Transfer dysphagia (also referred to as oropharyngeal dysphagia) usually occurs as a consequence of neurologic or neuromuscular disease and presents as choking or difficulty in initiating swallowing. The patient reports the symptom as beginning immediately on trying to swallow. In many instances, other neurologic symptoms dominate the clinical picture, but at times, difficulty in swallowing is the major complaint, with aspiration and regurgitation of fluid into the nose. The problem is particularly common among the very old. Cortical and brainstem lesions resulting from stroke, tumor, and degenerative disease are important causes. In addition, medications with central effects (e.g., benzodiazepines, L-dopa, phenothiazines) may blunt the swallowing mechanism. Unlike esophageal disease, oropharyngeal dysphagia is accurately localized to the suprasternal area. Patients with neuromuscular etiologies report more difficulty in swallowing liquids than solids, nasal regurgitation, coughing, and aspiration. Patients with anatomic narrowing and mechanical obstruction of the pharynx or upper esophagus have more difficulty with solids than liquids.

Achalasia is a common cause of motor dysphagia and a slowly progressive chronic motility disorder. The pathologic hallmark is a loss of cells in the myenteric ganglia. As a consequence of a loss of smooth muscle ganglion cells, the esophagus functions poorly, exhibits episodes of aperistalsis, and demonstrates exquisite sensitivity to gastrin and cholinergic agents. The gastroesophageal sphincter fails to relax properly, leading to functional obstruction at the gastroesophageal junction. In addition, a loss of peristaltic activity occurs in the distal esophagus. Dysphagia, regurgitation of undigested food, and substernal chest pain ensue. The resting pressure at the lower esophageal sphincter (LES) rises, and barium study shows an absence of
peristalsis and delay in esophageal emptying. Paradoxically, vigorous nonpropulsive (tertiary) esophageal contractions resulting in chest pain may be observed early in the disease in young patients. Swallowing liquids and solids is equally difficult, yet by eating slowly and drinking small amounts, the patient may be able to consume a full meal. Pain is reported by 70% to 80% of patients, especially if they eat or drink rapidly, but pain is not an invariable accompaniment, and only 2% of patients with chest pain caused by esophageal disease have achalasia. Very cold liquids or emotion may provoke symptoms. Patients find that repeated swallowing or performing a rapid Valsalva maneuver can help to pass material into the stomach. Regurgitation is common and can be provoked by changes in position or physical exercise; pulmonary aspiration sometimes results. Patients may have foul breath because of retained esophageal material. Squamous cell carcinoma of the esophagus is sometimes a complication of achalasia; it occurs in 5% to 10% of patients.

Carcinoma-induced achalasia is seen with tumors at the gastroesophageal junction. Adenocarcinoma of the stomach is the most common of these neoplasms. The mechanism by which tumor induces achalasia is often unclear, but sometimes is associated with neural invasion; manometric findings are identical to those of primary achalasia. Patients are typically older than the age of 50 years and complain of marked weight loss and symptoms of dysphagia of less than 1 year’s duration.

Scleroderma can impair neuromuscular function and result in a decrease in LES tone in addition to a lack of propulsive motor activity. Reflux is more of a problem than is dysphagia (helping to distinguish scleroderma from other motility disorders), but as many as 20% of patients may have some difficulty in swallowing. About 75% of patients with scleroderma have esophageal involvement as part of the CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia).

Diffuse esophageal spasm is characterized clinically by nonprogressive dysphagia and substernal chest pain that may mimic angina (see later discussion and Chapter 20). Radiologically and manometrically, patients manifest nonpropulsive simultaneous contractions (tertiary contractions) throughout the entire esophagus (especially the distal portion) in more than 10% of wet swallows. Such contractions are also observed in normal persons under conditions of emotional stress and dry swallows, which leads some to view this condition as little more than a transient abnormality in motor function induced by stress. However, as noted earlier, the condition may also be an early manifestation of neuromuscular disease that progresses to achalasia. Unlike achalasia, diffuse esophageal spasm is intermixed with periods of normal peristaltic activity. Dysphagia is noted with both liquids and solids. Diffuse esophageal spasm accounts for about 10% of cases of noncardiac chest pain caused by abnormal esophageal motility. Some asymptomatic patients manifest the radiologic and manometric criteria for esophageal spasm but rarely experience discomfort.

This picturesque radiologic description is applied to the finding of very high-amplitude contractions in the distal esophagus. These contractions can be sustained and visualized on barium swallow, hence the “nutcracker” designation. Some view the condition as a severe variant of diffuse esophageal spasm, where the resultant pressures exceed the mean by more than two standard deviations. These peristaltic contractions are not only of exceptionally high amplitude but also of long duration. There are no simultaneous contractions elsewhere in the esophagus, as there are in diffuse esophageal spasm, and only occasionally does impairment of esophageal function lead to dysphagia. The principal symptom is chest pain (see later discussion), with nutcracker esophagus accounting for about half of all cases of noncardiac chest pain associated with an esophageal motility disturbance. In many instances, reflux precipitates the contractile abnormalities and may contribute to the chest pain. At issue is whether the condition might be considered a component or consequence of gastroesophageal reflux disease (GERD). Achalasia develops in about 3% to 5% of patients, and degenerative changes are noted in ganglia and nerves, suggesting to some a possible link to achalasia. Supersensitivity to gastrin and cholinergic agents can be demonstrated.

Hypertensive LES is characterized by increased resting LES pressure but normal relaxation and peristalsis, with no impediment to bolus passage. About half of the patients have highamplitude contractions, consistent with nutcracker esophagus.

Nonspecific esophageal motility disorder designates persons who manifest abnormal esophageal manometry results but fail to meet specific diagnostic criteria for one of the preceding conditions. Contractions may be nontransmitted, retrograde, of high or low amplitude, prolonged, spontaneous, or even retrograde. In addition, the LES may fail to relax.

Mechanical obstruction differs clinically from motor dysfunction, in that the patient has more difficulty with solids than with liquids. The duration of symptoms is shorter (<1 year) for patients with malignancy than it is for those with benign causes of obstruction; progression is often rapid. Most patients with tumor are older than the age of 50 years and report marked weight loss. The location of discomfort does not necessarily correlate with the site of obstruction because the pain may be referred. Spontaneous pain is not a common feature of neoplasm involving the esophagus. Patients with stricture caused by severe esophagitis usually have a long history of reflux.

Inflammatory lesions of the pharynx or esophagus may cause odynophagia (pain on swallowing). Esophageal motility is not disturbed, but swallowing is made difficult by the pain. Even saliva may be irritating. Radiation therapy, tablet ingestion, malignancy, and infection are important causes of this severe esophageal irritation. Tetracycline, quinidine, potassium tablets, nonsteroidal anti-inflammatory drugs, alendronate, and iron preparations have been implicated. The elderly are at greatest risk because they are likely to consume more tablets with less water, and saliva production decreases with age. The discomfort is often associated with ingestion of the tablets and generally decreases during a period of a few days.

Infectious esophagitis caused by Candida, herpes simplex virus, or cytomegalovirus is being increasingly recognized in immunocompromised patients, including those with diabetes and HIV, especially with prolonged use of broad-spectrum antibiotics. Onset may be rapid and accompanied by fever, chills, nausea, vomiting, and epigastric pain. Viral or fungal esophagitis rarely occurs in immunologically intact persons; when it does, it is usually short-lived and self-limited.

Sometimes, globus hystericus, a condition seen in anxiety disorders, is confused with dysphagia. The patient complains of a constant “lump in the throat” and has a perception of obstruction, although he or she has no actual difficulty in swallowing food. An involuntary tightening of the cricopharyngeal muscle has been observed in some patients with this condition and may account for the symptoms. Symptoms are unrelated to swallowing, and esophageal function is normal.