Multidisciplinary management of juvenile nasopharyngeal angiofibroma

Background Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign, but the locally aggressive, vascular tumor that occurs almost exclusively in young men. Although this tumor accounts for only about 0.5% of head and neck tumors, it is by far the most common benign nasopharyngeal neoplasm. It is presented typically with epistaxis, obstructive symptoms, and chronic otomastoiditis. The examination of this tumor reveals pale reddish-blue mass. The tumor is seen on imaging as vividly augmenting soft-tissue mass centered on the sphenopalatine foramen. The treatment of choice is usually surgical resection; either open or endoscopic. Pre-operative embolization is usually done to help with hemostasis. We aim to address the efficiency and the safety of endovascular modality in JNA. Methods In the period between January 2012 to December 2017, 20 male patients with age ranging from 6 to 20 years were referred to the Endovascular Unit, at Departments of Neurosurgery in Sohag, Alexandria, and Tanta University Hospitals with JNF. Preoperative trans-arterial embolization was done, followed by endoscopic surgical resection by our ENT surgeons. All patients were subjected to clinical and radiological Imaging evaluation pre embolization, post embolization, and post endoscopic resection. All patients had clinical and radiological follow-ups for at least 1 year. Results 20 male patients with JNA underwent preoperative super-selective trans-arterial embolization. The average age at presentation was 13 years. All patients underwent successful embolization with total obliteration of vascular blush of the tumor in 15 patients, and near-total obliteration of blush in 5 patients. The dramatic effect of preoperative embolization was observed on limiting intraoperative blood loss (average 200 ml) and decreasing the time of surgery (average 2.5 h). There was no permanent morbidity or mortality related to either embolization or endoscopic resection in all patients. Conclusions Trans-arterial embolization is strongly recommended in patients with JNA, it offers a good assist to the surgeon, reduces blood loss, decreases the amount of transfusion, improves the degree of resection, reduces operative time, and decreases the incidence of recurrence.

JUVENILE NASOPHARYNGEALANGIOFIBROMA: OUR EXPERIENCE AND LITERATURE REVIEW

ABSTRACT Introduction: Juvenile nasopharyngeal angiofibroma is a benign vasculartumor.It is commonly found in teen age males. Its site of origin is sphenopalatine foramen. Exact pathogenesis of angiofibroma is not known. It has predictable natural history and growth pattern. This tumor most often involves nasopharynx, nasal cavity, paranasal sinuses, pterygopalatine fossa and infratemporal fossa. It can also involve orbit and can spread intracranially. Its very important to diagnose this tumor very early on the basis of clinical examination and imaging. As early tumor confined to nose and sinuses can be removed exclusively with endoscope. It is very helpful to do angiography before surgery to ascertain itsblood supply and then embolization can be done to reduce intraoperative bleeding. Objective: To describe our experience of Juvenile Nasopharyngeal Angiofibroma cases in ENT Unit-I of Lahore General Hospital. Study Design: Descriptive Study with retrospective analysis after approval from Institutional Review Board (IRB) of LGH/PGMI/AMC Lahore. Methods: We studied 20 patients who underwent surgery in our department from October 2019 to October 2020. We analyzed following factors: age, gender, symptoms, staging, mode of surgery and need for intraoperative blood transfusion, hospital stay, complications and recurrences. Results: Range of patient’s age was 12 to 25 years. Eight patients underwent surgery with endoscope. Mean blood loss was about 400 ml and mean operating time was 140 minutes. All the cases were embolized preoperatively. Conclusion: Endoscopic surgery is a safe and effective method in early stage JNA patients. While patients with advance stage tumors should be managed with combined endoscopic and conventional open approaches. KEYWORDS: juvenile nasopharyngeal angiofibroma, JNA, endoscopic surgery

Twenty Years of Experience in Juvenile Nasopharyngeal Angiofibroma (JNA) Preoperative Endovascular Embolization: An Effective Procedure with a Low Complications Rate

Juvenile nasopharyngeal angiofibroma (JNA) is a benign tumor of the nasal cavity that predominantly affects young boys. Surgical removal remains the gold standard for the management of this disease. Preoperative intra-arterial embolization (PIAE) is useful for reductions in intraoperative blood loss and surgical complications. In our series of 79 patients who underwent preoperative embolization from 1999 to 2020, demographics, procedural aspects, surgical management and follow-up outcome were analyzed. Embolization was performed in a similar fashion for all patients, with a superselective microcatheterization of external carotid artery (ECA) feeders and an injection of polyvinyl alcohol (PVA) particles, followed, in some cases, by the deployment of coils . Procedural success was reached in 100% of cases, with no complications such as bleeding or thromboembolic occlusion, and surgical intraoperative blood loss was significantly decreased. In conclusion, PIAE is a safe and effective technique in JNA treatment, minimizing intraoperative bleeding.

Subcutaneous emphysema and pneumomediastinum after endoscopic transnasal removal of nasopharyngeal angiofibroma

Introduction. Juvenile nasopharyngeal angiofibroma is a rare, benign, well-vascularized tumor of the skull base characterized by destructive growth. The development of endoscopic techniques and experience of surgeons have enabled the removal of this tumor both at early stages and late stages (advanced disease). Patients may develop various complications in the intraoperative and postoperative periods, including massive bleeding, nasal liquorrhea, facial paresthesia, lacrimal hyposecretion, etc. Air penetration into the subcutaneous fat and mediastinum during endoscopic surgery on the paranasal sinuses is rare. Such complication as subcutaneous emphysema and pneumomediastinum after endoscopic endonasal removal of juvenile nasopharyngeal angiofibroma has not been reported in the literature.Case report. A 19-year-old male patient has undergone endoscopic endonasal removal of juvenile nasopharyngeal angiofibroma. The tamponade was removed within the first 24 h postoperatively. Ten hours after it, the patient developed subcutaneous emphysema and pneumomediastinum triggered by sneezing. The diagnosis was confirmed by computed tomography of the neck and chest. The patient was transferred to the intensive care unit and received conservative treatment (including infusion, antibacterial, and antiinflammatory therapy). The symptoms of subcutaneous emphysema and pneumomediastinum subsided in response to treatment. Follow-up examinations (computed tomography and magnetic resonance imaging) confirmed that the tumor had been completely removed. The patient was discharged in a satisfactory condition.Conclusion. Subcutaneous emphysema and pneumomediastinum are exceedingly rare complications of endoscopic endonasal removal of juvenile nasopharyngeal angiofibroma and are caused by anatomical connection between the parapharyngeal / retropharyngeal spaces and mediastinum. To prevent such complications, it is necessary to keep tampons in the nasal cavity for at least 2 days, as well as to instruct patients after surgery (avoid sneezing with their mouth closed, lifting weights, coughing, and vomiting). The nasoseptal flap used to repair the nasopharyngeal defect after tumor removal also ensures its sealing. Patients with complications should undergo computed tomography of the neck and chest (in case of emergency) and should be transferred to an intensive care unit. Conservative treatment (antibacterial and antiinflammatory therapy) will ensure good results in most patients.