2019 EMage Contest First Place Winner: Helluva Uvula

Evan Laveman, MD Emergency Medicine PGY3
Kelly Kelley, MSN, RN, CPNP, CNS
Christopher Redgate, MD, MS, PEM Fellow PGY6

Pediatric Emergency Department, Department of Emergency Medicine, Harbor-UCLA

16-year-old male with no past medical history presented to the Pediatric Emergency Department (PED) for 4 months of nasal congestion worsening over the past month. He immigrated from Honduras with his father 20 days ago and over this time noticed a decreased ability to breath through his nose. Most troubling, he started to develop foul smelling nasal discharge mainly through his left nare. His only medical contact had been at a U.S. clinic when he first arrived where he was prescribed a nasal spray and cetirizine with no relief. He felt moderate sinus pressure but denied weight loss, fevers, chills, headache or vision changes.

On examination, his left nostril was obstructed by a mass covered in foul smelling pus that could not be cleared with sneezing. Once irrigated and cleaned, the mass appeared to be a pink, vascularized mucosal structure (Image 1). The right nare was patent and normal appearing.

On further examination, the nasopharynx appeared full and the uvula was midline but anteriorly displaced by a similarly pink mass which appeared to descend into the hypopharynx (Image 2). The submandibular spaces, peritonsillar and lingual architecture appeared normal. There was no proptosis, his phonation was normal, and remaining cranial nerve examination was unremarkable.

 

Pictures (Exam Findings)

Image 1:

 

Image 2:

Image 3:

CT (Pictures)

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Image 6:

A CT revealed an enhancing mass (Images 4-6) that occupied the entire left nasal cavity and obliterated the turbinates. The mass also invaded through the lateral wall of the left maxillary sinus down to the hypopharynx and was penetrated through the mucosa of the nasopharynx, explaining the anterior displacement and appearance of the uvula seen on examination.

Conclusion

Juvenile nasopharyngeal angiofibromas (JNA) are rare, highly vascular and aggressive tumors that almost exclusively present in adolescent males1. Primary vascular supply in almost all tumors arise from the internal maxillary artery, and some patients may present primarily with recurrent and poorly controlled epistaxis2. Almost all arise primarily in nasopharynx, but case reports have discussed extranasal manifestations that present with asymmetric facial swelling3. These tumors lead to local destruction and invasion, eventually extending through the ethmoid plate. This can lead to cranial nerve deficits that can present with anosmia, decreased visual acuity and limited extraocular movements. Treatment is surgical, and often is preceded by IR embolization2.

 

References

  1. Mehan, R., Rupa, V., Lukka, V. K., Ahmed, M., Moses, V., & Shyam Kumar, N. K. (2016). Association between vascular supply, stage and tumour size of juvenile nasopharyngeal angiofibroma. European Archives of Oto-Rhino-Laryngology, 273(12), 4295–4303. https://doi.org/10.1007/s00405-016-4136-9
  2. Mishra, A., & Verma, V. (2019). Implication of embolization in residual disease in lateral extension of juvenile nasopharyngeal angiofibroma. Journal of Oral Biology and Craniofacial Research, 9(1), 115–118. https://doi.org/10.1016/j.jobcr.2018.12.001
  3. Mishra, A., Verma, V., & Mishra, S. C. (2017). Juvenile ‘Perinasal’ Angiofibroma. Indian Journal of Otolaryngology and Head & Neck Surgery, 69(1), 67–71. https://doi.org/10.1007/s12070-016-1050-9

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