Patient summary
A 14-year-old male weighing 45 kilograms presented to our otolaryngology clinic with recurrent epistaxis for the past 8 months. Epistaxis occurred frequently during sleep, flowing down the nasal cavity or into the oral cavity. He was forced to breathe through the mouth due to nasal obstruction. The result of incisional biopsy undertaken 2 months ago in the outpatient clinic showed juvenile angiofibromas. And then symptoms including bleeding and nasal congestion exacerbated. For the last 2 weeks he suffered from breathing difficulties while sleeping and felt pressure in the right ear. The 14-year-old’s general mental and physical health also deteriorated. He lost about 4kg in 2 months, and became depressed and socially withdrawn. Initially he refused treatment and was admitted to a local hospital for suicide attempt.
Examination and treatment
Anterior rhinoscopy revealed a reddish mass blocking nasal passage on the right side. Complete blood count was notable for hemoglobin (Hb) of 50g/L, hematocrit of 17.7%. Transfusion of 4 units of packed red blood cells (PRBC) was required due to severe anemia. Imaging examinations showed a tumor measuring 7.3*5.7*6.5 cm in the right nasal cavity and exhibiting heterogeneous enhancement, posterior invasion to the nasopharynx, as well as the pterygopalatine fossa (PPF) and the infratemporal fossa (ITF). (Fig. 1). There were multiple bony destructions, but no intracranial extension. The patient was diagnosed with JNA, and the angiofibroma was classified as Radkowski stage ⅡC, Önerci stage III. The NSF-COR classification indicated that the tumor was stage NSF2R and recommended combined endoscopic and open surgical approaches. These approaches were adopted in this case.
Because of the advanced nature of the tumor, angiography employs superselective catheterization to adequately examine the tumor's blood supply and identify collateral vessels that may be dangerous for intracranial circulation. The vascular distribution to the tumor is provided by the internal maxillary branches of bilateral external carotid arteries (ECA), as well as a small contribution from the right internal carotid artery (ICA). Superselective bilateral embolization of the internal maxillary arteries (IMA) was performed to minimize collateral blood supply. Absorbable gel foam particles were used as embolic agent. However, both coils and gel foam particles were used for the right internal maxillary artery due to an arteriovenous fistula. After embolization, there were still a few arterial branches in the nasopharynx feeding the posterior part of the tumor. These vessels that originated from the ICA were not embolized. (Fig. 2)
The tumor was resected via sublabial Caldwell–Luc maxillectomy combine with an endoscopic resection under general anesthesia 48 hours after the embolization. Intraoral resection for tumor removal was performed, followed by an endonasal endoscopic resection and careful exploration of the Vidian canal and pterygoid process. No CT-guided navigation was utilized during the whole procedure.
Caldwell-Luc maxillectomy incision was conducted to open the anterior wall of the right maxillary sinus and expose the capsuled tumor. Oblation plasma technology was used to debulk the tumor and make a surgical space. The tumor capsule was separated from surrounding tissues with coblation enabling tumors to be removed in blocks from maxillary sinus, PPF and ITF. The right maxillary artery was identified and coagulated using bipolar forceps. Subsequently, only the mass in the nasal cavity was left. The tumor was debulked with Medtronic ENT power system, transected above the choanal and finally amputated from the nasopharynx mucosa. Attention was paid to protect the eustachian. Once the tumor was completely removed, it was pushed backward to the posterior nasal cavity and pulled out transorally. Afterwards the pterygoid process was drilled off, enabling probing of the Vidian canal. Intraoperative observation revealed the tumor related to the Vidian canal artery, suggesting that the tumor was also fed by this artery, and that most of the intraoperative bleeding came from the region at the posterior nasal cavity. The Vidian canal artery was coagulated using the coblator.
Total blood loss was 2000 mL. Eight units of PRBC were transfused in total during the 9-hour surgery.
Postoperative histopathology confirmed the diagnosis of JNA. After the procedure, the patient lost sensation in part of the right superior gum, which improved after removing the Bismuth iodoform paraffin paste (BIPP). The 14-year-old patient didn’t complain any discomfort during nasal endoscopy and nasal irrigation treatment. He was discharged from the hospital 10 days after operation. CT examination was carried out before discharge. Until now, the patient feels good and refuses to follow up.
The institutional review board approved publication of this case report and informed consent has been obtained from the patient’s guardians, the case report and related images can be published.