Concomitant Juvenile Nasopharyngeal Angiofibroma and Moyamoya Disease: A Rare Clinical Confluence

Ovais H1*, Ahmed H2, Ejaz AD1, Malaika H1, Maliha R1, Preet I1 and Aaqil SI1

1Department of Surgery, Jinnah Sindh Medical University, Pakistan 2Department of ENT Head & Neck Surgery, Jinnah Post Graduate Medical Centre, Pakistan

^*Correspondance to: Haziq Ovais 

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Abstract:

Juvenile Nasopharyngeal Angiofibroma (JNA) is a rare and invasive tumor primarily affecting adolescent males. This case report presents a unique scenario of a 15-year-old adolescent diagnosed with JNA concurrently with Moyamoya disease, a cerebrovascular disorder characterized by abnormal brain vascular networks. The patient experienced persistent nasal obstruction, anosmia, and recurrent episodes of epistaxis for 2.5 years which progressively worsened. Clinical examination revealed a prominent pink/red mass in the nasopharynx, and imaging confirmed a sizable mass in the sphenopalatine foramen, causing luminal obliteration and extending into nearby structures. The staging of the JNA indicated stage IIIa according to the Andrews-Fisch system. Additionally, the patient was found to have advanced Moyamoya disease (Suzuki stage VI) through cerebral angiography, showing complete occlusion of both internal carotid arteries and proximal segments of the anterior and middle carotid arteries. Due to the high stroke risk associated with Moyamoya disease and the presence of collateral blood supply, embolization as a preoperative intervention was not feasible. Unfortunately, delayed diagnosis and limited resources made surgical resection for JNA and revascularization for Moyamoya disease impractical. This case highlights the diagnostic complexities and resource limitations encountered when managing the coexistence of JNA and Moyamoya disease, underscoring the importance of early recognition, interdisciplinary collaboration, and optimal resource allocation in challenging clinical scenarios. The rarity of this coexisting condition calls for increased awareness and understanding among clinicians to make timely and informed decisions, ultimately improving patient outcomes. Further research and multidisciplinary collaboration are needed to develop optimal management strategies for complex presentations like this, considering the impact of resource availability and regional limitations on treatment options and outcomes.

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Cite the Article:

Ovais H, Ahmed H, Ejaz AD, Malaika H, Maliha R, Preet I, et al. Concomitant Juvenile Nasopharyngeal Angiofibroma and Moyamoya Disease: A Rare Clinical Confluence. Ann Clin Case Rep. 2024; 9: 2594.