Refractory Epistaxis Caused by an Angiomatous Nasal Polyp Unmasking Moderate Hemophilia A in an Adolescent Male

Abstract

Objectives

To report a diagnostically challenging case of refractory epistaxis in a 16-year-old male that mimicked juvenile nasopharyngeal angiofibroma (JNA), and to highlight critical perioperative lessons regarding coagulation disorders and postoperative wound healing.

Methods

Detailed clinical, radiologic, surgical, pathologic, and hematologic data were reviewed.

Results

An adolescent male presented with recurrent unilateral epistaxis and nasal obstruction. Imaging demonstrated a mildly enhancing posterior nasal mass, and laboratory testing revealed a persistently prolonged activated partial thromboplastin time (APTT). Based on a presumptive diagnosis of JNA, preoperative embolization followed by endoscopic resection was performed. Histopathology revealed an angiomatous nasal polyp. Despite adequate intraoperative hemostasis, the patient developed recurrent postoperative epistaxis refractory to repeated surgical exploration and nasal packing. Comprehensive coagulation studies ultimately demonstrated markedly reduced factor VIII activity, confirming moderate hemophilia A. Sustained hemostasis was achieved only after recombinant factor VIII replacement combined with prolonged, atraumatic nasal compression. No recurrent bleeding occurred during follow-up.

Conclusion

Angiomatous nasal polyp in adolescent males may closely mimic JNA, particularly when compounded by an unrecognized coagulation disorder. In patients with persistent APTT prolongation, postoperative epistaxis may reflect impaired wound healing rather than inadequate surgical hemostasis. Early hematologic evaluation and appropriate factor replacement are essential to prevent repeated unnecessary surgical interventions.

Introduction

Recurrent epistaxis in adolescent males classically raises suspicion for juvenile nasopharyngeal angiofibroma (JNA), a benign yet highly vascular tumor with characteristic demographic and radiologic features. ¹ Contrast enhancement on computed tomography (CT) or magnetic resonance imaging (MRI), posterior nasal cavity involvement, and male predominance during adolescence often prompt aggressive perioperative planning, including preoperative embolization and early surgical resection.^2,3

However, not all hemorrhagic nasal lesions in this population represent vascular tumors. Inflammatory nasal polyps with hemorrhagic and necrotic changes may demonstrate imaging characteristics that overlap with JNA.^4,5 Moreover, systemic coagulation disorders—particularly mild hemophilia—may remain clinically silent until surgery or trauma precipitates persistent bleeding. In such cases, intraoperative hemostasis may be readily achieved, while postoperative wound healing is compromised, leading to recurrent epistaxis that is resistant to conventional surgical measures.

We report a case of an adolescent male with an angiomatous nasal polyp and unrecognized moderate hemophilia A, initially misdiagnosed as JNA. This case illustrates a critical diagnostic pitfall and emphasizes the importance of distinguishing surgical bleeding from coagulation-related delayed hemorrhage in otolaryngologic practice.

Case Presentation

A 16-year-old male presented with recurrent right-sided epistaxis and progressive nasal obstruction for 2 years. The nasal septum was deviated to the right, resulting in a narrowed right nasal cavity. Nasal endoscopy revealed a dark red, smooth-surfaced mass occupying the right posterior nasal cavity and prolapsing into the nasopharynx, with a suspected origin from the lateral nasal wall (Figure 1). No personal or family history of bleeding disorders was reported.

Figure 1.

Preoperative nasal endoscopy demonstrating a smooth, dark-red mass occupying the right posterior nasal cavity and prolapsing into the nasopharynx, with suspected origin from the lateral nasal wall. IT: inferior turbinate; NS: nasal septum; T: tumor.

Initial laboratory evaluation demonstrated a persistently prolonged activated partial thromboplastin time (APTT), approximately 11–12 seconds above the upper limit of normal. Prothrombin time, platelet count, and baseline hemoglobin levels were within normal ranges.

Contrast-enhanced CT demonstrated a well-circumscribed soft-tissue lesion in the posterior right nasal cavity measuring approximately 22 × 14 × 17 mm, with partial extension into the nasopharynx. Additional opacification was noted in the right maxillary sinus. MRI revealed a lesion with heterogeneous signal intensity and internal low-signal foci. Overall imaging findings suggested a vascular tumor, with juvenile nasopharyngeal angiofibroma (JNA) considered most likely (Figure 2).

Figure 2.

Imaging of the Nasal Mass (A-B) Axial MRI scans without contrast: T1-weighted and T2-weighted images showing an oval-shaped mass with heterogeneous signal intensity, indicating the hemorrhagic–necrotic nature of the lesion. (C-D) Contrast-enhanced axial and coronal CT scans demonstrating a mass in the right nasal cavity and maxillary sinus, with irregular enhancement, suggesting a suspected vascular lesion.

Preoperative selective arterial embolization was performed via the feeding branches of the internal maxillary artery using microcoils, aiming to reduce intraoperative blood loss. In accordance with standard practice to minimize collateral revascularization, endoscopic tumor resection combined with septoplasty was undertaken 24 hours later under general anesthesia.

Intraoperatively, septoplasty was performed first to improve surgical exposure. The lesion was identified as a polypoid mass arising from the lateral wall of the right maxillary sinus and extending into the choana. Blood loss was minimal and easily controlled. Frozen-section analysis showed hemorrhagic necrotic tissue. Final histopathologic examination revealed respiratory epithelial mucosa with chronic inflammation, stromal edema, small-vessel proliferation, and areas of hemorrhage and fibrinoid necrosis, consistent with an angiomatous nasal polyp (Figure 3).

Figure 3.

Histopathologic examination revealed chronic inflammatory respiratory mucosa with hemorrhage, fibrinoid necrosis, and small-vessel proliferation, consistent with an angiomatous nasal polyp.

At the conclusion of surgery, absorbable packing was placed in the right maxillary sinus, and bilateral expandable nasal sponges were positioned in the common nasal meatuses. The sponges were removed 24 hours postoperatively. The patient recovered uneventfully and was discharged on postoperative day 3 (Figure 4).

Figure 4.

Timeline of the patient’s clinical course, including symptom onset, hospital admission, preoperative embolization, surgical intervention, postoperative bleeding events, diagnostic workup, and initiation of factor VIII replacement therapy.

On the night of discharge, mild epistaxis occurred during sleep and resolved spontaneously. Recurrent bleeding the following night prompted readmission. Hemoglobin remained within normal limits, while APTT persisted at approximately 11 seconds above normal. Liver and renal function tests and screening for coagulation factor inhibitors were unremarkable. Hematology consultation suggested a possible coagulation factor deficiency and recommended fresh frozen plasma (FFP) transfusion if bleeding worsened.

Endoscopic examination under local anesthesia revealed multiple clots in the choanal region without active bleeding. The nasal septum appeared mildly bulging. During observation, intermittent oozing persisted without massive hemorrhage. APTT mixing studies and comprehensive coagulation factor assays were initiated.

On the third day of readmission, acute anterior epistaxis recurred. Emergency endoscopic exploration under general anesthesia revealed marked septal bulging. After removal of septal sutures and reopening of the previous incision, a large volume of organized blood clot was evacuated from within the bilateral septal mucosal flaps, consistent with a septal hematoma. No discrete arterial bleeding source was identified. Hemostasis was achieved with limited bipolar cauterization at suspected suture penetration sites, followed by denser septal resuturing and bilateral expandable sponge packing for 72 hours.

Although bleeding ceased during nasal packing, slow bilateral mucosal oozing recurred shortly after sponge removal. A total of approximately 2,600 mL of fresh frozen plasma was transfused; however, the APTT was reduced by only 3 seconds, and intermittent bleeding persisted. When the patient’s hemoglobin level reached a nadir of 93 g/L. A second emergency exploration under general anesthesia was therefore performed. In light of the patient’s apparent bleeding diathesis, septal sutures were preserved, and only minimal cauterization was applied to suspected oozing areas of the septal mucosa. Firm, prolonged nasal compression was achieved using extensive gelatin sponge packing in both nasal cavities, supplemented by posterior nasal balloon tamponade to prevent displacement and ensure uniform pressure. Iodoform gauze was deliberately avoided to reduce the risk of mucosal trauma and rebleeding upon removal.

Subsequently, coagulation testing revealed a reduced factor VIII activity level of 4.1% (reference range: 70.0–150.0%), consistent with moderate hemophilia A. Recombinant human factor VIII replacement therapy was urgently initiated at a dose of 2000 IU every 24 hours. Following factor replacement, epistaxis resolved completely.

Fourteen days after the final procedure, all nasal packing materials were removed without bleeding. Factor VIII replacement was discontinued on postoperative day 17, and the patient was discharged in stable condition. Follow-up evaluations at 1 and 3 months demonstrated good mucosal healing and no recurrence of epistaxis.

Discussion

This case illustrates a multifactorial diagnostic and therapeutic pitfall in the management of adolescent epistaxis. Angiomatous nasal polyp are uncommon but may present with prominent bleeding and radiologic enhancement, thereby mimicking JNA.^4,5 In the present case, the patient’s age, sex, posterior nasal cavity involvement, and imaging features reasonably supported a presumptive diagnosis of JNA and justified preoperative embolization.

However, the coexistence of an unrecognized systemic coagulation disorder fundamentally altered the postoperative course. Mild hemophilia A is characterized by residual factor VIII activity sufficient to prevent spontaneous bleeding under normal conditions. ⁶ As a result, patients may remain undiagnosed until surgical intervention or trauma challenges hemostatic reserve. Importantly, bleeding in such patients often manifests not as uncontrolled intraoperative hemorrhage, but as delayed postoperative mucosal oozing and impaired wound healing.^7,8 In the present case, factor VIII activity was markedly reduced to 4.1% (reference range: 70.0–150.0%), consistent with moderate hemophilia A, ⁹ which may still present with relatively mild bleeding symptoms under baseline conditions. Although hemophilia A is an X-linked recessive disorder caused by mutations in the F8 gene encoding coagulation factor VIII, no family history of bleeding tendency was identified. The patient’s mother and extended family members reported no history of abnormal bleeding, suggesting the possibility of a sporadic mutation or an asymptomatic carrier state. Genetic testing identified a heterozygous TUBA8 variant (c.1205G>A/p. Arg402Gln) of uncertain significance, which is associated with autosomal dominant macrothrombocytopenia type 2. However, this finding does not explain the observed factor VIII deficiency. This finding highlights the importance of cautious interpretation of incidental genetic results in clinical practice. Further targeted analysis of the F8 gene may be warranted to clarify the underlying genetic mechanism.

In this patient, intraoperative bleeding was minimal, and the tumor base within the maxillary sinus demonstrated no active hemorrhage following complete mucosal eradication. Instead, postoperative bleeding predominantly originated from the nasal septal mucosa and suture penetration sites, leading to septal hematoma formation and recurrent epistaxis. These findings underscore that persistent postoperative bleeding in the absence of a clear surgical source should prompt immediate reconsideration of systemic hemostatic factors.

The case further highlights a practical challenge in clinical decision-making. Following preoperative embolization, early surgical resection within 24–48 hours is recommended to avoid collateral revascularization and increased intraoperative bleeding.^10,11 This time-sensitive strategy may inadvertently limit comprehensive evaluation of abnormal coagulation parameters, as occurred in this case. Persistent, unexplained APTT prolongation—even if modest—should therefore be interpreted cautiously in the perioperative setting.

Management of postoperative epistaxis in patients with coagulation disorders requires a paradigm shift. Repeated cauterization and short-term packing may be ineffective and potentially harmful. Instead, prolonged, atraumatic nasal compression combined with definitive correction of the underlying coagulation defect is essential. In this case, sustained hemostasis was achieved only after recombinant factor VIII replacement and long-term gelatin sponge packing with posterior tamponade, allowing sufficient time for mucosal healing. The use of FFP in this case underscores an important clinical consideration. While FFP may provide temporary coagulation support, it contains relatively low concentrations of factor VIII and requires large infusion volumes to achieve therapeutic levels.

Finally, heightened multidisciplinary awareness is critical. Particular attention was paid to minimizing airway mucosal trauma during intubation and extubation, as airway bleeding with clot formation poses a risk of acute airway obstruction in patients with coagulation abnormalities.^12-14

The patient and his family initially experienced significant anxiety due to recurrent and refractory epistaxis. Following diagnosis and appropriate management, they expressed relief in understanding the underlying cause. They emphasized the importance of early evaluation for persistent nasal bleeding and appreciated the multidisciplinary approach that led to the final diagnosis.

Conclusion

Angiomatous nasal polyp in adolescent males may closely mimic JNA, particularly when compounded by an unrecognized coagulation disorder. In patients with persistent APTT prolongation, refractory postoperative epistaxis often reflects impaired mucosal healing rather than inadequate surgical technique. Early hematologic evaluation, judicious perioperative planning, and appropriate factor replacement therapy are essential to prevent repeated surgical interventions and achieve durable hemostasis.