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Endoscopic Excision of Juvenile Nasopharyngeal Angiofibroma (JNA)

Abstract

Introduction :Juvenile Nasopharyngeal Angiofibroma (JNA) is a benign but a locally aggressive vascular tumor. This usually  affects the prepubertal or adolescent males. This video highlights a safe and affective endoscopic technique for JNA resection with minimal intraoperative bleeding and morbidity .

Case presentation : A 17-year-old male presented with recurrent right sided epistaxis and constant nasal obstruction. Imaging revealed a hypervascular mass in the right nasopharynx extending into right nasal cavity and pushing the septum towards left side.

Method: 6 vessel cerebral angiogram was performed and the feeding vessels were embolized with cyanoacrylate glue. The patient underwent endoscopic endonasal resection using a bi-nostril, four-handed technique with image guidance.

Conclusion: Endoscopic resection of JNA offers excellent visualization and reduce morbidity. Proper preoperative planning, embolization, and anatomical knowledge are key to successful outcomes.

Surgeons:

Deepa Shivnani, MD

Speed Olivia, MD

Sidarth Patel, MD

Gresham Richter, MD, FACS

Department of Otolaryngology – Head and Neck Surgery, University of Arkansas for Medical Sciences, Little Rock, AR, USA

Arkansas Children’s Hospital, Little Rock, AR, USA

Video description –

This video demonstrates the endoscopic surgical excision of a Juvenile Nasopharyngeal Angiofibroma
Juvenile Nasopharyngeal Angiofibroma or JNA is a relatively rare benign neoplasm generally seen in prepubertal and adolescent males, usually present with nasal airway obstruction, recurrent unilateral epistaxis, headache and facial swelling. JNA grows in close proximity to the posterior attachment of the middle turbinate near the superior border of the sphenopalatine foramen and can extend anteriorly into the nasal cavity and septum superiorly into the sphenoid sinus and laterally toward the pterego-palatine fossa.
“This video demonstrates the endoscopic surgical excision of a Juvenile Nasopharyngeal Angiofibroma in a 17-year-old male presenting with recurrent epistaxis and nasal obstruction. Preoperative imaging revealed- A well-defined enhancing vascular lesion epicentered in right pterygomaxillary fissure and sphenopalatine foramen. The lesion measures approximately 4 x 3 x 3 cm in greatest dimensions. Superiorly there is erosion of floor of right sphenoid sinus with focal extension Inferiorly it extends in nasopharynx and right nasal cavity and abuts right middle and inferior turbinates. No intra-orbital or intracranial extension noticed.
Patient underwent preoperative embolization of the right common carotid artery. 6 vessel cerebral angiogram was performed. The hyper vascular blush seen in the nasopharynx consistent with the diagnosis of JNA. It was primarily supplied by bilateral internal maxillary artery branches. Supplying arteries were embolized with cyanoacrylate glue.
Patient was placed under general anesthesia with hypotensive technique. Nasal cavity was decongested with adrenaline-soaked patties. 0-degree and 30-degree rigid endoscopes were used throughout the procedure.”

Under the stereotactic guidance- Anterior and post ethmoidectomy & maxillary antrostomy was performed.

The antrostomy was then widened circumferentially using the microdebrider until the maxillary sinus mucosa could be easily visualized.

The  middle turbinate was resected above the tumor and superior gently off of the tumor.

Tumor was bluntly distracted slowly releasing areas of adhesions using a mixture of bipolar cautery and microdebrider from the left lateral and posterior wall from the face of the sphenoid sinus.

The SPA was ligated with a hemoclip. Bipolar cautery was used to remove the final attachment and the tumor was freed. Once tumor was freed from all attachments except for the origin it was placed into the oropharynx. Careful blunt dissection was used to locate the neurovascular structures to check for any more tumor.

The tumor was removed through the oral cavity. The nasal cavity was packed with thrombin soaked gelfoam followed by surgiflo. Merocel was placed in right nares.

The tumor specimen itself measures approximately 3x 4 cm in diameter as seen here there were no complications during the procedure and the estimated blood loss was about 15 CC’s the patient is admitted overnight for post-operative monitoring and deemed stable for discharge on postoperative day one. Histopathology confirmed JNA,

To date the patient has no evidence of recurrence

Tips and tricks
Always evaluate the extent of the tumor on both CT and MRI. Identify feeding vessels and consider preoperative embolization if feasible.
Perform posterior septectomy and extended medial maxillectomy when needed for optimal exposure. Don’t hesitate to switch to a 30- or 45-degree scope for better visualization of lateral extensions.
Devitalize the tumor early by cauterizing or clipping the feeding branches from the internal maxillary artery. LigaSure or Bipolar cautery can significantly reduce intraoperative bleeding.
Lastly, Maintain hypotensive anesthesia and use local vasoconstrictors. Have adequate suction ready and use hemostatic agents like Surgicel or Floseal as needed.
Thank you

Endoscopic Endonasal Resection of Juvenile Nasopharyngeal Angiofibroma This video demonstrate the complete endoscopic removal of Stage II JNA using a bi-nostril four handed technique with image guidance
Radiologically confirmed hyper vascular mass in the nasopharynx suggestive of JNA.
Intracranial extension with cavernous sinus invasion Lack of surgical facilities or expertise
General Anesthesia with Orotracheal intubation Head in reverse Tredelenbeurg position to achieve hypotensive anesthesia Nasal endoscopes- 0/30 & 70 Navigation system Monopolar and bipolar suction cautery
Informed consent with counselling for possible excessive bleeding/ intensive care observation and recurrence risk Haematological workup including coagulation profile Contrast enhanced CT and MRI to evaluate the tumor extent Interventional radiology support for preoperative embolization with 24-48 hours of surgery
Nasopharynx & paranasal sinuses Sphenopalatine foramen & its contents Vidian canal Pterygopalatine Fossa and its contents Infratemporal Fossa & its contents Skull base landmarks
Advantages Minimal invasive technique without any external scar Post embolization – minimal risk of haemorrhage Better visualisation of tumor with angled scopes Shorter hospital stay Faster recovery Disadvantage Learning curve Limited access to extensive lesion High dependency on navigation system Risk of incomplete resection
Intraoperative bleeding Skull base breach Orbital injury Tumor recurrence
All the authors declare no conflict of interest related to this surgical video submission.
We thank the Department of Interventional Radiology Team at Arkansas Children’s Hospital for their collaboration and constant support for such cases.
1. Gullane, P.J. ∙ Davidson, J. ∙ O'Dwyer, Juvenile angiofibroma: A review of the literature and a case series report Laryngoscope. 1992; 102:928-933 2. Andrews, J.C. ∙ Fisch, U. ∙ Valavanis, The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach, Laryngoscope. 1989; 99:429-437 3. Tosun, F. ∙ Ozer, C. ∙ Gerek, Surgical approaches for nasopharyngeal angiofibromas: Comparative analysis and current trends J Craniofac Surg. 2006; 1:15-20 4. Wormald, P.J. ∙ Van Hasselt, A. Endoscopic removal of juvenile angiofibromas, Otolaryngol Head Neck Surg. 2003; 129:684-691

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