Juvenile nasopharyngeal angiofibroma: Difference between revisions
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*Juvenile nasopharyngeal angiofibromas (JNAs) are rare, highly vascular and aggressive tumors that almost exclusively present in adolescent males<ref>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</ref> | *Juvenile nasopharyngeal angiofibromas (JNAs) are rare, highly vascular and aggressive tumors that almost exclusively present in adolescent males<ref>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</ref> | ||
*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<ref>Mishra, A., Verma, V., & Mishra, S. C. (2017). Juvenile ‘Perinasal’ Angiofibroma. Indian Journal of Otolaryngology and Head & Neck Surgery, 69(1), 67–71. </ref> | *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<ref>Mishra, A., Verma, V., & Mishra, S. C. (2017). Juvenile ‘Perinasal’ Angiofibroma. Indian Journal of Otolaryngology and Head & Neck Surgery, 69(1), 67–71. </ref> | ||
==Clinical Features== | ==Clinical Features== | ||
*Adolescent males most common | *Adolescent males most common | ||
*Often presenting with history consistent with sinusitis, though with abnormal elements that may include cranial nerve abnormalities, or failure of treatment | *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 | *Highly prone to epistaxis | ||
Revision as of 03:00, 22 July 2019
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
- 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[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
- ↑ 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
- ↑ Mishra, A., Verma, V., & Mishra, S. C. (2017). Juvenile ‘Perinasal’ Angiofibroma. Indian Journal of Otolaryngology and Head & Neck Surgery, 69(1), 67–71.
- ↑ 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.