DYSPHAGIA
Dysphagia is difficulty moving food or liquid through the mouth, pharynx, and esophagus. The pt senses swallowed material sticking along the path. Odynophagia is pain on swallowing. Globus pharyngeus is the sensation of a lump lodged in the throat, with swallowing unaffected.
PATHOPHYSIOLOGY
Dysphagia is caused by two main mechanisms: mechanical obstruction or motor dysfunction. Mechanical causes of dysphagia can be luminal (e.g., large food bolus, foreign body), intrinsic to the esophagus (e.g., inflammation, webs and rings, strictures, tumors), or extrinsic to the esophagus (e.g., cervical spondylitis, enlarged thyroid or mediastinal mass, vascular compression). The motor function abnormalities that cause dysphagia may be related to defects in initiating the swallowing reflex (e.g., tongue paralysis, lack of saliva, lesions affecting sensory components of cranial nerves X and XI), disorders of the pharyngeal and esophageal striated muscle (e.g., muscle disorders such as polymyositis and dermatomyositis, neurologic lesions such as myasthenia gravis, polio, or amyotrophic lateral sclerosis), and disorders of the esophageal smooth muscle (e.g., achalasia, scleroderma, myotonic dystrophy).
APPROACH TO THE PATIENT Dysphagia
History can provide a presumptive diagnosis in about 80% of pts. Difficulty only with solids implies mechanical dysphagia. Difficulty with both solids and liquids may occur late in the course of mechanical dysphagia but is an early sign of motor dysphagia. Pts can sometimes pinpoint the site of food sticking. Weight loss out of proportion to the degree of dysphagia may be a sign of underlying malignancy. Hoarseness may be related to involvement of the larynx in the primary disease process (e.g., neuromuscular disorders), neoplastic disruption of the recurrent laryngeal nerve, or laryngitis from gastroesophageal reflux.
Physical exam may reveal signs of skeletal muscle, neurologic, or oropharyngeal diseases. Neck exam can reveal masses impinging on the esophagus. Skin changes might suggest the systemic nature of the underlying disease (e.g., scleroderma).
Dysphagia is nearly always a symptom of organic disease rather than a functional complaint. If oropharyngeal dysphagia is suspected, video-fluoroscopy of swallowing may be diagnostic. Mechanical dysphagia can be evaluated by barium swallow and esophagogastroscopy with endoscopic biopsy. Barium swallow and esophageal motility studies can show the presence of motor dysphagia. An algorithm outlining an approach to the pt with dysphagia is shown in Fig. 45-1.
FIGURE 45-1 Approach to the pt with dysphagia. Etiologies in bold print are the most common. ENT, ear, nose, and throat; GERD, gastroesophageal reflux disease.
OROPHARYNGEAL DYSPHAGIA
Pt has difficulty initiating the swallow; food sticks at the level of the suprasternal notch; nasopharyngeal regurgitation and aspiration may be present.
Causes include the following: for solids only, carcinoma, aberrant vessel, congenital or acquired web (Plummer-Vinson syndrome in iron deficiency), cervical osteophyte; for solids and liquids, cricopharyngeal bar (e.g., hypertensive or hypotensive upper esophageal sphincter), Zenker’s diverticulum (outpouching in the posterior midline at the intersection of the pharynx and the cricopharyngeus muscle), myasthenia gravis, glucocorticoid myopathy, hyperthyroidism, hypothyroidism, myotonic dystrophy, amyotrophic lateral sclerosis, multiple sclerosis, Parkinson’s disease, stroke, bulbar palsy, pseudobulbar palsy.
ESOPHAGEAL DYSPHAGIA
Food sticks in the mid or lower sternal area; can be associated with regurgitation, aspiration, odynophagia. Causes include the following: for solids only, lower esophageal ring (Schatzki’s ring—symptoms are usually intermittent), peptic stricture (heartburn accompanies this), carcinoma, lye stricture; for solids and liquids, diffuse esophageal spasm (occurs with chest pain and is intermittent), scleroderma (progressive and occurs with heartburn), achalasia (progressive and occurs without heartburn).
NONCARDIAC CHEST PAIN
Of pts presenting with chest pain, 30% have an esophageal source rather than angina. History and physical exam often cannot distinguish cardiac from noncardiac pain. Exclude cardiac disease first. Causes include the following: gastroesophageal reflux disease, esophageal motility disorders, peptic ulcer disease, gallstones, psychiatric disease (anxiety, panic attacks, depression).
EVALUATION
Consider a trial of antireflux therapy (omeprazole); if no response, 24-h ambulatory luminal pH monitoring; if negative, esophageal manometry may show motor disorder. Trial of imipramine, 50 mg PO qhs, may be worthwhile. Consider psychiatric evaluation in selected cases.
ESOPHAGEAL MOTILITY DISORDERS
Pts may have a spectrum of manometric findings ranging from nonspecific abnormalities to defined clinical entities.
ACHALASIA
Motor obstruction caused by hypertensive lower esophageal sphincter (LES), incomplete relaxation of LES, or loss of peristalsis in smooth-muscle portion of esophagus. Causes include the following: primary (idiopathic) or secondary due to Chagas’ disease, lymphoma, carcinoma, chronic idiopathic intestinal pseudoobstruction, ischemia, neurotropic viruses, drugs, toxins, radiation therapy, postvagotomy.
EVALUATION
Chest x-ray shows absence of gastric air bubble. Barium swallow shows dilated esophagus with distal beaklike narrowing and air-fluid level. Endoscopy is done to rule out cancer, particularly in persons >50 years. Manometry shows normal or elevated LES pressure, decreased LES relaxation, absent peristalsis.
TREATMENT Achalasia
Pneumatic balloon dilatation is effective in 85%, with 3–5% risk of perforation or bleeding. Injection of botulinum toxin at endoscopy to relax LES is safe and effective, but effects last only ~12 months. Myotomy of LES (Heller procedure) is effective, but 10–30% of pts develop gastroesophageal reflux. Nifedipine, 10–20 mg, or isosorbide dinitrate, 5–10 mg SL ac, may avert need for dilatation or surgery. Sildenafil may also augment swallow-induced relaxation of the LES.
SPASTIC DISORDERS
Diffuse esophageal spasm involves multiple spontaneous and swallow-induced contractions of the esophageal body that are of simultaneous onset and long duration and are recurrent. Causes include the following: primary (idiopathic) or secondary due to gastroesophageal reflux disease, emotional stress, diabetes, alcoholism, neuropathy, radiation therapy, ischemia, or collagen vascular disease.
An important variant is nutcracker esophagus: high-amplitude (>180 mmHg) peristaltic contractions; particularly associated with chest pain or dysphagia, but correlation between symptoms and manometry is inconsistent. Condition may resolve over time or evolve into diffuse spasm; associated with increased frequency of depression, anxiety, and somatization.
EVALUATION
Barium swallow shows corkscrew esophagus, pseudodiverticula, and diffuse spasm. Manometry shows spasm with multiple simultaneous esophageal contractions of high amplitude and long duration. In nutcracker esophagus, the contractions are peristaltic and of high amplitude. If heart disease has been ruled out, edrophonium, ergonovine, or bethanechol can be used to provoke spasm.
TREATMENT Spastic Disorders
Anticholinergics are usually of limited value; nitrates (isosorbide dinitrate, 5–10 mg PO ac) and calcium antagonists (nifedipine, 10–20 mg PO ac) are more effective. Those refractory to medical management may benefit from balloon dilatation. Rare pts require surgical intervention: longitudinal myotomy of esophageal circular muscle. Treatment of concomitant depression or other psychological disturbance may help.
SCLERODERMA
Atrophy of the esophageal smooth muscle and fibrosis can make the esophagus aperistaltic and lead to an incompetent LES with attendant reflux esophagitis and stricture. Treatment of gastroesophageal reflux disease is discussed in Chap. 44.
ESOPHAGEAL INFLAMMATION
VIRAL ESOPHAGITIS
Herpesviruses I and II, varicella-zoster virus, and cytomegalovirus (CMV) can all cause esophagitis; particularly common in immunocompromised pts (e.g., AIDS). Odynophagia, dysphagia, fever, and bleeding are symptoms and signs. Diagnosis is made by endoscopy with biopsy, brush cytology, and culture.
TREATMENT Viral Esophagitis
Disease is usually self-limited in the immunocompetent person; viscous lidocaine can relieve pain; in prolonged cases and in immunocompromised hosts, herpes and varicella esophagitis are treated with acyclovir, 5–10 mg/kg IV q8h for 10–14 d, then 200–400 mg PO 5 times a day for a week or valacyclovir 1 g PO tid for 7 days. CMV is treated with ganciclovir, 5 mg/kg IV q12h, until healing occurs, which may take weeks. Oral valganciclovir (900 mg bid) is an effective alternative to parenteral treatment. In nonresponders, foscarnet, 90 mg/kg IV q12h for 21 days, may be effective.
CANDIDA ESOPHAGITIS
In immunocompromised hosts, or those with malignancy, diabetes, hypoparathyroidism, hemoglobinopathy, systemic lupus erythematosus, corrosive esophageal injury, candidal esophageal infection may present with odynophagia, dysphagia, and oral thrush (50%). Diagnosis is made on endoscopy by identifying yellow-white plaques or nodules on friable red mucosa. Characteristic hyphae are seen on KOH stain. In pts with AIDS, the development of symptoms may prompt an empirical therapeutic trial.
TREATMENT Candida Esophagitis
Oral nystatin (100,000 U/mL), 5 mL q6h, or clotrimazole, 10-mg tablet sucked q6h, is effective. In immunocompromised hosts, fluconazole, 200 mg PO on day 1 followed by 100 mg daily for 1–2 weeks, is treatment of choice; alternatives include itraconazole, 200 mg PO bid, or ketoconazole, 200–400 mg PO daily; long-term maintenance therapy is often required. Poorly responsive pts may respond to higher doses of fluconazole (400 mg/d) or to amphotericin, 10–15 mg IV q6h for a total dose of 300–500 mg.
PILL-RELATED ESOPHAGITIS
Doxycycline, tetracycline, aspirin, nonsteroidal anti-inflammatory drugs, KCl, quinidine, ferrous sulfate, clindamycin, alprenolol, and alendronate can induce local inflammation in the esophagus. Predisposing factors include recumbency after swallowing pills with small sips of water and anatomic factors impinging on the esophagus and slowing transit.
TREATMENT Pill-Related Esophagitis
Withdraw offending drug, use antacids, and dilate any resulting stricture.
EOSINOPHILIC ESOPHAGITIS
Mucosal inflammation with eosinophils with submucosal fibrosis can be seen especially in pts with food allergies. This diagnosis relies on the presence of symptoms of esophagitis with the appropriate findings on esophageal biopsy. Eotaxin 3, an eosinophil chemokine, has been implicated in its etiology. IL-5 and TARC (thymus and activation-related chemokine) levels may be elevated. Treatment involves a 12-week course of swallowed fluticasone (440 μg bid) using a metered-dose inhaler.
OTHER CAUSES OF ESOPHAGITIS IN AIDS
Mycobacteria, Cryptosporidium, Pneumocystis, idiopathic esophageal ulcers, and giant ulcers (possible cytopathic effect of HIV) can occur. Ulcers may respond to systemic glucocorticoids.
For a more detailed discussion, see Hirano I, Kahrilas PJ: Dysphagia, Chap. 38, p. 297; and Kahrilas PJ, Hirano I: Diseases of the Esophagus, Chap. 292, p. 2427, in HPIM-18.