Timely resection of juvenile nasopharyngeal angiofibroma following embolization for uncontrolled acute epistaxis case report

Abstract

Uncontrolled epistaxis in patients with juvenile nasopharyngeal angiofibroma requires prompt intervention. When embolization is required, neovascularization can occur, making expedited surgical resection essential. We conducted a single-institution, retrospective review of one patient. This case study details the patient’s clinical presentation and his preoperative, intraoperative, and postoperative course. We present the management of a 14-year-old male with a juvenile nasopharyngeal angiofibroma, who 3 days after initial evaluation at our institution, developed uncontrolled epistaxis requiring embolization at an outside hospital before transfer back to our institution for further management. He initially presented to an outside emergency department with acute epistaxis refractory to medical treatment. He was subsequently transferred to another facility for embolization. After successful embolization, he was transferred to our institution and underwent surgical resection within 48 h. A combined transnasal endoscopic and transmaxillary approach was used for excision. Pathological analysis confirmed the diagnosis of juvenile nasopharyngeal angiofibroma. Twenty-four months after post-embolization and resection, the patient remains asymptomatic with no evidence of recurrence on magnetic resonance imaging. This case report highlights the potential need to develop a clinical tool to risk stratify juvenile nasopharyngeal angiofibroma patients at risk for uncontrolled epistaxis. It also underscores the importance of timely transfer from resource-limited settings to centers capable of coordinating interventional radiology and surgical resection within 48 h of embolization.

Introduction

Juvenile nasopharyngeal angiofibroma (JNA) is a rare tumor that typically presents in adolescent males. When present, surgical resection is the mainstay of treatment.

However, a database review demonstrated that 31.5% of individuals with JNAs had a history of epistaxis. ¹ Acute epistaxis can be managed with first-line and complex management. First-line treatments include nasal compression, application of vasoconstrictors, hemostatic agents, nasal packing, and nasal cautery. ² If bleeding persists, arterial ligation and/or interventional radiology procedures such as embolization can be considered. ³

Embolization is performed in conjunction with bilateral carotid angiography to visualize and occlude the feeder vessels to the tumor. ⁴ Common feeder vessels targeted during embolization include the external carotid, internal maxillary artery, Vidian artery, accessory meningeal artery, mandibular artery, and facial artery. This technique serves as an effective adjuvant therapy prior to surgery by reducing intraoperative bleeding and may shrink the tumor.^4,5

However, post-embolization, neovascularization can occur,^3,4,6,7 with an Italian clinical trial indicating that neovascularization can happen within 96 h, depending on the type of embolization material used—resorbable versus non-resorbable. ⁷ Literature suggests that surgical resection of a JNA should occur 24–48 h after embolization. Kuan et al. suggest that embolization should be performed within 24 h to prevent collateral blood supply development, ⁴ while Wilson et al. highlight the importance of timely surgical intervention, within 48 h, to avoid neovascularization and reduce the risks of tumor swelling and necrosis. ³

We present the case of an adolescent male with JNA and acute epistaxis requiring embolization to discuss perioperative care. This case report is unique in that it highlights the importance of timely transfer of JNA patients experiencing uncontrolled epistaxis from resource-limited sites to centers capable of coordinating with an interventional radiologist for embolization and an otolaryngologist for timely surgical resection (Supplementary Material). It also reveals the need for further research to develop clinical risk assessment tools for uncontrolled epistaxis and a standardized protocol for the management of intractable epistaxis in JNA patients. This report is exempt from IRB review based on the University of Wisconsin Human Research Protection Program.

Case report

A 14-year-old male with a left-sided vascular nasal mass presented to our otolaryngology clinic after being referred from an outside hospital.

One month prior, at the outside hospital, the patient presented to an otolaryngologist with symptoms of epistaxis, including one emergency department (ED) visit that was controlled by conservative management, and epiphora. The nasal mass was identified on nasal endoscopy and was initially suspicious for an antrochoanal polyp, but the vascularity of the lesion prompted further imaging. After computed tomography (CT) and magnetic resonance imaging (MRI) were obtained, the patient was sent to our institution for evaluation.

At our institution, he reported having epistaxis, which resolved on its own, and epiphora. He otherwise denied any headaches, facial numbness, lymphedema, and blurred or double vision. The patient’s family history was positive for Factor V Leiden and negative for vascular lesions. His examination was notable for reduced soft palate elevation on his left side. Facial sensation to light touch was normal bilaterally. On nasal endoscopy, a left-sided nasal mass was identified. His physical examination findings and outside imaging were consistent with an Andrews-Fisch stage III JNA (Figure 1). His CT scan revealed a lobulated soft tissue mass that extended from the posterior left choanal region to fill the left nasal passage with osseous remodeling. In addition, anterior bowing of the posterior and medial left maxillary sinus was appreciated—also known as the Holman–Miller sign. On MRI, the mass was measured to be 5.5 × 5.3 × 4.7 cm and centered within the left pterygopalatine fossa and sphenopalatine foramen. T2 imaging showed a hypointense mass with flow voids present (Figure 1(b)), and T1 imaging showed an isointense lesion with an atelectatic left maxillary sinus with opacified fluid (Figure 1(c)). A plan for angiography, embolization, and resection was made, and a date was being discussed.

Figure 1.

Imaging of left-sided JNA. (a) Axial CT without contrast revealing a left pterygomaxillary space tumor. (b) Axial T2 MRI of left-sided hypoenhancing tumor. (c) Coronal T2 FSE MRI of left-sided hypoenhancing tumor. (d) Nasal endoscopic view. Nasal tumor (*).

Three days later, the patient presented to an outside ED with acute-onset unilateral epistaxis. Conservative medical management in the form of topical epinephrine and tranexamic acid was administered; however, the epistaxis persisted. Given the patient’s known diagnosis of a JNA, he was transferred to another local hospital with interventional radiology and otolaryngology services for further management. There, diagnostic nasal endoscopy revealed bleeding from the JNA. Attempts to identify and control the bleeding source endoscopically were unsuccessful. The volume of blood loss prior to embolization was unknown. To control bleeding, angiography and embolization were required. Written informed consent was obtained from the patient’s legally authorized representative prior to undergoing embolization. Pre-embolization laboratory values were as follows: hemoglobin (Hbg) 13.7, hematocrit (Hct) 38.7%, and international normalized ratio 1.4. Angiography revealed that the distal internal maxillary artery supplied the tumor. Transarterial embolization with Embospheres devascularized 70%–80% of the tumor and resulted in control of the epistaxis, with a post-embolization Hbg 13.0 (Figure 2). Angiography demonstrated a left-sided eccentric hyper-vascular mass located in the posterior nasal cavity. The lesion was supplied by branches of the internal maxillary artery with collateral blood flow (Figure 2(a)). After successful embolization, there was a ~70%–80% devascularization of the mass (Figure 2(b)).

Figure 2.

Coronal view of left internal maxillary artery angiogram pre- (a) and post-embolization (b). Left internal maxillary artery (*).

The following day, the patient was stable and transferred to our hospital for resection the next day. Pre-resection labs were as follows: Hb 8.6 and Hct 21%. Prior to undergoing resection, written informed consent was obtained from the patient’s legally authorized representative. He underwent a combined transnasal endoscopic and transmaxillary approach for excision. Intraoperatively, a transfusion of packed red blood cells was administered. The estimated intraoperative blood loss was 1200 ml, but the case was otherwise uncomplicated. Pathological examination confirmed the specimen to be a JNA. Twenty-four months after embolization and resection, the patient remains asymptomatic, and MRI imaging showed expected post-surgical changes and aeration of paranasal sinuses (Figure 3).

Figure 3.

Axial T1 MRI 24-month status post-embolization and excision of JNA, revealing postsurgical and no signs of recurrent disease.

Discussion

JNA is a benign but invasive vascular tumor that primarily affects adolescent males, often presenting with unilateral epistaxis and nasal obstruction. ⁶ It is crucial for clinicians to consider JNA in cases of sudden unilateral epistaxis, that is, resistant to medical management, particularly in this demographic. ³

Management of acute epistaxis in patients with JNA typically begins with conservative measures, including nasal compression, vasoconstrictors (oxymetazoline, epinephrine, or phenylephrine), and cautery. ² When these approaches fail, more invasive options such as embolization become necessary (Figure 4). In our case, the surgical date was still being decided when the patient experienced his episode of uncontrolled epistaxis. This highlights the need for clinical decision models to identify and stratify JNA patients at risk for such events. Had such a tool been available, the patient might have received preoperative embolization sooner.

Figure 4.

Algorithm for management of acute epistaxis in JNA patients. When patients with a known history of JNA present to the ED or urgent care with acute epistaxis, the first step is conservative management, including nasal compression, topical decongestants, and vasoconstrictors. A risk stratification tool would aid in triaging which patients would benefit from initial conservative management versus those who should be referred directly to IR for embolization. If the epistaxis is controlled with conservative measures, patients should follow up with ENT and plan for embolization and resection of their JNA. If bleeding is not controlled, an ENT consult is warranted for endoscopic evaluation and possible intervention. Following urgent ENT evaluation, IR should be consulted for embolization. The lesion should be resected within 24–48 h after embolization.

Angiography is essential for identifying the tumor’s vascular supply of the tumor prior to embolization. ³ In this case, the distal internal maxillary artery was identified as the primary blood supply, allowing for effective embolization and resulting in substantial devascularization of the tumor and immediate hemostasis. Previous studies have shown that embolization not only provides hemostatic control but also significantly reduces intraoperative blood loss during subsequent surgical resection.^3,5

Following embolization, our patient underwent surgical resection within 48 h, which aligns with recommendations in the literature. ³ Delayed resection can increase the risk of tumor neovascularization, which may lead to potential complications such as greater intraoperative bleeding and prolonged recovery. ³ In such cases, blood transfusion should be prepared in advance of the operation, as large blood loss occurs in some cases.

For patients presenting with refractory epistaxis, our findings support a treatment protocol prioritizing embolization followed by prompt surgical resection—within 24–48 h—to optimize patient outcomes.

Further research is needed to establish standardized protocols for the management of acute epistaxis in JNA patients. These studies should address the optimal timeframe between embolization and resection, transfer criteria to tertiary centers, and the development of clinical decision tools for identifying patients at high risk of uncontrolled bleeding. Larger cohort studies are also essential to evaluate the long-term outcomes and recurrence rates associated with different management strategies.

Conclusion

For patients with JNA experiencing uncontrolled epistaxis, embolization should be prioritized, followed by timely surgical resection.

Supplemental Material

sj-pdf-1-sco-10.1177_2050313X251364086 – Supplemental material for Timely resection of juvenile nasopharyngeal angiofibroma following embolization for uncontrolled acute epistaxis case report

Supplemental material, sj-pdf-1-sco-10.1177_2050313X251364086 for Timely resection of juvenile nasopharyngeal angiofibroma following embolization for uncontrolled acute epistaxis case report by Koffi L. Lakpa, Timothy McCulloch and Michael D. Puricelli in SAGE Open Medical Case Reports