent aspiration, e.g. patients with stroke, or neurologic impairment such as cerebral palsy, MS, PD, MG, motor neuron disease, systemic sclerosis, and connective tissue disorders such as SLE, and RA.
• VSFS not only allows estimation of risks of aspiration and respiratory complications, but also helps in determining dietary modifications and training in swallowing techniques and maneuvers.
Radionucleotide Study7 (Tc-99m Sulfur Colloid Bolus)
• These assess gastroesophageal reflux, esophageal motility disorders, or to identify silent aspiration.
In GERD, reflux is present if the isotope is seen to travel back up into the esophagus. In esophageal motility disorders, computer programs measure
transit time in the upper, middle and lower thirds of the esophagus. Scintigraphy also can be combined with 24-hour pH manometry to check the cause of chest pain between esophageal and possible cardiac factors.
CT Scan/MRI
• In suspected structural CNS abnormalities, e.g.
primary or secondary tumors, MS; or, aortic vascular lesions, e.g. aneurysm, lusorian artery.
Muscle Enzymes
• Elevated serum CK may be observed in motor neuron diseases, e.g. polymyositis, dermatomyositis.
TFTs
• To detect hypothyroid or hyperthyroid disorders causing dysphagia, e.g. Graves’
disease or thyroid carcinoma.
RF, ANA
• In patients with autoimmune disorders, e.g.
RA, SLE, scleroderma.
Antiacetylcholine Antibodies
• Antiacetylcholine receptor antibody activity may be elevated in patients suspected with MG, and polymyositis.
Genetic Analysis
• For example, in familial forms of ALS, or muscular dystrophy.
CLINICAL NOTES
• It is important to ascertain that the patient truly has dysphagia, because some may misinterpret ‘lump in the throat’ (globus) as dysphagia
• The clinical features of ‘globus’ sensation may occur with organic disease in the neck, pharynx, or cervical esophagus; hence this diagnosis should only be made after a complete evaluation
‡‡ The VSFS is similar to the MBS, except that the protocol for the MBS specifies quite small bolus volumes and does not include drinking from a cup. In practice, the terms
“VSFS” and “MBS” are often used interchangeably.
Diagnosis: A Symptom-based Approach in Internal Medicine 102
• By asking following specific questions, the cause of dysphagia can usually be identified:
Do you have difficulty swallowing? In what way?
Do you have this sensation without swallowing food?
Can you localize dysphagia?
Do you have trouble with solids or liquids?
Is the swallowing difficulty greater for solids or liquids?
Is the dysphagia intermittent or progressive?
Do you have heartburn?
Any eating habits adopted to relieve symptoms?
Are there any associated problems, e.g. stress, cough, chest pain, dysphonia, collagen disease, immunosuppression, weight loss?
• Generally, dysphagia at the very beginning of swallowing characterizes oropharyngeal dysphagia; whereas dysphagia a few seconds after starting to swallow indicates an esopha-geal cause
• Onset—Acute dysphagia is commonly due to oropharyngeal causes such as infection, inflammation, food bolus impaction, or stroke (see above). Intermittent dysphagia suggests esophageal dysmotility syndrome, webs, or rings. Progressive dysphagia indicates an organic cause such as achalasia, carcinoma, mediastinal expanding lesion, or other systemic disorder, e.g. PD, scleroderma
• The type of bolus that elicits symptoms of dysphagia may indicate its cause (Table 15.2)
• Odynophagia often accompanies dysphagia in esophagitis, pharyngitis, opportunistic infections, pill esophagitis, diffuse esopha-geal spasm, rheumatoid arthritis (inflam-mation of cricoarytenoid joint), or malignant process involving the mucosa
• Associated signs and symptoms as impor-tant clues for possible cause of dysphagia are given in Table 15.3
Table 15.2: Types of bolus causing dysphagia
Bolus Cause of dysphagia
Dysphagia with both Muscular or neural control liquids and solids from disorders of swallowing the outset (bulbar, pseudobulbar palsy);
motility disorders Dysphagia initially for Esophageal obstructive solids lesions (rings, webs,stricture) Intermittent, non- Benign obstructive lesions progressive dysphagia, (rings, webs, stricture);
more for solids than esophageal spasm liquids
Progressive dysphagia Obstructive inflammatory first for solids, then for stricture; achalasia;
semisolids and liquids malignancy (carcinoma esophagus); scleroderma Cold food or beverages Esophageal dysmotility, precipitating dysphagia especially spasm or causing chest pain
Table 15.3: Signs and symptoms of causes for dysphagia Signs and symptoms Diagnostic clues
Sudden dysphagia Obstructive dysphagia, esophagitis, brainstem stroke, acute thyroiditis, Intermittent symptoms Rings and webs, DES, NE Progressive symptoms Obstructive lesions,
carcinoma, scleroderma Difficulty initiating swallow Oropharyngeal dysphagia Food ‘sticks’ after swallow Esophageal dysphagia Pain with dysphagia Esophagitis—infection,
pill-induced
Regurgitation of old food, Zenker’s diverticulum halitosis
Ulcers on the tongue; Lesions obvious on tonsillar infection/abscess; examination of the oral retropharyngeal abscess cavity
Tongue fasciculations A L S
Pallor, koilonychia, Plummer-Vinson syndrome hypochromic microcytic
anemia
Mask-like facies, saliva Parkinson’s disease drooling from the mouth,
tremors, cog-wheel rigidity
Drooping of eyelids, Myasthenia gravis diplopia, swallowing
becomes more difficult as meal progresses, extreme weakness
Palpable mass medial to Pharyngeal pouch sternomastoid
Hoarseness of voice, Mediastinal syndrome, visible neck, goiter, recurrent laryngeal nerve tracheal compression palsy, retrosternal goiter Puckered and narrow
mouth, inability to open Scleroderma, progressive mouth, thickened-hard-and systemic sclerosis tight skin, Raynaud’s
Phenomenon
Dysphagia 103
• The swallow test8—The patient is observed during the act of swallowing liquids (a few ounces of water) and solids. Normally a person can drink/chew, mix, and propel a food bolus to the posterior pharynx without chocking or coughing, and no significant amount of material is retained after a swallow. Drooling, delayed swallow initiation, coughing, throat clearing, or a change in voice may indicate a disorder. After the swallow, the patient is observed for a minute or more to see if there is delayed cough response indicating aspiration
• Oropharyngeal examination for ulcers, tonsillar infection, patch on mucosa, movements of the tongue, epiglottis, and soft-pallet, as well as examination of neck for masses, thyromegaly, and lymphadenopathy is important. Tongue fasciculations suggest ALS
• Pharynx and Gag reflex— Does the uvula elevate symmetrically when the patient says, “Aah”?
Does the patient gag when the posterior pharynx is brushed? Unilateral movement of uvula or total absence of its upward movement, or gagging indicates palatal muscle paresis due to IX and X cranial nerve or brainstem lesion.
Noting the patient’s speech and voice often confirm the oropharyngeal causes of dysphagia. The gag reflex needs to be tested only in patients with suspected brainstem pathology, impaired consciousness, or impaired swallowing
• A decreased gag reflex, though commonly associated with an increased risk of aspiration, absence of a gag reflex does not necessarily indicate that a patient is unable to swallow safely
• Laryngoscopy— Indirect laryngoscopy helps to confirm the diagnosis of lesions of oropharynx, larynx, and hypopharynx.
Direct laryngoscopy may be indicated to evaluate obstructive or neuromuscular causes of oropharyngeal dysphagia. Changes in speech, hoarseness, or a weak cough may represent vocal cord paralysis. Slurred speech may indicate weakness or incoordination of
muscles involved in articulation and swallowing. Dysarthria, nasal speech, or regurgitation of food into the nose may represent weakness of the soft palate or pharyngeal constrictors. The combination of hoarseness, dysphonia (difficulty or pain in speaking), and nasal speech accompanying dysphagia is associated with the muscular dystrophies. Direct laryngoscopy also helps to differentiate dysphagia due to laryngopharyngeal reflux disorder (LPRD)§§, and GERD (Table 15.4). Laryngopharyngeal reflux (LPR) and GERD are different disorders.
The term LPR is used to describe the acid in the stomach that comes up into the throat at the level of the laryngopharynx. LPR causes irritation and changes in the larynx. GERD is caused by the backflow of gastric contents into the esophagus, which leads to tissue damage or esophagitis and heartburn.
Table 15.4: Difference between GERD and LPRD
GERD LPRD
Symptoms
Mainly supine reflux Mostly daytime, upright reflux
Heartburn and/or Heartburn and/or regurgitation common regurgitation unusual Hoarseness, cough, Hoarseness, cough, dysphagia, globus dysphagia, globus normally present not usually present Physical findings
Laryngeal inflammation Laryngeal inflammation
uncommon common
Dysfunction of the lower Dysfunction of the upper Esophageal esophageal sphincter sphincter
Test results
Normal pharyngeal pH Abnormal pharyngeal pH
monitoring monitoring
Abnormal esophageal pH Usually normal monitoring monitoring esophageal pH Dysmotility Good gastrointestinal
motility
Erosive or Barrett’s Erosive or Barrett’s Esophagus H uncommon esophagus may be present
§§LPRD is also termed as extraesophageal, reflux disorders, i.e. EERD.
Diagnosis: A Symptom-based Approach in Internal Medicine 104
• Neurologic examination includes testing of all cranial nerves, especially those involved in swallowing (sensory components of cranial nerves V, IX and X, and motor components of cranial nerves V, VII, X, XI and XII)
• Associated central nervous system deficits may suggest neuromuscular diagnosis, e.g. evidence of cogwheeling suggests Parkinson’s disease;
impaired memory indicates Alzheimer ’s disease or other dementia; ataxia, dementia or dysarthria suggests central nervous system disease.
RED FLAGS
• Esophageal dysphagia, e.g. spasm, reflux esophagitis, may mimic angina pectoris in every respect, including site (retrosternal);
radiation (neck, jaw, shoulder, thoracic spine, arm, epigastrium); nature (crushing or burning); vasovagal symptoms (pallor, sweating, tachycardia); and promptly relieved by nitroglycerine, or sublingual nifedipine, as in angina pectoris. The crucial historical differentiating points are—esophageal pain is generally much longer in duration (15-30 minutes), has no relation to exertion, and ECG or cardiac enzymes are normal
• In complicated cases, both esophageal and CAD may coexist; hence it is prudent to exclude CAD by more definitive tests such as barium esophagography, esophageal pH monitoring, Holter ECG monitoring, or stress thallium myocardial perfusion scintigraphy
• Consider esophageal disease as a possible cause in a patient with recurrent pneumonia;
symptoms are often nocturnal due to tracheal aspiration or regurgitation of esophageal contents
• A new onset dysphagia may indicate esophageal cancer, especially in the elderly
• Dysphagia due to recurrent oral/pharyn-geal ulcers or thrush may indicate an
immunocompromised patient, especially with HIV infection
• Although patients with dysphagia usually present with a variety of signs and symptoms, these can be quite subtle or even absent, e.g. in those with silent aspiration.
SELECTIVE GLOSSARY
Amyotrophic lateral sclerosis (ALS)—This is the most common variety of degenerative motor neuron disease, with a combination of UMN and LMN signs, although one type may predominate.
Virtually all patients with ALS have dysphagia - the initial complaintis food sticking at the level of the cervical esophagus.Bulbar involvement leads lip and tongue weakness, followed by jaw and suprahyoid weakness.This, in turn, leads to labial spillage, poor bolus propulsion,poor laryngeal elevation, depressed gag reflex, and poor airway protection. This disorder is sometimes associated with dementia or Parkinsonism.
Barrett’s esophagus—BE is a condition in which columnar cells replace the usual squamous cell in the mucosa of the esophagus. The condition is recognized as a complication of GERD or inflammatory disorders of the esophagus. It occurs more often in men than in women (3:1 ratio); with the average age at diagnosis being 55 years. Barrett’s esophagus does not produce symptoms distinct from GERD or esophageal inflammation. Most patients complain of heartburn pain, indigestion, blood in vomit or stool, difficulty in swallowing solid foods, or nocturnal regurgitation. Its importance lies in its predisposition to evolve into adenocar-cinoma in the esophagus. EGD is the procedure of choice for the diagnosis of BE. The diagnosis requires biopsy confirmation (from 4 quadrants at standard intervals within the esophagus).
Chromoendoscopy, i.e. vital staining with Lugol’s solution performed at the time of upper
Dysphagia 105
endoscopy to aid in cancer detection to identify abnormal mucosa, may be used as a means of esophageal cancer screening. In patients who are at increased risk for squamous cell carcinoma, the dye stains the glycogen in normal squamous epithelium a dark brown color. Areas that are unstained, particularly those that are larger than 5 mm in size, are likely to be dysplastic or malignant, and can be readily targeted for endoscopic biopsy.
Dysphagia lusoria—This is caused by a rare anomaly of the subclavian artery. This artery arises from the aortic arch distal of the left subclavian artery, crossing the midline, behind the esophagus. This abnormality is generally silent and often an incidental X-ray finding, but can result in dysphagia, which generally appears after the age of 40 years. The diagnosis can be overlooked at endoscopy, but barium contrast examination of the esophagus shows a characteristic diagonal impression at the level of the fourth thoracic vertebra. CT with contrast study or MR angiography confirm the diagnosis and exclude aneurysms.
Eosinophilic esophagitis—This is an emerging cause of dysphagia, typically seen in young adults. Often, the history of dysphagia dates to adolescence. In children it is responsible for feeding disorders, vomiting, reflux symptoms and abdominal pain, and in adults it causes intermittent solid food dysphagia or food impaction. The natural history of this disorder has not been well-characterized, but appears to be marked by periods of spontaneous remission and exacerbations that are not linked to specific factors, although food allergies and exposure have been implicated. Some have a history of atopy. Endoscopy may reveal a number of subtle features that include a
‘corrugated’ esophagus with fine rings; a diffusely narrowed esophagus that may have proximal strictures; the presence of linear
furrows, adherent white plaques, or a friable (crepe paper) mucosa, prone to tearing with minimal contact; or even normal looking esophagus. Frank esophagitis is not part of the macroscopic endoscopic picture of eosinophilic esophagitis. The most important element in the diagnosis of eosinophilic esophagitis is to know its macro- and micromorphological character-istics. Biopsies from the proximal to the distal esophagus demonstrating > 15-20 eosinophilic granulocytes per high powered field favor the diagnosis. With increasing recognition, this entity is taking its place as an established cause of solid food dysphagia.
Progressive systemic sclerosis—Patients with progressive systemic sclerosis usually present with heartburn, dysphagia, and regurgitation. The oral, pharyngeal, and esophagealphages of swallowing can all be affected. Progressive atrophyand fibrosis of esophageal smooth muscle is usually prominent. Gastroesophageal reflux is frequent. Esophageal manometrytypically shows lower pressures and sometimes aperistalsis.Poor peristalsis, decreased lower esophageal sphincter pressures,and gastroesophageal reflux can result in a transition froma patulous dilated esophagus to one that is strictured and scarred.Barrett esophagus may develop. The presence of oropharyngealdisease is usually accompanied by pulmonary disease. Patientswith progressive systemic sclerosis may also have xerostomia (which is frequently due to medications), dental problems (dueto fibrosis of the ligamentous tooth attachments), trigeminalnerve involvement (decreased bolus sensation), and lingual atrophy that can also contribute to dysphagia.
Schwartz ring (pronounced - shats-ke or schatz·ki’s ring)—This is narrowing in the lower part of the esophagus, consisting of a membrane-like structure, lined by squamous epithelium on its superior aspect and columnar epithelium inferiorly. Such a ring is quite common, being
Diagnosis: A Symptom-based Approach in Internal Medicine 106
detected in up to 10% of all upper GI barium X-rays. Few produce sufficient luminal obstruction to cause dysphagia. When the lumen is narrowed to a diameter of 13 mm or less, the patient will experience intermittent solid-food dysphagia or even episodic food-bolus obstruction.
Sjögren’s syndrome (pronounced — “SHOW-grins”)— SS is a chronic disease, probably due to auto- immunologic factors with genetic predis-position. Viral infections such as Epstein-Barr virus (EBV), human T-lymphotrophic virus 1 (HTLV-1), human herpesvirus 6 (HHV-6), human immunodeficiency virus 1 (HIV-1), hepatitis C virus (HCV), and cytomegalovirus (CMV) could be involved in the induction of SS. Women are affected more often than men — the female-to-male ratio is 9:1; usually occurring between 30-50 years.
The hallmark symptoms are the “sicca complex”, a combination of dry eyes (keratoconjunctivitis sicca—KCS) and dry mouth (xerostomia). SS can affect the oral phase of swallowingwhen the salivary glands are involved. The oral dryness of SS leads to difficulty swallowing dry food, unless they are washed down with liquids. Patients with xerostomiahave a delayed swallow; some patients will have dysphagia forsolids and some may take longer to complete a meal. Abnormal esophageal motility consists of absent or decreased contractilityin the upper third of the esophagus, while decreased esophageal peristalsis can be seen in the distal esophagus.
SS occurs in a primary (glandular) form not associated with other diseases (i.e. KCS and xerostomia only) and in a secondary (extraglandular) form (i.e. KCS, xerostomia, and an autoimmune disease). Most commonly, this autoimmune disease is RA. The disease is usually benign; however, in a few with systemic involvement, there is increased incidence of non-Hodgkin’s lymphoma.
Wallenberg or lateral medullary or posterior inferior cerebellar artery (PICA) syndrome— It is a type of brainstem (medulla and cerebellum)
stroke manifested by imbalance, vertigo, difficulty swallowing, hoarseness of voice, and sensory disturbance. Brainstem stroke is associated with ahigher frequency of dysphagia.
This is because the swallowingresponse control center resides primarily in the medulla. These patients often exhibit dysphagia at 1–2 weeks after stroke,but can make improvements up to week 3. Common problemsinclude delayed or absent swallow response, unilateral pharyngeal paresis, and upper esophageal sphincter dysfunction. VSFS demonstratesa lack of upper esophageal sphincter (UES) opening during swallow in patients with lateralmedullary syndrome, leading to varying degrees of aspiration.
Zenker’s diverticulum—It is also called a pharyn-goesophageal diverticulum—an outpouching of posterior hypopharyngeal wall at an area of potential weakness in the inferior pharyngeal constrictor muscle referred to as the Killian dehiscence (usually at the C5/6 level), seen most commonly in the elderly, in the seventh or eighth decade of life. The most common presenting feature is upper esophageal dysphagia; other common symptoms include halitosis, regurgitation of undigested food, noisy swallowing, and aspiration. Hoarseness can be present when the diverticulum is large enough to compress the recurrent laryngeal nerve. Some patients also report excessive salivation and the sensation of a mass within the throat. A very large diverticulum can produce an external neck mass, usually on the left side. Weight loss and recurrent pulmonary infections occur in approximately one-third of patients. There is an association with hiatal hernia, gastroduodenal ulcer, midesophageal diverticulum, esophageal spasm, and achalasia.
Fluoroscopic barium esophagography is the mainstay of diagnosis of Zenker diverticulum.
Care must be taken in performing endoscopy in patients with known Zenker diverticulum, as passage of the endoscope into the diverticulum carries some risk of perforation.
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REFERENCES
1. Deary, et al. Convert psychiatric disturbances in patients with globus pharyngis; Br J Med Psychol
1. Deary, et al. Convert psychiatric disturbances in patients with globus pharyngis; Br J Med Psychol