Uvular Edema, Acute

CHAPTER 60

Uvular Edema, Acute

Presentation

The patient complains of a foreign body sensation or a lump or fullness in the throat, possibly associated with a slightly muffled voice and gagging. The patient may be worried after looking in the mirror and seeing a large swelling in his throat. On examination of the throat, the uvula is boggy, swollen, pale, and somewhat translucent and gelatinous appearing (uvular hydrops). If greatly enlarged, the uvula might rest on the tongue and move in and out with respiration. There should be no associated rash or pruritus, soreness, fever, dyspnea, or other areas of edematous involvement, such as the tongue, sublingual region, soft palate, and tonsils.

What To Do:

Because of the known association of uvular edema with hypopharyngeal edema, watch for signs of airway compromise. If the patient complains of respiratory difficulty, breathes with stridor, or has significant voice change or hoarseness, prepare for treatment with IV lines, intubation, and cricothyrotomy equipment at the bedside, and obtain a portable lateral soft tissue neck radiographic examination to rule out epiglottic swelling. Begin medical treatment immediately with signs of impending airway compromise.

If there is no acute respiratory difficulty, ask the patient about precipitating events. Consider foods, drugs, physical agents, inhalants, insect bites, and, rarely, hereditary angioedema.

When fever, sore throat, and pharyngeal injection are present, swab the throat for a rapid streptococcal screen. If the streptococcal screen is negative, administer an antibiotic effective against Haemophilus influenzae, such as clarithromycin (Biaxin), amoxicillin plus clavulanate (Augmentin), or trimethoprim plus sulfamethoxazole (Bactrim). With a positive streptococcal screen, treat acute streptococcal pharyngitis as described in Chapter 38.

If the presentation is confusing, it is reasonable to obtain a complete blood count with a manual differential to demonstrate eosinophilia, which would support the possibility of an allergic reaction, or to demonstrate a high leukocyte count with increased granulocytes and bands, which would support a bacterial infection.

If there is general pruritus, urticaria, flushing, or facial edema, and an allergic reaction is suspected, the patient should initially receive parenteral H₁– and H₂-blocking antihistamines, such as hydroxyzine (Vistaril), 50 to 100 mg IM, or diphenhydramine (Benadryl), 25 to 50 mg IV, along with cimetidine (Tagamet), 300 mg IV or PO, or ranitidine (Zantac), 50 mg IV or 150 mg PO or famotidine (Pepcid), 20 mg IV or PO.

For more severe cases, give repeated doses of epinephrine, 0.3 mL of 1:1000 SC every 20 minutes for up to three doses. Nebulized isomeric or racemic epinephrine or albuterol is also effective. Topical application of a vasoconstrictor, such as cocaine gel, works very well.

Parenteral corticosteroids, such as methylprednisolone (Solu-Medrol), 125 mg IV, are also typically used.

When symptoms are mild and there is no clear cause (idiopathic isolated uvular edema), it is reasonable to provide the same treatment as one would give for an allergic reaction, although it is unclear whether or not this will bring about any diminution of the patient’s uvular swelling.

If the patient is on an angiotensin-converting enzyme (ACE) inhibitor, such as lisinopril (Prinivil, Zestril), benazepril (Lotensin), captopril (Capoten), cilazapril (Inhibace), enalapril (Vasotec), fosinopril (Monopril), benazepril (Lotensin), moexipril (Univasc), perindopril (Aceon), quinapril (Accupril), ramipril (Altace), or trandolapril (Mavik), use of that medication should be discontinued, and therapy with an alternative antihypertensive should begin. The patient will more likely have swelling of the tongue or lips and should be held for several hours of observation. Hospital admission should be considered if the swelling worsens. Airway compromise can occur rapidly and unpredictably when it is secondary to ACE inhibitor administration, and standard treatments are usually ineffective. Pronounced edema of the tongue and floor of the mouth are predictors of a need for airway intervention.

Eight patients with acute ACE inhibitor-induced angioedema, in a case series, were successfully treated with a single subcutaneous injection of icatibant. This small series showed complete relief of symptoms at 4.4 hours (standard deviation [SD] 0.8 hours), with the first symptom improvement occurring at a mean time of 50.6 minutes (SD 21 minutes). There were no adverse effects, except erythema occurring at the injection site. The external validity of these impressive results still need to be confirmed in a prospective, randomized, controlled clinical trial.

If the patient has a history of recurrent episodes of edema, and there is a family history of the same, consider ordering tests to determine the C4 complement level or the C1 esterase inhibitor level to screen for hereditary angioedema. In this condition, the edema often involves the uvula and soft palate together. This is a rare condition that also does not respond to standard therapy.

Uvular decompression may be useful in patients who are resistant to medical therapy or who have rapidly progressing symptoms and in whom there is concern about possible airway compromise. This procedure consists of grasping the uvula with forceps and either making several lacerations with a sterile needle or snipping the distal centimeter as a partial uvulectomy. Injecting the uvula with lidocaine 1% (Xylocaine) with epinephrine will provide anesthesia and may be therapeutic.

All patients should be observed for an adequate period of time to ensure that there is either improvement or no further increase in the swelling before being sent home. When discharged, the patient with an allergic or idiopathic cause for their edema should be given a 4- to 5-day supply of H₁ and H₂ blockers and steroids if required.

What Not To Do:

Do not perform a comprehensive and costly laboratory evaluation on every patient. Order only those specific tests that are clearly indicated and will provide results that can be followed up.

Do not obtain lateral soft tissue neck radiographs on patients with acute spontaneous uvular edema who are asymptomatic other than for their foreign body sensation.

Discussion

The uvula (Latin for “little grape”) is a small, conical, peduncular process hanging from the middle of the lower border of the soft palate. The soft palate is composed of muscle, connective tissue, and mucous membrane, and the bulk of the uvula consists of glandular tissue with diffuse muscle fibers interspersed throughout. During the acts of deglutition and phonation, the uvula and soft palate are directed upward, thereby walling off the nasal cavity from the pharynx. During swallowing, this prevents ingested substances from entering the nasal cavity.

Most patients who present acutely with isolated angioedema of the uvula have mild symptoms and a benign clinical course with an obscure cause. This idiopathic form of spontaneous swelling of the uvula may or may not be related to other forms of angioedema.

Angioedema, also known as angioneurotic edema and Quincke disease, is defined as a well-localized edematous condition that may variably involve the deeper skin layers, subcutaneous tissues, and mucosal surfaces of the upper respiratory and gastrointestinal tracts.

Immediate hypersensitivity type I reactions, seen with atopic states and specific allergen sensitivities, are the most common causes of angioedema. These reactions involve the interaction of an allergen with IgE antibodies bound to the surface of basophils or mastocytes. Physical agents, including cold, pressure, light, and vibration, or other processes that increase core temperature may also cause edema through the IgE pathway.

Hereditary angioedema, a genetic disorder of the complement system, is characterized by either an absence or a functional deficiency of C1 esterase inhibitor. This absence or deficiency allows unopposed activation of the first component of complement, with subsequent breakdown of its two substrates, the second (C2) and fourth (C4) components of the complement cascade. This process, in the presence of plasmin, generates a vasoactive kinin-like molecule that causes angioedema. Acquired C1 esterase inhibitor deficiency and other complement consumption states have been described in patients with malignancies and immune complex disorders, including serum sickness and vasculitides.

Other causes of angioedema include certain medications and diagnostic agents (e.g., opiates, D-tubocurarine, curare, radiocontrast materials) that have a direct degranulation effect on mast cells and basophils; substances such as aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), azo dyes, and benzoates that alter the metabolism of arachidonic acid, thus increasing vascular permeability; and ACE inhibitors, implicated presumably by promoting the production of bradykinin.

The known infectious causes of uvulitis include group A streptococci, Haemophilus influenzae, and Streptococcus pneumoniae. An associated cellulitis may contiguously involve the uvula and the tonsils, posterior pharynx, or epiglottis.

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