A Serious Diagnosis With a Common Presentation: Case Quiz

A 33-year-old man presents to the emergency department 2 hours after eating complaining of "food stuck in my esophagus." His symptoms began suddenly while eating chicken. After a period of coughing, he is now unable to swallow saliva, which he has been spitting up since the onset of symptoms. He claims to have had problems with swallowing solid food since childhood but has not required a medical evaluation. For the last few months, his symptoms have worsened. His oropharynx reveals no foreign bodies or abrasions. Image courtesy of Wikimedia Commons.

Which of the following statements about dysphagia is correct?

A. Dysphagia is difficulty swallowing

B. Dysphagia is distinguished from odynophagia, which is painful swallowing

C. Dysphagia results from impeded solids, liquids, or both

D. Types of dysphagia are oropharyngeal dysphagia and esophageal dysphagia

E. All of the above

Answer: E. All of the above

A routine dysphagia checklist is performed (shown above). The patient's symptoms over the last few months seem to be intermittent, and they are rectified by chewing carefully and drinking water after swallowing solids. He reports a remote history of esophageal reflux unresponsive to esomeprazole. He also reports current sinusitis and seasonal allergies, for which he is taking antihistamines. He denies any history of oral thrush, odynophagia, smoking, heavy alcohol use, or any illicit drug use. He denies having any weakness, numbness, diplopia, or other visual changes.

On physical examination, his oral temperature is 98.6°F (37.0°C). His pulse is regular at 60 beats/min. His blood pressure is 120/70 mm Hg. His respiratory rate is 12 breaths/min. The patient appears very uncomfortable and is clutching his chest. Although he is spitting up saliva, he is not drooling, and no stridor, cyanosis, or pallor are noted. An examination of his head and neck reveals no palpable masses or cervical lymphadenopathy. His lungs are clear to auscultation, and the patient has a normal respiratory effort. Cardiac auscultation reveals normal S1 and S2 heart sounds without any murmurs. The ECG shown above reveals a sinus rhythm without signs of acute ischemia. His abdomen is soft, nontender, and has normal bowel sounds. No skin is rash noted.

The laboratory analysis of the patient, including a complete blood cell count, basic metabolic panel, and thyroid-stimulating hormone, are normal. Plain chest radiographs (shown) are unremarkable, with no radiolucent objects or extravasations of air seen.

Which of the following studies is most helpful in evaluating a patient for a structural cause of dysphagia?

A. Soft-tissue CT scan of the neck

B. Lateral soft-tissue neck radiograph

C. Fiber-optic nasopharyngoscopy

D. Barium esophagram (barium swallow)

E. CT scan of the chest

Answer: D. Barium esophagram (barium swallow)

A barium swallow (shown) will help evaluate for structural causes of dysphagia, such as narrowing or inflammation of the esophagus, swallowing disorders, hiatal hernias, esophageal varices, esophageal ulcers, tumors, and polyps. Patients with dysphagia are usually given a 13-mm barium tablet to swallow at the end of the examination. The 13-mm rule indicates that a barium tablet stuck in the esophagus (shown) often signifies solid food dysphagia.^[1]

The list of differential diagnoses for dysphagia is broad and covers both motility disorders and mechanical obstructions. Image courtesy of The Dave Project.

Which of the following possible causes of mechanical obstruction is shown in this barium swallow image?

A. Schatzki ring

B. Peptic stricture

C. Pill-induced esophagitis/stricture

D. Extrinsic compression (eg, bone spurs)

E. Cancer (eg, esophageal)

Answer: A. Schatzki ring

A Schatzki ring (shown during this endoscopy) is quite common and may be found in as many as 15% of all patients undergoing barium swallow studies. A Schatzki ring is a diaphragm-like, thin mucosal ring usually located at the squamocolumnar junction. Symptoms are dependent on the degree of narrowing. A Schatzki ring may have a classic appearance on barium studies, but esophagogastroduodenoscopy is the definitive diagnostic modality.^[2] Image courtesy of The Dave Project.

Barrett esophagus is the replacement of esophageal squamous epithelium with metaplastic columnar epithelium, usually secondary to gastroesophageal reflux disease. These changes place patients at increased risk for esophageal dysplasia and adenocarcinoma. Patients classically report reflux symptoms over time that may eventually include progress reflux. The above image shows patches of esophagus demonstrating intestinal metaplasia (salmon-pink colored areas; the white area is squamous epithelium). Definitive diagnosis is made via endoscopy and biopsy.

Boerhaave syndrome is a transmural perforation of the esophagus, classically occurring after a prolonged period of retching in an alcoholic. One third of cases may present in an atypical fashion, and the mortality rate is 35% even with treatment. Diagnosis is often suggested by mediastinal air on chest radiography and is confirmed with chest CT. A water-soluble contrast esophagram may help localize the defect and extent of extravasation but should not delay definitive surgical treatment.

Esophageal motility disorders, including achalasia, diffuse esophageal spasm, nutcracker esophagus, and hypertensive lower esophageal spasm, may all present with varying degrees of solid and liquid dysphagia. Diagnosis is typically made by a combination of an elevated pressure reading on manometry and stereotypical morphologic changes on a barium esophagram. The classic "bird's beak” deformity of the distal esophagus in achalasia is demonstrated here.

Foreign-body or large food bolus ingestion may cause acute dysphagia. The primary locations of obstruction are at the thoracic inlet, in the mid-esophagus at the level of the aortic arch, and at the diaphragmatic hiatus. Chest radiographs may reveal radiopaque foreign bodies, as shown in this child who swallowed a coin. Definitive diagnosis is obtained via direct endoscopy. A barium swallow study should be avoided because it may impair endoscopic visualization.

The patient was initially treated with IV fluids and 1 mL of glucagon IV followed by a trial of effervescent granule packets of sodium bicarbonate, citric acid, and simethicone. However, this treatment was unsuccessful. The patient was scheduled for an emergent esophagogastroduodenoscopy and the esophageal food obstruction was identified and removed; further assessment revealed circumferential contractions (blue arrows). Additionally, the mucosa was thin and fragile and sheared easily on dilation following the gentle passage of a 14-mm savory dilator, resulting in the so-called “tissue-paper sign."

What condition is revealed on the endoscopic image?

A. Esophageal varices

B. Barrett esophagus

C. Eosinophilic esophagitis

D. Gastroesophageal reflux disease

E. Chemical esophagitis

Answer: C. Eosinophilic esophagitis

Eosinophilic esophagitis is an inflammatory condition of the esophagus characterized by eosinophilic infiltration, usually presenting as dysphagia.^[3] It classically affects children; the adult form has only recently gained recognition as a distinct entity. Often, the patient's history of dysphagia dates to childhood or adolescence. On endoscopy, concentric rings are seen throughout the esophagus, termed trachealization (shown). Image courtesy of Wikimedia Commons.

Which of the following endoscopic abnormalities is NOT typically associated with eosinophilic esophagitis?

A. Strictures

B. Narrowed lumen of the esophagus

C. Mucosal furrowing

D. Multiple esophageal ulcerations

E. Esophageal rings

Answer: D. Multiple esophageal ulcerations

Multiple esophageal ulcerations are not part of the common endoscopic findings during evaluation of patients with eosinophilic esophagitis. However, esophageal strictures, narrowed lumen, and rings (including Schatzki rings) have been described.^[4] Other subtle findings include longitudinal mucosal furrowing, white spikes, and easy tearing of the mucosa. Eosinophilic microabscesses may be seen (shown); these appear as white papules resembling Candida esophagitis and are more common in the pediatric population.

In a study by Desai and colleagues,^[5] eosinophilic esophagitis was noted to be the most common cause of food impaction in a primary gastroenterology practice. The study also emphasized that for young adults, eosinophilic esophagitis is the most likely underlying cause of food impaction. Of note, the authors also reported that eosinophilic esophagitis may have an identical appearance to a Schatzki ring, as seen in this case. In some patients, eosinophilic esophagitis may occur in association with eosinophilic gastroenteritis.^[5] A barium swallow (shown) demonstrates these findings of concentric ring-like contractions in the proximal half of the esophagus. Image courtesy of The Dave Project.

Is the following statement TRUE or FALSE?: Eosinophilic esophagitis is more common in men.

Answer: True

Adult patients with eosinophilic esophagitis are usually men (male-to-female ratio, 3:1) and are most commonly in the third or fourth decade of life. Most adult patients do not respond to antisecretory therapy but may have objective evidence of reflux on a 24-hour pH study. Manometry studies are usually normal, but 40% of cases have uncoordinated contractions. About 50% of patients give a history of allergies (food, atopic dermatitis, allergic rhinitis), and 31% have peripheral blood eosinophilia. Serum immunoglobulin E levels are increased in about 55% of patients. An endoscopy image demonstrates longitudinal tearing, termed "crepe-paper esophagus."

What is the test of choice for diagnosing eosinophilic esophagitis?

A. Transesophageal ultrasonography

B. Plain radiographs of the chest

C. MRI

D. Endoscopy

Answer: D. Endoscopy

Histology is critical to the diagnosis of eosinophilic esophagitis, and endoscopy (with biopsies taken from the upper and lower esophagus) is the test of choice for a definitive diagnosis. There is significant variability in the histopathologic diagnostic criteria for eosinophilic esophagitis. Most studies suggest that findings of > 20 eosinophils per high-power field (hpf) in a single field or > 15 eosinophils per hpf in 2 fields are diagnostic of eosinophilic esophagitis. Esophageal reflux may produce an eosinophilic infiltration (< 10 eosinophils per hpf) that is usually limited to the distal esophagus. The above image shows a very high magnification micrograph of eosinophilic esophagitis with a substantial number of eosinophils (arrow). Image courtesy of Wikimedia Commons.

The cause of eosinophilic esophagitis is poorly understood. Most studies report a high prevalence of allergies in family members. It is not clear whether eosinophilic esophagitis has a purely genetic base or whether this condition occurs in genetically predisposed individuals with an environmental trigger component. In many cases, the disease manifestations are intermittent in nature. Optimal treatment for eosinophilic esophagitis has not been clearly defined, and treatment recommendations are limited mostly to clinical experiences, case series, and small controlled trials.

After specific food allergies are identified, pediatric or adolescent patients can be started on a targeted elimination diet, an elemental diet, or a 6-food elimination diet for 6 weeks (see table). Emerging data in adults also suggest that a 6-food elimination diet can improve symptoms, improve esophageal eosinophilia, and help to identify causative foods. Systemic glucocorticoids are effective in the majority of patients, with improvement usually occurring within 7-10 days. Most patients, however, relapse upon withdrawal of glucocorticoids. A recent preliminary controlled trial suggested that topical glucocorticoids can be as effective as oral glucocorticoids and may produce fewer side effects. However, the efficacy of topical glucocorticoids has not been truly verified.^[6]

Topical glucocorticoid therapy utilizes a fluticasone metered-dose inhaler without a spacer. Patients are instructed not to inhale but rather to swallow when the actuation is delivered. Patients should not eat or drink for 30 minutes following administration. A small amount of water may be sipped and swallowed immediately after actuation to help carry the fluticasone from the oropharynx to the esophagus. Additional pharmacologic therapies are shown above.

The patient was subsequently referred to an allergist for skin prick and/or patch testing for food and other environmental allergens. He was then started on a course of oral fluticasone. The patient was doing well 6 weeks after the diagnosis, with minimal dysphagia.

Note that some patients with eosinophilic esophagitis may present with concentric rings with a white papular exudate in the proximal esophagus (shown).

More Slideshows

• Case Quiz: A Deadly Cause of Vomiting in a Newborn
• Critical Findings on Plain Abdominal Radiographs
• Easy-to-Miss Findings on Chest Radiographs
• Foreign Body Sensation
• Foreign Objects Found in Patients
• What to Look for on a Chest X-Ray

For more information, see the following resources:

• Medscape Reference: Aspiration Syndromes
• Medscape Reference: Eosinophilia
• Medscape Reference: Esophagitis
• Medscape Reference: Gastroesophageal Reflux Disease
• Medscape Reference: Pediatric Esophagitis

Contributor Information

Authors
Juan Carlos Munoz, MD
Clinical Assistant Professor of Medicine
Department of Gastroenterology
University of Florida, Jacksonville

Disclosure: Juan Munoz, MD, has disclosed no relevant financial information.

Carmela Monteiro, MD
Associate Professor
Department of Pathology and Laboratory Medicine
University of Florida College of Medicine
Jacksonville, Jacksonville, Florida

Disclosure: Carmela Monteiro, MD, has disclosed no relevant financial information.

Authors (continued)
Ivan E. Rascon-Aguilar, MD
Fellow, Department of Internal Medicine
Division of Gastroenterology
University of Florida College of Medicine
Jacksonville, Florida

Disclosure: Ivan E. Rascon-Aguilar, MD, has disclosed no relevant financial information.

Editors
Mark P. Brady, PA-C
Chief Physician Assistant
Department of Emergency Medicine
Cambridge Hospital
Cambridge Health Alliance
Cambridge, Massachusetts

Disclosure: Mark P. Brady, PA-C, has disclosed no relevant financial relationships.

Editors (continued)
Catherine A. Lynch, MD
Assistant Professor
Department of Surgery, Division of Emergency Medicine
Duke University Medical Center
Faculty, Duke Global Health Institute
Durham, North Carolina

Disclosure: Catherine A. Lynch, MD, has disclosed no relevant financial relationships.

Lars Grimm, MD, MHS
House Staff
Department of Internal Medicine
Duke University Medical Center
Durham, North Carolina

Disclosure: Lars Grimm, MD, MHS, has disclosed no relevant financial relationships.

Editors (continued)
Tomasz Guzowski, MD, FRCPC
Internal Medicine and Gastroenterology
Stanton Territorial Hospital
Assistant Professor of Medicine
University of Alberta
Yellowknife, Northwest Territories, Canada

Disclosure: Tomasz Guzowski, MD, FRCPC, has disclosed no relevant financial relationships.