Juvenile nasopharyngeal angiofibroma

Background
  • Juvenile nasopharyngeal angiofibromas (JNAs) are rare, highly vascular and aggressive tumors that almost exclusively present in adolescent males[1]
  • Primary vascular supply in almost all tumors arise from the internal maxillary artery, and some patients may present primarily with recurrent and poorly controlled epistaxis[2]


Clinical Features
  • Adolescent males most common
  • Almost always unilateral
  • Often presenting with history consistent with sinusitis, though with abnormal elements that may include cranial nerve abnormalities, or failure of treatment
  • Can present with asymmetric facial swelling/pain
  • Tumors can lead to local destruction and invasion, and can extend through ethmoid plate and cause cranial nerve deficits, decreased visual acuity, and abnormal extraocular movements
  • Almost all are primarily in nasopharynx, but extranasal cases have been reported
  • Highly prone to epistaxis

Differential Diagnosis

Rhinorrhea

Evaluation
  • In conjunction with consultants, will need MRI preferably, CT if unavailable
  • Thorough evaluation of CN I-XII, with emphasis on smell, visual acuity, extraocular movements

Management
  • Urgent ENT consultation for surgical planning
    • Though some cases will resolve later in life, management is generally surgical with prior embolization[3]
  • If uncontrolled bleeding, follow standard epistaxis treatments and contact IR for possible embolization

Disposition
  • Discharge with ENT follow up
    • Strict no digital trauma to nose, no foreign bodies, no steroid inhalers-avoid anything that would increase bleeding risk
  • If cranial nerve involvement may consider admission for observation prior to urgent surgery

See Also

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., & Mishra, S. C. (2017). Juvenile ‘Perinasal’ Angiofibroma. Indian Journal of Otolaryngology and Head & Neck Surgery, 69(1), 67–71.
  3. 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.
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