The Combined Subtemporal - Transfacial Approach 1 Supplemental Results 2 3 Case 1 (Fig 3a) 4 5 An eighteen-year-old male patient presented with a history of nasal obstruction and allergy-like 6 symptoms, progressing during the past four years, and with right hearing loss and headaches 7 during the past one year. He had jaw numbness. The pre-operative MRI showed a massive 8 JNA of the involving the paranasal sinuses and nasopharynx, with intracranial extension 9 through the skull base to adjoin the right cavernous sinus, Meckel's cave, and medial temporal 10 lobe. There was compression of the right orbit as well as erosion of the clivus. Angiography 11 revealed that the tumor derived blood supply from both the right internal and external carotid 12 arteries. Resection was performed using two procedures staged two days apart. The day after 13 embolization, a subtemporal approach was performed to separate the tumor from the 14 intracranial cavity and vascular supply. This approach allowed resection of the portion of the 15 tumor that was compressing the right temporal lobe, and divorced the tumor from its right 16 internal carotid artery blood supply. In the second stage, an endoscopic transnasal resection 17 was employed to successfully remove the remainder of the tumor. Post-operative MRI 18 revealed only a slight amount of residual disease within the cavernous sinus, which has 19 remained stable for 16 months. 20 21 Case 2 (Fig 3b) 22 23 An 11 year old boy presented with recurrent, severe epistaxis, left facial numbness along the 24 jaw, and a left serous middle ear effusion. MRI revealed a JNA that eroded through the skull 25 base through the foramen ovale. It also extended inferiorly into the parapharyngeal space (not 26 shown in included image). After embolization, a subtemporal approach was performed and the 27 tumor was separated from the intracranial cavity. An endoscopic transfacial approach was then 28 attempted to resect the rest of the disease. However, bleeding remained extensive, particularly 29 from the inferior aspect of the tumor, and only ~50% of the tumor could be removed. Four days 30 later, a midface degloving procedure was performed to complete a subtotal resection. In 31 contrast to all of the other procedures, where the only residual disease was within the 1 The Combined Subtemporal - Transfacial Approach 32 cavernous sinus, this tumor was based too inferiorly in the neck to be completely resected. 33 Thus, two foci of residual tumor were left behind in this sub-total resection, at the cavernous 34 sinus and near the carotid bifurcation. 35 36 Both areas recurred eight months postoperatively and a repeat midface degloving was 37 performed. Again, only a subtotal resection could be performed, with both foci of residual 38 disease remaining. The residual in the neck regrew rapidly and at 18 months post-operatively 39 a transcervical approach was performed to ligate the external carotid artery and completely 40 resect the lower focus of residual tumor. With only one focus of residual disease remaining, the 41 ongoing plan is to manage the expected recurrences in the central sinonasal cavity solely with 42 endoscopic debridement (in this now 13 year old patient). 43 44 Case 3 (Fig 3c) 45 46 A 13 year old boy presented with recurrent epistaxis and left middle ear effusion. He was found 47 to also have facial numbness in the V2 and V3 distributions. A large JNA with cavernous sinus 48 invasion was found. After embolization, a combined subtemporal approach and open 49 transfacial resection using a lateral rhinotomy approach was performed. A near total resection 50 was achieved, with only minimal residual at the cavernous sinus. This recurred on an annual 51 basis and was managed with annual endoscopic debridements as an outpatient. Once the 52 child reached 19 years old, growth of the tumor remnant spontaneously arrested as shown by 53 MRI. He is now undergoing interval surveillance imaging and observation. 54 55 Case 4 (Fig 3d) 56 A 21 year old male was diagnosed with a Stage IVb JNA at an outside facility, and underwent 57 1 past craniotomy by neurosurgery and later, 5 partial endoscopic resections by 58 otolaryngology. The patient presented to our center after experiencing a life threatening 59 nosebleed while driving, to a Hgb of 5 on admission. We then performed a staged cranio- 60 orbito-zygomatic lateral approach followed by an open transfacial approach 6 days apart. A 61 near total resection was again achieved without any concerning long term sequelae. He had 62 one episode of severe epistaxis 15 months postoperatively, and recurrent JNA was found in 2 The Combined Subtemporal - Transfacial Approach 63 the region of the left pterygopalatine fossa that was managed endoscopically with minimal 64 blood loss. 65 66 3