Juvenile Nasopharyngeal Angiofibroma in a Female Child Presenting with Massive Epistaxis and Hemorrhagic Shock: A Rare Case Report

Abstract

Juvenile nasopharyngeal angiofibroma (JNA) is a rare, hypervascular tumor almost exclusively found in adolescent males. Its occurrence in females is an extraordinary clinical outlier, representing less than 1% of cases. This case report aims to describe a rare case of JNA in a female child and to highlight the importance of preoperative imaging in preventing catastrophic hemorrhagic complications. An 8-year-old girl presented with massive epistaxis and hemorrhagic shock ten days after an adenoidectomy performed for presumed adenoid hypertrophy. Computed tomography angiography (CTA) and digital subtraction angiography (DSA) revealed a hypervascular mass supplied predominantly by branches of the bilateral internal maxillary arteries. After hemodynamic stabilization, preoperative transarterial embolization using coils and Onyx was performed, followed by successful endoscopic tumor resection. Histopathological examination confirmed JNA, demonstrating dilated vascular channels lacking a muscular layer. The patient recovered well with no evidence of recurrence on follow-up. This case highlights that the absence of a muscular layer in JNA vessels prevents effective vasoconstriction, predisposing patients to severe hemorrhage when the tumor is manipulated without prior devascularization. Its clinical resemblance to adenoid hypertrophy may lead to misdiagnosis and inappropriate intervention. Therefore, comprehensive imaging and preoperative embolization are essential to minimize intraoperative bleeding and optimize surgical outcomes. In conclusion, JNA should be considered in the differential diagnosis of nasopharyngeal masses in pediatric patients regardless of sex. Routine preoperative imaging is crucial to identify vascular lesions and ensure safe, multidisciplinary management. (www.actabiomedica.it)