4.12 Uvular edema
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agk's Library of Common Simple Emergencies

Presentation
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A patient complains of a foreign body sensation 
or fullness in the throat, possibly associated 
with a muffled voice and gagging. Upon exam- 
ination of the throat, the uvula is swollen, 
pale, and somewhat translucent (uvular 
hydrops). If greatly enlarged, the uvula might 
rest on the tongue and move in and out with 
respiration. There might be an associated rash 
or a history of exposure to phsical stimuli, 
allergens, or a recurrent seasonal incidence.

What to do:
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- Because of the known association of uvular 
   with hypopharyngeal edema, watch for signs 
   of airway compromise. If a patient complains 
   of respiratory difficulty or breathes with 
   stridor, commence treatment with intravenous 
   lines and intubation and cricothyrotomy 
   equipment at the bedside, and a crosstable 
   lateral soft tissue neck x-ray to rule out 
   epiglottic swelling.
- If there is no acute respiratory difficulty, 
   ask about precipitating events. Consider 
   foods, drugs, physical agents, inhalants, 
   insect bites and hereditary angioedema.
- When fever, sore throat and pharyngeal 
   injection are present, culture the throat 
   with a rapid strep screen and give an 
   antibiotic that covers Haemophilus 
   influenzae (e.g., Biaxin, Augmentin, 
   Bactrim).
- It is reasonable to obtain a complete blood 
   count with a manual differential to 
   demonstrate eosinophilia to support the 
   possibility of an allergic reaction or a 
   high leukocyte count with increased granulo- 
   cytes and bands to support a bacterial 
   infection.
- Initially the patient should receive 
   parenteral H1 and H2 antihistamines like 
   hydroxazine 50-100mg im or diphenhydramine 
   25-50mg iv along with cimetidine 300mg iv or 
   po or ranitidine 50mg iv or 150mg po.
- More severe cases should receive repeated 
   doses of epinephrine 0.3ml of 1:1000 sq 
   every 20 minutes x3. Nebulized isomeric or 
   racemic epinephrine or albuterol are also 
   effective.
- Parenteral corticosteroids like SoluMedrol 
   125mg iv are also typically used, although 
   efficacy remains umproven.
- If there is a history of recurrent episodes 
   of edema and there is a family history of 
   the same, consider ordering a C4 complement 
   level or C'1 esterase inhibitor levels as a 
   screening for hereditary angioedema. In this 
   condition, the edema often involves the 
   uvula and soft palate together.
- Uvular decompression may be useful in 
   patients that are resistant to medical 
   therapy or whose symptoms progress rapidly. 
   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 patial 
   uvulectomy.
- All patients should be observed for an 
   adequate period of time to insure that there 
   is either improvement or no further 
   worsening of the swelling before being 
   discharged home. Upon discharge, they should 
   receive 4-5 days of H1 and H2 blockers and 
   steroids if required.

What not to do:
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Do not perform a comprehensive and costly 
laboratory evaluation on every case. do only 
specific tests that are clearly indicated with 
results that will be followed up.

Discussion
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The uvula (Latin for "little grape") is a small 
conical pedulous process hanging from the 
middle of lower border of the soft palate. It 
is composed of muscle, connective tissue and 
mucous membrane, with the bulk of the uvula 
consisting of glandular tissue with diffuse 
muscle fibers intersperced throughout. During 
the acts of degluttination 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.

Angioedema, also known as angioneurotic edema 
and Quincke's disease, is defined as a well- 
localized edematous condition that may variably 
involve the deeper skin layers and subcutaneous 
tissues as well as 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 basophil or mastocytes. 
Physical agents, including cold, pressure, 
light and vibration, or processes that increase 
core temperature, may also cause edema throuth 
the IgE pathway.

Hereditary angioedema, a genetic disorder of 
the complement system, is characterized by 
either an obsence of functional deficiency of 
C'1 esterase inhibitor. this allows unopposed 
activation of the first component of 
complement, with subsequent breakdown of its 
two substrates, the second (C'2) and fourth 
(C'4) components of the complement cascade. 
This process, in the presence of plasmin, 
generates a vasoactive kinin-like molecule that 
causes angioedema. Acquired C'1 esterase 
inhibitor deficiency and other complement 
consumption states have been described in 
patients with malignancies and immune complex 
disorders, including serum sickness and 
vasculidities.

Other causes of angioedema include a direct 
degranulation effect on mast cells and baso- 
phils by certain medications and diagnostic 
agents (opiates, d-tubocurarine, curare and 
radiocontrast materials); substances such as 
aspirin, nonsteroidal anti-inflammatory drugs, 
azo dyes and benzoates that alter the metabol- 
ism of arachidonic acid, thus increasing smooth 
muscle permeability; and angiotensin converting 
enzyme 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 contiguouly involve the uvula 
with the tonsils, posterior pharynx, or 
epiglottis.

References
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- Evans TC, Roberge RJ: Quincke's disease of 
   the uvula. *Am J Emerg Med* 1987;5:211-216.
- Goldberg R, Lawton R, Newton E et al: 
   Evaluationand management of acute uvular 
   edema. *Ann Emerg Med* 1993;22:251-255.

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