A 26-year-old man presented with a 1-month history of nasal obstruction and left-sided epistaxis. The patient had no prior history of nasal trauma or sinusitis. Nasal saline, steroid sprays, and oral antihistamines did not relieve his obstructive symptoms. On endoscopic examination, the patient was found to have a fibrous-appearing mass filling the left nasal vault. A maxillofacial computed tomography (CT) scan (Fig. 3.1) revealed a large mass centered in the left nasal cavity extending to the ipsilateral paranasal sinuses, and central skull base. Heterogeneous uptake of gadolinium on magnetic resonance imaging (MRI) suggested a hypervascular mass (Fig. 3.2d–f).
Fig. 3.1 Maxillofacial CT scan. (a) Axial soft tissue window CT showed a 7.9 × 3.7 cm mass extending from nasal cavity into the nasopharynx, pterygopalatine fossa, and left maxillary sinus. (b) Coronal bone window CT demonstrated erosion of the posterior and medial maxillary sinus wall as well as destruction of the left pterygoid plates. (c) Sagittal bone window CT showed no obvious invasion of the skull base.
Fig. 3.2 Brain MRI with and without contrast. Precontrast T1-weighted MRI in (a) axial, (b) sagittal, and (c) coronal views showed lobulated mass measuring approximately 8.5 × 5.0 × 4.5 cm. The mass extended from the left nasal cavity and nasopharynx into the left posterior and medial maxillary sinus walls and left pterygoid plates, with extension into the left maxillary sinus, pterygopalatine fossa, and infratemporal fossa. Postcontrast T1-weighted MRI scans in the same views (d–f) demonstrated heterogeneous enhancement with irregular central hypointensity.
The differential diagnosis for a unilateral sinonasal mass in a young adult causing nasal obstruction and epistaxis is wide. The primary differential includes inverted papilloma, juvenile nasopharyngeal angiofibroma (JNA), antrochoanal polyp, hemangiopericytoma, and lymphoma. The patient’s associated epistaxis, and imaging findings of a locally invasive and hypervascular mass, makes JNA the most likely diagnosis, although hemangiopericytoma cannot be ruled out.
JNAs are the most common benign tumor of the nasopharynx, and are almost exclusively seen in adolescent males. Although some controversy remains over the exact site of origin, these lesions tend to arise from the sphenopalatine foramen and spread anteriorly into the nasal cavity and posteriorly into the nasopharynx. They may spread laterally into pterygopalatine fossa (PPF), through the pterygomaxillary fissure into the infratemporal fossa (ITF), or posteriorly into the bony pterygoid plates. Less commonly, these locally aggressive tumors can extend, via the PPF and inferior orbital fissure into the orbit, or via the pterygoid plates into the middle cranial fossa. If the sphenoid sinus walls are eroded, the cavernous sinus and pituitary glands may become involved as well. Clinically, these tumors present with unilateral nasal obstruction and epistaxis, and a smooth, lobulated red or pale blue hypervascular lesion in the nasal vault is usually seen on endoscopic examination. CT scans often show bowing of the posterior maxillary sinus wall (Holman–Miller sign), widening of the PPF, bony erosion or remodeling of the skull base, and deviation of the nasal septal wall. On MRI, JNAs enhance brightly, often showing obvious flow voids on T2. Both characteristics are indicative of tumor hypervascularity.
Given its typical clinical presentation and radiographic findings, biopsies of JNA are controversial because of the risk of severe hemorrhage. The general consensus is that biopsy is unnecessary and too risky when suspicion is high, but some surgeons continue to advocate for pretreatment biopsy to avoid misdiagnosis of sinonasal malignancy in the setting of atypical clinical presentation. Though rare, solitary fibrous tumor/hemangiopericytoma (SFT) must be considered in the differential diagnosis due to a high distant metastasis rate of around 15%. These tumors are most commonly seen in middle-aged patients without significant gender discrepancy. One clue that would differentiate JNA from SFT is that the latter appears more fibrous than erythematous on endoscopy.
Our patient had an atypical presentation for JNA. First, the patient’s age was above the upper limit of what is commonly seen with JNA, which has a reported average age of 15 years. Additionally, his initial endoscopic examination finding of a fibrous intranasal mass which did not fit the classic smooth, red or pale blue vascular appearance of JNA. As such, this patient underwent a nasal mass biopsy. He did not have any hemorrhagic complications, and the results showed vascular proliferation with thin-walled blood vessels, along with a fibro-collagenous stroma. No mitotic activity or cytologic atypia was seen. These findings were all suggestive of a diagnosis of JNA.
Once diagnosed, JNAs are most often treated with surgical excision, with the goal of complete resection with minimal cosmetic deficit. With that goal in mind, the anatomy of the tumor was reviewed in detail. The tumor occupied the left nasal cavity and nasopharynx, and extended into the left maxillary sinus, PPF, and ITF (Fig. 3.2). On the CT bone windows (Fig. 3.1b, c), the mass remodeled and partially destroyed the left pterygoid plates, as well as the posterior and medial walls of the maxillary sinus. There was, however, no sign of erosion of the middle fossa floor.
The ITF is the anatomical space under the middle fossa floor (Fig. 3.3). Anteriorly, it is limited by the lateral part of the posterior wall of the maxillary sinus (PWMS), and its medial wall is formed by the lateral pterygoid plate. The ITF contains the pterygoid muscles, the maxillary artery, the pterygoid venous plexus, and branches of V3. Via the pterygomaxillary fissure, the ITF communicates with the PPF which is medial to it (Fig. 3.4).
Fig. 3.3 Infratemporal and pterygopalatine fossa (IFT and PPF). Dissection of the posterior limit of the nasal cavity. The orbit has been resected up to the apex, and the walls of the maxillary sinus, the nasal septum, and part of the palate have been removed. The PPF, containing the pterygopalatine ganglion (*) and the IFT, with structures related to the posterior wall of the maxillary sinus, have been exposed. The sphenoid sinus has been opened. The anterior and middle fossae have been exposed, and part of the tongue, the ramus, and the body of the mandible have been removed. At the level of the choanae, the lateral relationship of the nasal cavity includes the PPF and IFT. A., artery; Cav., cavernous; CN, cranial nerve; Fiss., fissure; Lat., lateral; M., muscle; Mand., mandibular; Max., maxillary; Med., medial; Proc., process; Pteryg., pterygoid; Sphen., sphenoidal. (Reproduced from Stamm A, ed. Transnasal Endoscopic Skull Base and Brain Surgery. 1st ed. Thieme; 2011.)
Fig. 3.4 Cranial base, coronal view. The posterior wall of the right maxillary sinus was removed, exposing the infratemporal fossa laterally, and the pterygopalatine fossa medially. The white dashed line is the demarcation of the two. The maxillary division of the trigeminal nerve (V2) is exiting the sphenoid bone through the foramen rotundum and running in the superior wall of the maxillary sinus as the infraorbital nerve. The maxillary artery loops in the infratemporal fossa, entering the pterygopalatine fossa and giving its terminal branches. The yellow dotted line demonstrates the relationship of the axis of the middle turbinate tail and the sphenopalatine foramen. A., artery; Div, division; Ethm., ethmoidal; Inf., inferior; Infraorb., infraorbital; Max./Maxil., maxillary; N., nerve; Sphenop., sphenopalatine; Trigem., trigeminal; Turb., turbinate.
The PPF is an inverted pyramid-shaped space behind the medial portion of the PWMS. It is bordered medially by the palatine bone and posteriorly by the pterygoid plate. Its apex, which is its inferior-most point, is the greater palatine canal. The contents of the PPF include the pterygopalatine ganglion, V2, the infraorbital and vidian nerves (Fig. 3.5).
Fig. 3.5 Cadaveric dissection in the midsagittal plane, medial view of the left pterygopalatine fossa (PPF). A segment of the cavernous part of the internal carotid artery has been resected to expose the nerves in the cavernous sinus. The PPF has been opened and most of the pterygoid process removed. The auditory tube has been removed. (Reproduced with permission from Peris-Celda M, Martinez-Soriano F, Rhoton AL Jr., eds. Rhoton’s Atlas of Head Neck and Brain. 2D and 3D Images. 1st ed. Thieme; 2017.)
Multiple staging systems have been developed based on the relationship between JNA and its surrounding anatomical structures. One of the early JNA staging systems proposed by Sessions was based on the degree of local tumor extension rather than JNA tumor size alone. A revised system, currently more popular, was proposed by Radkowski, and it distinguishes between isolated skull base erosion versus erosion with extensive intracranial and/or dural spread. Both JNA staging systems aimed to guide the amount of operative exposure necessary for full surgical resection, as well as the need for radiation therapy in unresectable tumors (Table 3.1).
Sessions et al 1981a
Radkowski et al 1996b
Full occupation of the pterygomaxillary fossa, displacing the posterior wall of the maxillary antrum, lateral/anterior displacement of maxillary artery branches, with or without erosion of orbital bones
As JNAs are believed to originate from the sphenopalatine foramen, all surgical approaches should be able to expose this anatomical landmark. Anterior and medial exposure into the nasopharynx and nasal cavity is also required, as these structures become involved early in the natural course of tumor spread. Lateral exposure is often necessary, as JNA extension into the PPF is reported between 26 and 100%, with further extension into the ITF reported between 20 and 54%. The incidence of intracranial involvement is reported at 10 to 20%, but extent of surgical exposure for these tumors depends on the level of invasion and involvement of intracranial structures. The significant morbidity associated with resection of tumors involving the internal carotid artery (ICA), optic nerve, or oculomotor nerves, makes partial resection with adjuvant radiotherapy worthy of consideration.
Various open approaches have been developed to achieve adequate surgical exposure to facilitate total resection, while limiting intraoperative blood loss. A transpalatal approach can be used to resect tumors limited to the nasopharynx and nasal cavity, while a combined transpalatal and transantral approach can resect tumors that extend laterally into the PPF and/or maxillary sinuses. The combined transpalatal–transantral approach was found to effectively treat tumors of Radkowski’s stage IB or less with minimal recurrence. Risks of transpalatal tumor resection include palate dehiscence and oronasal fistula. A combined transpalatal–transantral approach presents additional risks of facial deformity and paresthesia due to infraorbital nerve injury.
Lateral rhinotomy with extension to the upper lip mucosa came into favor for resection of more extensive tumors. This approach provides access to medial structures such as the nasal cavity and nasopharynx, as well as to lateral structures such as the maxillary sinus, PPF, pterygomaxillary fossa, and infratemporal region. Besides an unattractive nasal scar, other risks of the lateral rhinotomy include facial paresthesia in the infraorbital nerve distribution and lacrimal apparatus injury from the superior incision site.
A midfacial degloving technique was developed to address the cosmetic deformity problem presented by lateral rhinotomy; its intranasal and sublabial incisions provided excellent surgical exposure while eliminating external scarring. The initial incision is made in the gingivolabial and gingivobuccal sulci and runs laterally to the maxillary tuberosities (Fig. 3.6). A transfixion incision is made along the nasal septum and is then extended around the limen vestibulae. The soft tissue of the nose is degloved up to the level of the infraorbital foramen, exposing the bony structures of the nose and maxilla below. The midfacial degloving approach provides access to the nasal cavity, nasopharynx, PPF, pterygomaxillary space, ITF, maxillary and sphenoid sinuses, cheek, and orbit. Risks of the midfacial degloving technique include facial paresthesia, nasal crusting, oroantral fistula, and epiphora.
Fig. 3.6 Midface degloving. Midface degloving is accomplished by a combination of bilateral intranasal (1), intercartilaginous (2), septal transfixion (3), and sublabial (4) incisions followed by subperiosteal elevation of all soft tissue of the midface (5). (Reproduced with permission from Casson PR, Bonanno PC, Converse JM. The midfacial degloving procedure. Plast Reconstr Surg 1974;53:102–103.)
If the tumor has significant involvement of the ITF and medial extension into the cavernous sinus, a LeFort I osteotomy approach can provide better exposure (Fig. 3.7). A fracture is made from the pyriform aperture to the pterygoid plates to detach the maxilla from the cranial base, thus allowing for exposure of the central skull base. This approach also involves resection of the posterior maxillary sinus wall, allowing for good visualization of the vascular supply to maximize hemostasis. Risks of this approach include stunted vertical maxillary growth in developing children, dental denervation, aseptic necrosis of the maxilla, cerebrospinal fluid (CSF) leak, and extraocular nerve palsy.
For tumors involving the ITF, middle cranial fossa, and lateral cavernous sinuses, an ITF approach may be used. Given the wide exposure, the internal maxillary artery is identified and ligated early on for improved intraoperative hemostasis. Cosmetically, this approach minimizes facial scarring, and does not risk midface deformity. However, this approach is unable to reach tumors medial to the abducens nerve in the cavernous sinus, so additional medial approaches must be supplemented for those tumors.
Recent advances in skill and technology have made it possible to access the PPF and beyond with endoscopic technique and made endoscopy a viable option for JNA surgery (Fig. 3.8). Advantages to endoscopic JNA resection include decreased intraoperative time and shorter postoperative hospitalization stays. With endoscopic surgery, there is of course no skin incision, but it also minimizes bone removal, thus avoiding complications associated with open approaches including epiphora, trismus, and possible facial growth deformity. JNAs characteristically have a well-defined capsule which creates an optimal surgical plane for endoscopic dissection.
Fig. 3.8 The endoscopic transpterygoid approach (right side with a 0-degree endoscope). (a) After exposure of pterygopalatine fossa and greater palatine nerve. (b) Note the vidian canal opening with lateral mobilization of pterygopalatine fossa. (c, d) Vidian and greater palatine nerve preserved after the pterygoid base and the medial pterygoid plate were partially removed. (Reproduced from Pinheiro-Neto C, Fernandez-Miranda J, Prevedello D, et al. Transposition of the pterygopalatine fossa during endoscopic endonasal transpterygoid approaches. J Neurol Surg B Skull Base 2013; 74(5):266–270.)
Major challenges of endoscopic sinus surgery include hemorrhage control, small operative field, and limited visualization. Intraoperative hemorrhage control can be addressed with the use of preoperative embolization, which can reduce intraoperative blood loss as much as 70%. The most commonly involved vessels include branches of the internal maxillary artery (e.g., sphenopalatine artery and vidian artery), ascending pharyngeal artery, mandibular artery, and facial artery, which can individually be targeted with superselective catherization techniques. Further improvement to control intraoperative blood loss came in the form of coblation technology. The coblator acts at a low temperature (60–70°C), allowing for it to ablate tumor tissue with less damage to adjacent tissues, while concurrently sealing feeding blood vessels. Recent studies have demonstrated the superiority of coblator-assisted endoscopic JNA resection in decreasing blood loss as well as operative time, although studies are currently limited to tumors of Radkowski stage IIC or less.
Hemostasis and surgical access are further enhanced by switching from one-hand endoscopy to a two-surgeon technique. As first described, this technique involved removal of the bony septum and incision of the bilateral mucosal surfaces to allow for additional instruments to be placed via the nonoperative nostril into the operative field. The second surgeon retracted the tumor into the opposite nasal cavity to create space for the first surgeon to resect the tumor, and/or to provide additional suctioning in the event of intraoperative hemorrhage. A variation of this technique was later developed that used a more posterior transseptal perforation, which provides a wider operative field and decreases the need for piecemeal resection.
With the aforementioned enhancements, endoscopic techniques started to gain acceptance for use on JNAs. Although exposure is still restricted compared to open approaches, the use of an endoscope brings better lighting to the field and provides a magnified, multiangled view of the mass and its surrounding anatomy. Additionally, with integrated image-guidance technologies, the location of vital structures, such as the orbital apex, optic nerve, cavernous sinus, and carotid artery can be tracked. Recent studies showed equal efficacy between endoscopic and open approaches, even for high-stage tumors (up to IIIA) and with that, endoscopic techniques may well be applicable now for all JNAs except those with extensive intracranial extension.
The tumor in our patient was classified as Radkowski stage IIC, involving the nasal cavity, nasopharynx, maxillary, ethmoid and sphenoid sinuses, as well as the PPF, ITF, and orbital apex. Buoyed by the studies mentioned above which showed equivalency in open and endoscopic techniques for higher-stage tumors, we chose the endoscopic approach with the goal of total resection of his large tumor.
Prior to tumor resection, the patient underwent a cerebral angiogram with preoperative embolization. On cerebral angiogram (Fig. 3.9), significant blood supply was seen originating from the left maxillary artery. The left maxillary artery was subsequently embolized using Embosphere particles ranging from 300 to 500 µm and 500 to 700 µm, with immediate postembolization decrease in vascularization. The patient underwent endoscopic resection of JNA involving the nasal cavities, paranasal sinuses, PPF, ITF, orbital apex, and pterygoid plates (see The Three Approach Elements).
Fig. 3.9 Cerebral angiogram and embolization of sphenopalatine artery. (a) Sagittal view of the patient’s pre-embolization cerebral angiogram showed a rich vascular network feeding the JNA, most prominently from the left maxillary artery. (b-d) The left maxillary artery was successfully embolized with embosphere particles with immediate decrease in vascularization.
The patient was placed supine and draped in the standard fashion. Nasal endoscopy on the left revealed a large nasal mass obstructing the nasal vestibule. The Coblator surgical system was used to begin piecemeal resection of this massive JNA. Ablation of approximately the anterior one-third of tumor was performed with a combination of coblation, monopolar cauterization, and blunt and sharp dissection (Fig. 3.10). Similar techniques were utilized to bisect the tumor horizontally and truncate the tumor in the nasal cavity from the maxillary, PPF, and ITF components. This was necessary to create space for lateral and superior dissection of the tumor. Bleeders from the ethmoidal vessels were addressed using monopolar cautery. Using similar instrumentation and techniques, the inferior portion of the tumor was resected to the nasopharynx, and sphenoid face. The posterior and inferior portion of the tumor was removed transorally given its size (Fig. 3.11). The tumor was encapsulated over the posterior portion of the mass.
Next, an endoscopic maxillectomy, along with a maxillary antrostomy, superior ethmoidectomy, and frontal sinusotomy was performed. The JNA was dissected out of the maxillary sinus using blunt dissection and placing cottonoids in the sinus to deliver this tumor component into the nasal cavity (Fig. 3.12). The medial PPF component in continuity to the superior ethmoid and maxillary components of the tumor was dissected using coblation and delivered through the nasal cavity (Fig. 3.13). Using Kerrison rongeurs, the anterior wall of the PPF (equivalent to the medial portion of PWMS) was removed next, and the PPF and ITF component of the tumor abutting the orbital apex was dissected with sharp and blunt technique from the anterior fat and posterior neural compartments. Suction monopolar on a low setting, as well as blunt dissection under angled endoscopic visualization, were used to accomplish this task (Fig. 3.14).
Fig. 3.14 Pterygopalatine fossa (PPF) and infratemporal fossa (ITF) portion of tumor dissection. Anterior wall of PPF was removed (not shown) to expose the PPF and ITF tumor components. (a) Suction monopolar and blunt dissection were used to carry out the dissection of tumor abutting the orbital apex (b–d).
Using two-hand technique through the ipsilateral nostril, bipolar cautery, as well as a suction cautery, the ITF mass was resected. Multiple clips were placed on feeders to the mass from the internal maxillary artery, which included the posterior superior alveolar artery, descending palatine arteries as well as sphenopalatine arteries. Once the ITF of the mass was resected, a margin was obtained and sent for pathologic examination. Large pieces of Gelfoam and Surgiflo were placed on the PWMS, PPF, and sphenoid regions.
A postoperative MRI was obtained, and a tumor residual involving the orbital apex, lateral recess of the sphenoid sinus, and ITF was discovered (Fig. 3.15a–c). We concluded that inadequate exposure of the sphenoid sinus and lateral sphenoid wall was the cause of the problem, and as such, the patient was scheduled for a repeat operative procedure 5 days after the first surgery. A wide sphenoidotomy with extensive exenteration of the sphenoid sinus was performed. Kerrison rongeurs were used to thoroughly expose the lateral recess of the sphenoid sinus. Two small dumbbell-shaped tumor components were resected from the lateral recess of the sphenoid sinus interdigitating with the pterygoid plates, medial contents of the PPF, and the ITF. The vidian nerve and V2 were sacrificed, while V3 was spared, to achieve a complete resection. Cautery and clips were applied for hemostasis. Hemostatic agents were placed in the surgical cavity as this second-stage operation concluded.
Fig. 3.15 Postoperative MRI scan (a–c) Immediate postoperative MRI scans and (d–f) revision postoperative MRI scans. (a) Coronal, (b) sagittal, and (c) axial T1-weighted postcontrast MRI images demonstrated residual tumor in the lateral sphenoid sinus, infratemporal fossa, and orbital apex. (d–f) Comparable MRI images obtained after revision surgery demonstrated complete tumor removal and normal postoperative changes.
The use of two-surgeon, four-hand technique is critical for success in the resection of such a complex and extensive JNA. It improves hemostasis, maximizes surgical access, and minimizes operative time. While as a regular operative partner in endoscopic procedures the neurosurgeon may seem to have no role in this tumor resection, she or he may be an appropriate assistant in the two-surgeon paradigm.
Although this operation turned into a staged operation, routine staging of these procedures can be avoided by aggressively widening the surgical access from the beginning. A sphenoidotomy at the first operation would have likely eliminated the need for this patient’s second operation.
The patient returned to clinic for his first follow-up visit within 2 weeks of surgery. Left-sided numbness along the cranial nerve V2 distribution was noted on examination, as expected after intraoperative nerve sacrifice. No trigeminal nerve (V3) deficit or visual changes were observed. The patient also denied postoperative epistaxis, and his residual nasal congestion and crusting were eventually resolved with consistent nasal saline spray use. An MRI after surgery demonstrated expected postoperative changes without residual tumor (Fig. 3.15d–f). The patient had consistent follow-up in clinic, and a 2-year follow-up MRI showed no evidence of residual or recurrent disease and resolving postoperative changes.
Close postoperative follow-up with imaging can help detect residual disease and/or recurrence. Early postoperative CT imaging of patients with Radkowski stage III tumors obtained within 5 days of surgery has a specificity of 83%, indicating a high reliability in detecting true residual disease. However, imaging obtained any earlier than 72 hours may have limited value due to confounding by postoperative inflammatory changes. Patients who are found to have residual disease on imaging must undergo careful evaluation regarding further management. Small tumor remnants in asymptomatic patients may be observed with serial imaging, as they have been shown to stabilize or regress spontaneously.
Although the exact timing of additional postoperative imaging is not clear, one study done on endoscopically resected JNAs recommends CT scans 6 to 12 weeks after resection, followed by a second CT scan performed 4 to 6 months postoperatively to identify recurrent tumor. Overall JNA recurrence rates range from 22 to 37.5%, although the exact rate is highly dependent on preoperative tumor staging, rate of growth, and degree of surgical resection. Higher recurrence rates are seen in JNAs that involve the ITF, sphenoid sinus, pterygoid plates and clivus, cavernous sinus, foramen lacerum, and anterior fossa. Proper identification, meticulous subperiosteal dissection, and drilling of these areas may successfully prevent future recurrence.
Hussam Abou-Al-Shaar, Wayne D. Hsueh, Jean Anderson Eloy, and James K. Liu
The authors of the previous section have eloquently described their skillful use of endonasal endoscopic techniques to remove a large JNA, avoiding transfacial surgery and its multiple risks. However, despite the advancement of technique, improvement of equipment, and accumulation of experience, endoscopic endonasal approaches still have inherent limitations. One such limitation is insufficient access and exposure of large JNA with multicompartmental extension to lateral locations in the skull base. For such large tumors, which extends well into the ITF, possibly reaching its lateral extreme, additional exposure can be obtained if multiple endoscopic corridors are utilized in combination with each other. We discuss the application of such a strategy in this section.
An otherwise healthy 13-year-old boy presented to our service complaining of nasal obstruction for the past 6 months. His neurological examination was unremarkable. Endoscopic examination of both nasal cavities demonstrated a nasopharyngeal mass extending into the right nasal cavity. CT scan and MRI depicted a large mass in the nasopharynx extending bilaterally into both nasal cavities, bilateral sphenoid sinuses, right PPF, ITF, and right inferior orbital fissure. Erosion was also evident in the anterior clivus and pterygoid plate (Fig. 3.16).
The constellation of symptoms including nasal obstruction, epistaxis, and a nasopharyngeal mass in an adolescent male should raise the suspicion for the diagnosis of JNA. These patients should undergo further evaluation and definitive surgical management by a multidisciplinary skull base team. All patients with JNA should receive a high-resolution CT and MRI scans in order to evaluate the bony architecture, delineate the tumor location and extension, and plan the surgical approach.
For reasons already discussed in the previous section, biopsy is usually unnecessary in the setting of a typical presentation, but if an atypical history or presentation warrants a tissue diagnosis, the procedure should be performed in an operating room under general anesthesia due to the high vascularity of the tumor and the potential of life-threatening bleeding. The various staging systems for JNA have also been discussed, and the most commonly utilized systems include the Radkowski, Andrews–Fisch, and Snyderman (UPMC) staging systems, among others. These staging systems classify JNAs primarily based on their location and tumor extension to aid surgeons in planning the surgical approach.
Since JNA is a hypervascular tumor, embolization of the blood supply prior to surgical resection is commonly recommended (Fig. 3.17). In our practice routine, the angiogram and endovascular procedure is done within 24 to 48 hours before surgery to prevent revascularization of the tumor, which can take place rapidly. We utilize polyvinyl alcohol particles (diameter between 150 and 200 µm) to embolize the feeders, and have found that this makes intraoperative blood loss much more manageable during tumor resection. The primary arterial blood supply of JNA comes from the internal maxillary artery. It is important to rule out the presence of shunts with the ICA or vertebrobasilar system during diagnostic angiography, as embolization in these scenarios could lead to a catastrophic stroke. It is also important to recognize arterial feeding vessels that arise from the ICA circulation, which are usually seen in larger and giant JNAs with intracranial extensions. These ICA feeders typically cannot be safely embolized, making resection much more challenging for these tumors.
Fig. 3.17 Preoperative angiogram. Right internal carotid injection, (a) anteroposterior view, and (b) lateral view showed hypervascularity of the JNA with arterial supply fed from the right cavernous internal carotid artery. (c, d) Right external carotid artery injection and lateral views showed supply from the right internal maxillary arteries. Only the arterial feeders from the external carotid artery could be safely mobilized prior to surgery.
The patient in our case had a typical presentation of JNA. Given his age, as well as his clinical and radiological presentation, we elected to proceed with surgical resection of his lesion with an aim of achieving a gross total resection. As previously mentioned, multiple classification and staging schemes have been developed to aid the surgeon in planning and choosing the optimal surgical approach. Whichever scheme one chooses, it is of paramount importance to study the radiological studies in detail to plan the operative approach accordingly.
The large tumor in our example was centered in the nasopharynx and extended bilaterally into nasal cavities, bilateral sphenoid sinuses, the right PPF, ITF, and right inferior orbital fissure. Given the lateral extension of the tumor, a purely endonasal endoscopic approach (EEA) may not provide adequate exposure to remove the tumor in an efficient manner. Alternatively, open transfacial approaches can potentially be utilized to access the tumor, and these include the lateral rhinotomy, midfacial degloving, Le Fort I osteotomy, transcervical, and facial translocation approaches. However, these approaches are associated with limited visualization, lack of adequate illumination, and many facial cosmetic complications such as facial growth retardation, malocclusion, transfacial scars, and facial nerve injury.
Given the multicompartment location of the tumor, we elected a combined endoscopic graduated multiangle, multicorridor approach to remove his JNA. This combined endoscopic-assisted technique allows one to fully access the tumor in all compartments and obviates the need for open transfacial approaches with their associated complications. We have utilized these approaches in a variety of tumors extending into various compartments with excellent outcomes. Table 3.2 outlines the surgical corridors, approaches, and the locations that can be accessed using these endoscopic graduated multiangle, multicorridor approaches. Since the specific tumor in our case example occupied the nasopharynx on both sides, with significant lateral extension into the right ITF, we elected to use a combined EEA–sublabial (Caldwell–Luc) maxillotomy approach, using both the binostril–endonasal, as well as the sublabial–transmaxillary corridors (Fig. 3.18).
After preoperative embolization, the patient was taken to the operating room where general anesthesia was established with controlled hypotension (mean arterial pressure goals of 60–70 mm Hg). The patient was placed supine with 15 to 20 degrees of reverse Trendelenburg to decrease the central venous pressure. Neurosurgical pledgets (3 × 0.5 in) soaked with 10 mL of 1:1,000 epinephrine were placed inside the nose for decongestion. The root of the middle turbinate, lateral nasal wall, and nasal septum were injected with 1% lidocaine with 1:100,000 epinephrine.
The tumor in the nasopharynx was exposed on both sides, using a binostril endoscopic endonasal approach with a septostomy to allow bimanual binostril access (Fig. 3.19). Tumor in the ITF was exposed using an ipsilateral endoscopic medial maxillotomy (maxillary antrostomy, inferior turbinectomy) from the right side, and also a right-sided sublabial Caldwell–Luc maxillotomy via a sublabial incision (Fig. 3.19b). During the Caldwell–Luc maxillotomy, care was taken to avoid injury to the infraorbital nerve superiorly and teeth roots inferiorly. In total, three portals of access were created (ipsilateral nostril, contralateral nostril, ipsilateral sublabial maxillotomy) to allow multiportal, multicorridor access. The endoscope was used in all three corridors to obtain the best visualization for tumor removal.
Fig. 3.19 Intraoperative endoscopic views. (a) A 30-degree endoscope was placed in the nasal cavity looking laterally to the right through the modified medial maxillotomy corridor. The tumor (T) in the nasal cavity and the tumor in the infratemporal fossa (ITF) was visualized. (b) A right Caldwell–Luc maxillotomy was performed with a sublabial incision. (c) The endoscope was placed into the sublabial maxillotomy to obtain a more direct view of the tumor in the ITF and the lateral maxillary wall. (d–f) The tumor in the sphenoid sinus was carefully peeled off of the skull base underneath the sella from the clival recess (CR). Care was taken not to injure both paraclival internal carotid arteries (ICA). The tumor was initially debulked with a microdebrider until it was small enough to deliver from the nostril (f).
For these far laterally positioned JNAs, addition of the sublabial maxillotomy corridor allows more direct anterior access to the far-lateral portion of the ITF and excellent surgical freedom when mobilizing surgical instruments (Fig. 3.20). In this patient, maximal surgical freedom was achieved while placing the endoscope in the right nostril and introducing instruments through the left nostril and right maxillotomy corridors. We also prefer to use the 30-degree endoscope during skull base procedures because it provides increased angles of visualization in a multidirectional fashion. Rotating the 30-degree endoscope can provide optimal visualization to the maxillary sinus, ITF, anterior skull base, nasal floor, and posterior nasopharynx. Moreover, in the anterior maxillotomy corridor, the 30-degree endoscope can be angled medially to visualize the maxillary antrostomy and nasal septum.
Fig. 3.20 Increased surgical freedom from sublabial Caldwell–Luc maxillotomy. The oval area is the surgical freedom for (a) the endonasal approach and (b) the sublabial approach. In this patient, maximal surgical freedom was achieved while placing the endoscope in the right nostril and introducing instruments through the left nostril and right maxillectomy corridor. (Reproduced from Elhadi A, Almefty K, Mendes G, et al. Comparison of surgical freedom and area of exposure in three endoscopic transmaxillary approaches to the anterolateral cranial base. J Neurol Surg B Skull Base 2014;75(5):346–353.)
The tumor was efficiently removed with a rotation–suction microdebrider. Special care was taken to avoid injury to the orbit and ICA with this device. Thus, it is important for the surgeon to be cognizant of the anatomical compartment they are working in and remain in “safe zones” (nasopharynx, nasal cavity, ITF) when using the microdebrider. Sequential bipolar cautery and early takedown of the internal maxillary artery was paramount for early devascularization of the tumor, even with preoperative embolization. Bimanual microsurgical technique must be used for safely dissecting the tumor away from important neurovascular structures (Fig. 3.19d–f). In this patient, there was no intraoperative CSF leak, as in most cases of extracranial JNAs. However, if an intraoperative CSF leak is detected, one should be prepared to reconstruct the skull base dural defect with a multilayered technique with a vascularized pedicled nasoseptal flap.
The patient tolerated the procedure well. We did not use a lumbar drain postoperatively, per our routine. The patient was neurologically intact without complications and was discharged home on postoperative day 4. Because of the radiation exposure during embolization, the patient later developed transient geometrical alopecia that was managed conservatively. On his last follow-up appointment, 6 months after surgery, the patient was doing well without any evidence of residual tumor or recurrence (Fig. 3.21). Moreover, his geometrical alopecia had completely recovered.
Because of the high rate of JNA recurrence, it is recommended to follow these patients for a minimum of 5 years after surgery. In our practice, we recommend a follow-up nasal endoscopy 3 months after surgery, and every 6 months after that for the first 3 years. Similarly, we recommend follow-up MRIs at 3 months after surgery, and once yearly thereafter for lifetime follow-up. If recurrence occurs, various treatment modalities can be instituted including reoperation, stereotactic radiosurgery, conventional radiotherapy, or watchful waiting. Some tumors may regress or stabilize when patients reach adulthood. However, this is not always the case, and therefore, continued radiologic follow-up is warranted.
The case described in this section elucidates the importance of individualized approaches for managing JNAs. Traditional open transfacial approaches are rarely used in clinical practice nowadays because of their limited visualization, high morbidity profile, and poor aesthetic outcomes, especially in this young patient population. With the advancements of surgical techniques and instrumentations, endoscopic surgery for JNA has better outcomes, lower complications, less blood loss, and superior aesthetic outcomes compared to open transfacial approaches. However, despite its excellent clinical and safety profile, EEAs have their inherent limitations and complications. The case presented in this chapter demonstrated one such limitation. The EEAs remain limited in the management of laterally extending tumors occupying multiple compartments. The JNA in our patient extended laterally into the ITF and inferior orbital fissure, and utilizing a purely endonasal approach would prove challenging in dissecting the tumor from the far-lateral component of the ITF. In these cases, addition of the Caldwell–Luc maxillotomy allows multiportal, multicorridor access to this region with direct anterior access. This corridor provides better visualization, increased surgical freedom, and less trauma to the nasal structures.
In complex JNAs with intracranial extension, cavernous sinus involvement, and vascular supply from ICA feeders, we recommend adding a transcranial approach from above (orbitozygomatic transcavernous approach, in most cases) to the endonasal approach from below. The orbitozygomatic transcavernous approach from above allows the surgeon to safely peel the tumor away from the cavernous sinus and provide early vascular control of the cavernous ICA. Once this is established, the remainder of the tumor can be removed from the endonasal and transmaxillary corridors from below. In our experience, this combined approach has been very useful in managing these larger complex JNAs.
We favor an individualized, tailored, endoscopic graduated multiangle, multicorridor skull base approach for the resection of extensive JNA (Table 3.2). These endoscopic endonasal and endoscopic-assisted approaches provide wide access to tumors extending into different compartments that cannot be removed completely using a single endoscopic corridor and provide superior aesthetic outcomes compared to traditional transfacial approaches. The endoscope can also be utilized in traditional microsurgical corridors to visualize the tumor and aid in the resection process. These two-surgeon four-handed techniques are making major open transfacial surgery, with its risk of facial deformity and scarring, nearly obsolete.
In our case example, the JNA extended to the far-lateral portion of the ITF. Thus, a combined binostril EEA and ipsilateral endoscopic Caldwell–Luc maxillotomy provided three different corridors to fully access the tumor including the ITF component. Denker’s anteromedial maxillotomy is an alternative approach to Caldwell–Luc maxillotomy that can be utilized in such patients. However, we prefer the Caldwell–Luc maxillotomy approach, as Denker’s anteromedial maxillotomy has a higher risk of cosmetic nasal deformity and nasolacrimal duct obstruction.
Routine use of preoperative angioembolization in these patients is of a paramount importance, as it provides excellent delineation of the tumor vasculature and significantly reduces intraoperative bleeding (by 70%), making complete tumor removal easier and safer. Despite embolization, however, early intraoperative ligation and sectioning of the ipsilateral internal maxillary artery in the ITF is important to further devascularize the tumor. Once this is achieved, tumor removal is much easier to perform. Finally, all patients with JNA should undergo routine, long-term radiologic follow-up, even if the initial resection is complete. It is not uncommon for the tumor to recur in a delayed fashion.
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