Juvenile nasopharyngeal angiofibroma

Background

• Juvenile nasopharyngeal angiofibromas (JNAs) are rare, highly vascular and aggressive tumors that almost exclusively present in adolescent males
• 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

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

• Upper respiratory infection, influenza
• Sinusitis
• Juvenile nasopharyngeal angiofibroma
• Nasal polyp
• Nasal mass
• Nasal foreign body
• CSF leak (e.g. basilar skull fracture)
• Toxic inhalation (e.g. selenium toxicity, neurotoxic shellfish poisoning)

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
• 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