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The Unexpected Uvula

Wiske, Clay P.; McGregor, Alyson J. MD

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doi: 10.1097/01.EEM.0000414681.09689.6e
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    A 37-year-old man presents with eight days of bilateral ear pain and productive cough with green sputum. His past medical history is significant only for recurrent upper respiratory infections. He takes no prescribed medications.

    On examination, he is afebrile with stable vital signs. After noting coarse breath sounds and mild fullness in the middle ear bilaterally, you instruct the patient to open his mouth and say ah. You unexpectedly find a healthy appearing uvula that is bifurcated.

    A bifid or bifurcated uvula exists in two percent of the general population. Its significance lies in its associations: recurrent otitis media, submucous cleft palate, and in rare cases, aortic aneurysm and rupture at an early age. It is caused by incomplete fusion of the palatine shelves during development. Thirty percent of those with a submucous palatal cleft have a bifid uvula. Submucous cleft palate results in a bony defect in the palate that is usually not visualized. Patients with submucous cleft palate are unable to elevate the soft palate effectively for swallowing and phonation, which can lead to nasal speech, difficulty swallowing food, and recurrent otitis media.

    Loeys-Dietz syndrome is a rare autosomal recessive disease characterized by bifid uvula, hypertelorism, and arterial tortuosity. It strongly predisposes to early aneurysms anywhere in the arterial tree but especially at the aortic root. These patients may present to the emergency department with intense or prolonged pain that is often caused by arterial aneurysm. Imaging should be performed promptly in Loeys-Dietz patients presenting with pain.

    Although bifid uvula is in itself a benign finding and should not be treated, its association with recurrent infections and arterial aneurysms may be significant in an emergency department. This patient was eventually diagnosed with viral URI, and did well with symptomatic treatment. The emergency physician discussed the risks of recurrent URI and potential future aneurismal complications with him and his primary care physician.

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