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Expanded endonasal endoscopic approach for resection of a juvenile nasopharyngeal angiofibroma with skull base involvement
Journal of Clinical Neuroscience 17 (2010) 1423–1427
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Technical Note
Expanded endonasal endoscopic approach for resection of a juvenile nasopharyngeal angiofibroma with skull base involvement Gary L. Gallia a,*, Murugappan Ramanathan Jr. b, Ari M. Blitz c, Douglas D. Reh b a
Department of Neurosurgery and Oncology, Johns Hopkins Hospital, Phipps Building, Room 118, Baltimore, Maryland 21287, USA Department of Otolaryngology, Head and Neck Surgery, Johns Hopkins Hospital, Baltimore, Maryland, USA c Department of Neuroradiology, Johns Hopkins Hospital, Baltimore, Maryland, USA b
a r t i c l e
i n f o
Article history: Received 2 April 2010 Accepted 8 April 2010
Keywords: Endoscopy Expanded endonasal approach Juvenile nasopharyngeal angiofibroma JNA Skull base Surgery
a b s t r a c t Juvenile nasopharyngeal angiofibromas (JNAs) are rare vascular tumors which arise in the nasopharynx of adolescent males. Patients with these tumors can be cured by surgery, which is the treatment of choice in the majority of patients. Traditional surgical techniques for patients with JNAs have been via open surgical approaches. Since 2000, however, the surgical management of JNAs has changed due to advances in endoscopic procedures and such approaches are standard for early stage lesions which are limited to the nasal cavity, nasopharynx and the paranasal sinuses. The role and limitations of endoscopic approaches for JNAs with skull base and intracranial involvement are being defined. In this report, we describe a patient with a JNA with skull base involvement who underwent an expanded endonasal endoscopic approach for a complete resection. Additionally, we review the literature of endoscopic approaches to JNAs with skull base involvement. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction Juvenile nasopharyngeal angiofibromas (JNAs) are relatively rare, benign, vascular neoplasms which occur in adolescent males and are characterized by slow but locally aggressive growth. These tumors typically present with unilateral symptoms including nasal obstruction and epistaxis. Other associated signs and symptoms can include facial swelling and proptosis. Cranial nerve compression may result in diplopia, sensory changes on the face and visual loss.1,2 Although there is some debate regarding the specific cell of origin, JNAs typically originate from the superior margin of the sphenopalatine foramen.1 From its origin, the tumor can grow into the nasal cavity and nasopharynx, paranasal sinuses, infratemporal fossa, orbit via the inferior orbital fissure and intracranial compartment. Intracranial extension can occur either through the middle cranial fossa anterior to foramen lacerum and lateral to the cavernous sinus and carotid artery or through the sella medial to the carotid artery and lateral to the pituitary gland.1,3,4 There have been numerous staging systems described for JNAs based on the anatomic extent of the tumor.3–5 Patients with JNAs can be cured with complete excision and therefore surgical resection is the treatment of choice for this tumor.6,7 Traditional surgical approaches for these lesions include * Corresponding author. Tel.: +1 410 614 0585; fax: +1 410 614 1853. E-mail address: [email protected] (G.L. Gallia). 0967-5868/$ - see front matter Ó 2010 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2010.04.013
transpalatal, transfacial (through a lateral rhinotomy or midfacial degloving) and infratemporal skull base approaches as well as combined surgical approaches.8–14 More recently, endonasal endoscopic surgical approaches have become integral in the management of these tumors and are considered the treatment of choice for lesions limited to the nasal cavity and nasopharynx.15–20 The utility and limitations of endoscopic techniques for more advanced lesions with skull base and/or intracranial involvement is less defined. In this report, we describe a patient with a large JNA with skull base involvement who underwent an expanded endonasal endoscopic approach for complete resection. 2. Case report A 20-year-old man with no significant past medical history was referred to our institution for a nasopharyngeal mass discovered after an evaluation for intermittent, recurrent right sided epistaxis. A maxillofacial CT scan demonstrated a mass centered in the right posterior nasopharynx, with involvement and erosion of the sphenoid bone in the region of the base of the ptyergoids and clivus. There was erosion and widening of the right vidian canal. High resolution MRI of the skull base with isotropic T1-weighted and T2weighted sequences and post-gadolinium T1-weighted sequences demonstrated the mass involving the right posterior nasal cavity and right nasopharynx. There was involvement of the sphenopalatine foramen with minimal extension into the medial portion of the pterygopalatine fossa. There was also involvement of the right
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side of the clivus and sphenoid bone. Within the sphenoid bone on the right, there was extension to the cerebral surface of the greater wing of the sphenoid with potential involvement of the dura in this region, corresponding to a Radkowski stage IIIA tumor (Fig. 1).5 Initial endoscopic examination demonstrated a large hyperemic mass occupying the entire right nasopharynx. Due to the vascularity of the mass, the patient was taken to the operating room for an endoscopic biopsy under general anesthesia. The pathology of this biopsy specimen returned as a JNA. The patient underwent a preoperative angiogram which demonstrated vascular supply to the mass from both terminal and proximal branches of the right internal maxillary artery as well as the vidian artery (Fig. 2). The right internal maxillary artery branches supplying the tumor were embolized with Embospheres (BioSphere Medical, Inc., Rockland, MA, USA) and coil embolization; the vidian artery was not embolized. The patient was awakened from anesthesia, examined neurologically and then taken to the operating room for an expanded endonasal endoscopic approach and tumor resection. Surgery was performed with neuronavigation using both the preoperative high resolution CT and MRI scans. The nasal cavity was injected with lidocaine and epinephrine; given the size and location of the tumor, the right sphenopalatine artery was injected via a trans-palatal injection. A right middle turbinectomy and uncinectomy were performed for endoscopic access to the tumor. A large maxillary antrostomy was then performed in conjunction with a total ethmoidectomy and sphenoidotomy. A left-sided nasoseptal ‘‘rescue flap”21 preserving the vascular pedicle (left posterior nasal artery) was elevated for possible reconstruction. The inferior cut in the flap was not made to allow easy tacking to the remnant septum if the flap was not required for reconstruction of the surgi-
cal defect. A posterior septectomy was performed allowing binaral access. The tumor was then defined circumferentially. The right sphenoid sinus was opened widely to the lateral most extent and the intersinus septum removed. The anterior aspect of the clivus was removed with a high-speed drill medially and inferiorly defining the margin of the tumor. The back wall of the maxillary sinus was removed with rongeurs to gain access to the pterygopalatine fossa and the lateral aspects of the tumor. Given the size of the lesion, the anterior aspect of the tumor in the nasal cavity and nasopharynx was amputated using endoscopic scissors and debulked with a microdebrider to enable better visualization of the tumor invading the skull base and clivus. Bleeding from the vidian artery was controlled with the bipolar and suction cauteries. The medial and inferior aspects of the clivus were found to have extensive tumor invasion and were further reduced with the drill until normal appearing bone was encountered. Using various dissectors, tumor was reflected laterally and numerous fronds of tumor were removed from bony cavities in the clivus and skull base (Fig. 3). As the tumor resection continued laterally along the skull base, the vidian artery and canal were identified. A large tumor frond within the vidian canal was identified and removed further exposing the vidian artery and nerve which were coagulated with the bipolar cautery and cut sharply (Fig. 3). The tumor resection then continued laterally along the skull base. Given the corrugated and mottled appearance of the bone as well as the very fibrous nature of the tumor, the fronds were gently pulled from the bony crevices. This resection continued along the skull base posteriorly towards foramen ovale and foramen lacerum and laterally to the lateral pterygoid plate. Inferiorly, the tumor was
Fig. 1. Soft tissue mass compatible with a juvenile nasopharyngeal angiofibroma centered in the right posterior nasal cavity and nasopharynx (asterisk). (A) Axial CT scan showing an expansile lytic component of the mass within the right pterygoid (arrow) and with focal clival erosion (interrupted arrow). (B) Axial precontrast T1-weighted MRI showing preservation of fatty signal within the pterygopalatine fossa (arrow) except in the region of the sphenopalatine foramen. (C) The mass enhances intensely on the corresponding axial T1-weighted postcontrast MRI. (D) Coronal reformat of post contrast T1-weighted imaging demonstrates involvement of the floor of the right middle cranial fossa (arrow).
G.L. Gallia et al. / Journal of Clinical Neuroscience 17 (2010) 1423–1427
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Fig. 2. Anteroposterior (left) and lateral (right) preoperative angiography showing a vascular supply from proximal and terminal branches of the internal maxillary artery.
Fig. 3. Intraoperative photographs. (A) Early endoscopic view following binaural access and exposure of the pterygopalatine fossa. (B) Endoscopic picture following drilling of the clivus. Normal appearing bone is seen on the right side of the image, bony crevices are seen on the left from which numerous nodules of tumor have been removed. (C) Endoscopic view following identification of the vidian canal and vidian artery. A large tumor nodule is seen and was removed from the vidian canal. (D) Endoscopic view following the tumor resection. The proximal coagulated vidian artery, middle cranial fossa, and Eustachian tube orifice are visualized. Normal appearing fat in the pterygopalatine fossa is present on the left side of this image. C = clivus, ETO = Eustachian tube orifice, MCF = middle cranial fossa, PPF = pterygopalatine fossa, SS = sphenoid sinus, T = tumor, TN = tumor nodule, VA = vidian artery, VC = vidian canal.
resected from the superior portion of the Eustachian tube cartilage (Fig. 3). The numerous bony crevices along the clivus and skull base associated with tumor invasion were extensively drilled to remove microscopic disease. A gross total resection was achieved. The dura was exposed in several areas; there was no cerebrospinal fluid leak encountered. The rescue nasal septal flap was then replaced along the nasal septum and sutured into position. Bilateral Doyle splints were then placed and secured with a nylon stitch. The patient tolerated the procedure well and had no neurologic deficits postoperatively. His postoperative skull base MRI scan demonstrated a gross total resection (Fig. 4). He was discharged home on postoperative day 3. On writing, the patient was 4 months postsurgery with no radiographic or clinical evidence of recurrent disease.
3. Discussion Complete surgical extirpation is the treatment of choice for patients with JNA. The traditional surgical approaches to these lesions historically include transoral, transfacial or combined craniofacial approaches.8–14 Advances in endoscopic surgery have provided a minimally invasive means to gain excellent tumor visualization that allows for safe and effective resection. For early stage lesions limited to the nasal cavity, nasopharynx and paranasal sinuses, endoscopic approaches are currently considered the treatment of choice.15–20 For more extensive JNAs with skull base and/or intracranial involvement, the optimal surgical approach is less well defined. Although many advocate open skull base approaches or even radiotherapy for advanced lesions, there are several reports
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G.L. Gallia et al. / Journal of Clinical Neuroscience 17 (2010) 1423–1427
Fig. 4. Axial (upper) and coronal (lower) postoperative T1-weighted MRI after gadolinium contrast showing a gross total resection.
describing endoscopic resection of JNAs with skull base involvement. Roger et al. reported a series of 20 patients with a mean follow-up of 22 months. Nine patients had a Radkowski stage IIIa tumor. In all but one of these patients, complete eradication was possible.19 Nicolai et al. reported a series or 15 patients with JNA who were treated endoscopically.17 Four of these patients had advanced disease. Three patients had stage IIIa disease and one patient had stage IIIb according to the system described by Andrews et al.4, defined as tumor invading the infratemporal fossa or orbital region without intracranial involvement (a) or with extradural (parasellar) involvement (b), respectively. All advanced lesions were successfully resected without a recurrence with a mean of 39 months of follow-up. Only one patient of the 15 developed a recurrence and this patient had early stage disease at presentation. Onerci et al. reported a series of 12 patients with JNA treated endoscopically. Four patients were classed as Radkowski stage IIIA. Two of these four patients had minimal residual disease around the cavernous sinus, but showed no progression of disease over a 2 year follow-up with MRI scans. Two patients had no residual or recurrent tumor over a follow-up of 6 months.18 Midilli et al. reported a series of 42 patients, 12 of whom were endoscopically treated; one patient had Radkowski stage IIIA disease22 and Tosun et al. reported two patients with Radkowski IIIA tumors resected endoscopically.14 Hackman et al. recently updated the Pittsburgh experience with JNA.23 A total of 31 cases were identified with 15 treated using a purely endoscopic approach. The authors specifically looked at the subset of patients with skull base involvement defined as radiographic evidence of bone erosion of the skull base excluding the pterygoid plates, anterior wall of the sphenoid sinus, orbital walls and maxillary sinus walls (that is, Radkowski stage IIIA or higher). There were a total of 12 patients in this subgroup. Three of these patients underwent a purely endoscopic resection, two underwent an open procedure and six underwent a combined
approach. There was one recurrence noted in the skull base subset in a patient who underwent a combined procedure.23 There are numerous advantages to an endoscopic approach including better illumination and a magnified field of view. This can lead to more complete inspection of the resection cavity especially for the tumor fronds, which intercalate into the bony crevices of the skull base (Fig. 3). This benefit is also well recognized in endoscopic-assisted procedures. An additional advantage of endoscopic approaches includes the avoidance of facial incisions and facial osteotomies requiring bone plating. This avoids potential craniofacial growth alterations in this patient population. One potential disadvantage of endoscopic approaches includes the obscuration of the operative field with hemorrhage. Given the vascularity of these tumors, however, and the risk of intraoperative hemorrhage, many groups including ours utilize preoperative angiography and embolization. This has been shown to reduce the intraoperative blood loss. Another disadvantage of endoscopic surgery is the limitation of being a one-handed surgeon. Our endonasal endoscopic skull base surgeries are performed together with an otolaryngologist and neurosurgeon allowing for the use of threehanded and four-handed techniques. This allows the operating surgeon the equivalent technical ability as with an open microscopic approach. An important aspect not only of the current patient, but of all of our endonasal endoscopic skull base cases, is the preoperative radiographic evaluation and surgical planning. All patients receive a maxillofacial CT scan for evaluation of the bony anatomy. Additionally, our patients undergo high-resolution skull base imaging with submillimeter isotropic sequences. The exceptional detail obtained from these scans enables us to determine preoperatively the optimal surgical approach as well as the anatomic and pathologic details expected at the time of surgery. Additionally, both CT scans and MRI are used for image guidance for our endonasal endoscopic skull base procedures.
4. Conclusion In this report, we describe a patient with a JNA with skull base involvement. He underwent an expanded endonasal endoscopic approach for gross total resection. This report adds to the growing body of data supporting the use of endoscopic resection of carefully selected JNAs with skull base involvement. Preoperative high-resolution skull base imaging is essential for surgical planning.
References 1. Neel 3rd HB, Whicker JH, Devine KD, et al. Juvenile angiofibroma. Review of 120 cases. Am J Surg 1973;126:547–56. 2. Tyagi I, Syal R, Goyal A. Staging and surgical approaches in large juvenile angiofibroma – study of 95 cases. Int J Pediatr Otorhinolaryngol 2006;70: 1619–27. 3. Bremer JW, Neel 3rd HB, Desanto LW, et al. Angiofibroma: treatment trends in 150 patients during 40 years. Laryngoscope 1986;96:1321–9. 4. Andrews JC, Fisch U, Valavanis A, et al. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. Laryngoscope 1989;99:429–37. 5. Radkowski D, McGill T, Healy GB, et al. Angiofibroma. Changes in staging and treatment. Arch Otolaryngol Head Neck Surg 1996;122:122–9. 6. Douglas R, Wormald PJ. Endoscopic surgery for juvenile nasopharyngeal angiofibroma: where are the limits? Curr Opin Otolaryngol Head Neck Surg 2006;14:1–5. 7. Enepekides DJ. Recent advances in the treatment of juvenile angiofibroma. Curr Opin Otolaryngol Head Neck Surg 2004;12:495–9. 8. Cansiz H, Guvenc MG, Sekercioglu N. Surgical approaches to juvenile nasopharyngeal angiofibroma. J Craniomaxillofac Surg 2006;34:3–8. 9. Fagan JJ, Snyderman CH, Carrau RL, et al. Nasopharyngeal angiofibromas: selecting a surgical approach. Head Neck 1997;19:391–9. 10. Hosseini SM, Borghei P, Borghei SH, et al. Angiofibroma: an outcome review of conventional surgical approaches. Eur Arch Otorhinolaryngol 2005;262:807–12.
G.L. Gallia et al. / Journal of Clinical Neuroscience 17 (2010) 1423–1427 11. Pryor SG, Moore EJ, Kasperbauer JL. Endoscopic versus traditional approaches for excision of juvenile nasopharyngeal angiofibroma. Laryngoscope 2005;115:1201–7. 12. Scholtz AW, Appenroth E, Kammen-Jolly K, et al. Juvenile nasopharyngeal angiofibroma: management and therapy. Laryngoscope 2001;111:681–7. 13. Tang IP, Shashinder S, Gopala Krishnan G, et al. Juvenile nasopharyngeal angiofibroma in a tertiary centre: ten-year experience. Singapore Med J 2009;50:261–4. 14. Tosun F, Ozer C, Gerek M, et al. Surgical approaches for nasopharyngeal angiofibroma: comparative analysis and current trends. J Craniofac Surg 2006;17:15–20. 15. Mair EA, Battiata A, Casler JD. Endoscopic laser-assisted excision of juvenile nasopharyngeal angiofibromas. Arch Otolaryngol Head Neck Surg 2003;129:454–9. 16. Mann WJ, Jecker P, Amedee RG. Juvenile angiofibromas: changing surgical concept over the last 20 years. Laryngoscope 2004;114:291–3. 17. Nicolai P, Berlucchi M, Tomenzoli D, et al. Endoscopic surgery for juvenile angiofibroma: when and how. Laryngoscope 2003;113:775–82.
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18. Onerci TM, Yucel OT, Ogretmenoglu O. Endoscopic surgery in treatment of juvenile nasopharyngeal angiofibroma. Int J Pediatr Otorhinolaryngol 2003;67:1219–25. 19. Roger G, Tran BA, Huy P, et al. Exclusively endoscopic removal of juvenile nasopharyngeal angiofibroma: trends and limits. Arch Otolaryngol Head Neck Surg 2002;128:928–35. 20. Yiotakis I, Eleftheriadou A, Davilis D, et al. Juvenile nasopharyngeal angiofibroma stages I and II: a comparative study of surgical approaches. Int J Pediatr Otorhinolaryngol 2008;72:793–800. 21. Madhok R, Prevedello DM, Gardner P, et al. Endoscopic endonasal resection of rathke cleft cysts: clinical outcomes and surgical nuances. J Neurosurg 2010;112:1333–9. 22. Midilli R, Karci B, Akyildiz S. Juvenile nasopharyngeal angiofibroma: analysis of 42 cases and important aspects of endoscopic approach. Int J Pediatr Otorhinolaryngol 2009;73:401–8. 23. Hackman T, Snyderman CH, Carrau R, et al. Juvenile nasopharyngeal angiofibroma: the expanded endonasal approach. Am J Rhinol Allergy 2009;23:95–9.
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Technical Note
Expanded endonasal endoscopic approach for resection of a juvenile nasopharyngeal angiofibroma with skull base involvement Gary L. Gallia a,*, Murugappan Ramanathan Jr. b, Ari M. Blitz c, Douglas D. Reh b a
Department of Neurosurgery and Oncology, Johns Hopkins Hospital, Phipps Building, Room 118, Baltimore, Maryland 21287, USA Department of Otolaryngology, Head and Neck Surgery, Johns Hopkins Hospital, Baltimore, Maryland, USA c Department of Neuroradiology, Johns Hopkins Hospital, Baltimore, Maryland, USA b
a r t i c l e
i n f o
Article history: Received 2 April 2010 Accepted 8 April 2010
Keywords: Endoscopy Expanded endonasal approach Juvenile nasopharyngeal angiofibroma JNA Skull base Surgery
a b s t r a c t Juvenile nasopharyngeal angiofibromas (JNAs) are rare vascular tumors which arise in the nasopharynx of adolescent males. Patients with these tumors can be cured by surgery, which is the treatment of choice in the majority of patients. Traditional surgical techniques for patients with JNAs have been via open surgical approaches. Since 2000, however, the surgical management of JNAs has changed due to advances in endoscopic procedures and such approaches are standard for early stage lesions which are limited to the nasal cavity, nasopharynx and the paranasal sinuses. The role and limitations of endoscopic approaches for JNAs with skull base and intracranial involvement are being defined. In this report, we describe a patient with a JNA with skull base involvement who underwent an expanded endonasal endoscopic approach for a complete resection. Additionally, we review the literature of endoscopic approaches to JNAs with skull base involvement. Ó 2010 Elsevier Ltd. All rights reserved.
1. Introduction Juvenile nasopharyngeal angiofibromas (JNAs) are relatively rare, benign, vascular neoplasms which occur in adolescent males and are characterized by slow but locally aggressive growth. These tumors typically present with unilateral symptoms including nasal obstruction and epistaxis. Other associated signs and symptoms can include facial swelling and proptosis. Cranial nerve compression may result in diplopia, sensory changes on the face and visual loss.1,2 Although there is some debate regarding the specific cell of origin, JNAs typically originate from the superior margin of the sphenopalatine foramen.1 From its origin, the tumor can grow into the nasal cavity and nasopharynx, paranasal sinuses, infratemporal fossa, orbit via the inferior orbital fissure and intracranial compartment. Intracranial extension can occur either through the middle cranial fossa anterior to foramen lacerum and lateral to the cavernous sinus and carotid artery or through the sella medial to the carotid artery and lateral to the pituitary gland.1,3,4 There have been numerous staging systems described for JNAs based on the anatomic extent of the tumor.3–5 Patients with JNAs can be cured with complete excision and therefore surgical resection is the treatment of choice for this tumor.6,7 Traditional surgical approaches for these lesions include * Corresponding author. Tel.: +1 410 614 0585; fax: +1 410 614 1853. E-mail address: [email protected] (G.L. Gallia). 0967-5868/$ - see front matter Ó 2010 Elsevier Ltd. All rights reserved. doi:10.1016/j.jocn.2010.04.013
transpalatal, transfacial (through a lateral rhinotomy or midfacial degloving) and infratemporal skull base approaches as well as combined surgical approaches.8–14 More recently, endonasal endoscopic surgical approaches have become integral in the management of these tumors and are considered the treatment of choice for lesions limited to the nasal cavity and nasopharynx.15–20 The utility and limitations of endoscopic techniques for more advanced lesions with skull base and/or intracranial involvement is less defined. In this report, we describe a patient with a large JNA with skull base involvement who underwent an expanded endonasal endoscopic approach for complete resection. 2. Case report A 20-year-old man with no significant past medical history was referred to our institution for a nasopharyngeal mass discovered after an evaluation for intermittent, recurrent right sided epistaxis. A maxillofacial CT scan demonstrated a mass centered in the right posterior nasopharynx, with involvement and erosion of the sphenoid bone in the region of the base of the ptyergoids and clivus. There was erosion and widening of the right vidian canal. High resolution MRI of the skull base with isotropic T1-weighted and T2weighted sequences and post-gadolinium T1-weighted sequences demonstrated the mass involving the right posterior nasal cavity and right nasopharynx. There was involvement of the sphenopalatine foramen with minimal extension into the medial portion of the pterygopalatine fossa. There was also involvement of the right
1424
G.L. Gallia et al. / Journal of Clinical Neuroscience 17 (2010) 1423–1427
side of the clivus and sphenoid bone. Within the sphenoid bone on the right, there was extension to the cerebral surface of the greater wing of the sphenoid with potential involvement of the dura in this region, corresponding to a Radkowski stage IIIA tumor (Fig. 1).5 Initial endoscopic examination demonstrated a large hyperemic mass occupying the entire right nasopharynx. Due to the vascularity of the mass, the patient was taken to the operating room for an endoscopic biopsy under general anesthesia. The pathology of this biopsy specimen returned as a JNA. The patient underwent a preoperative angiogram which demonstrated vascular supply to the mass from both terminal and proximal branches of the right internal maxillary artery as well as the vidian artery (Fig. 2). The right internal maxillary artery branches supplying the tumor were embolized with Embospheres (BioSphere Medical, Inc., Rockland, MA, USA) and coil embolization; the vidian artery was not embolized. The patient was awakened from anesthesia, examined neurologically and then taken to the operating room for an expanded endonasal endoscopic approach and tumor resection. Surgery was performed with neuronavigation using both the preoperative high resolution CT and MRI scans. The nasal cavity was injected with lidocaine and epinephrine; given the size and location of the tumor, the right sphenopalatine artery was injected via a trans-palatal injection. A right middle turbinectomy and uncinectomy were performed for endoscopic access to the tumor. A large maxillary antrostomy was then performed in conjunction with a total ethmoidectomy and sphenoidotomy. A left-sided nasoseptal ‘‘rescue flap”21 preserving the vascular pedicle (left posterior nasal artery) was elevated for possible reconstruction. The inferior cut in the flap was not made to allow easy tacking to the remnant septum if the flap was not required for reconstruction of the surgi-
cal defect. A posterior septectomy was performed allowing binaral access. The tumor was then defined circumferentially. The right sphenoid sinus was opened widely to the lateral most extent and the intersinus septum removed. The anterior aspect of the clivus was removed with a high-speed drill medially and inferiorly defining the margin of the tumor. The back wall of the maxillary sinus was removed with rongeurs to gain access to the pterygopalatine fossa and the lateral aspects of the tumor. Given the size of the lesion, the anterior aspect of the tumor in the nasal cavity and nasopharynx was amputated using endoscopic scissors and debulked with a microdebrider to enable better visualization of the tumor invading the skull base and clivus. Bleeding from the vidian artery was controlled with the bipolar and suction cauteries. The medial and inferior aspects of the clivus were found to have extensive tumor invasion and were further reduced with the drill until normal appearing bone was encountered. Using various dissectors, tumor was reflected laterally and numerous fronds of tumor were removed from bony cavities in the clivus and skull base (Fig. 3). As the tumor resection continued laterally along the skull base, the vidian artery and canal were identified. A large tumor frond within the vidian canal was identified and removed further exposing the vidian artery and nerve which were coagulated with the bipolar cautery and cut sharply (Fig. 3). The tumor resection then continued laterally along the skull base. Given the corrugated and mottled appearance of the bone as well as the very fibrous nature of the tumor, the fronds were gently pulled from the bony crevices. This resection continued along the skull base posteriorly towards foramen ovale and foramen lacerum and laterally to the lateral pterygoid plate. Inferiorly, the tumor was
Fig. 1. Soft tissue mass compatible with a juvenile nasopharyngeal angiofibroma centered in the right posterior nasal cavity and nasopharynx (asterisk). (A) Axial CT scan showing an expansile lytic component of the mass within the right pterygoid (arrow) and with focal clival erosion (interrupted arrow). (B) Axial precontrast T1-weighted MRI showing preservation of fatty signal within the pterygopalatine fossa (arrow) except in the region of the sphenopalatine foramen. (C) The mass enhances intensely on the corresponding axial T1-weighted postcontrast MRI. (D) Coronal reformat of post contrast T1-weighted imaging demonstrates involvement of the floor of the right middle cranial fossa (arrow).
G.L. Gallia et al. / Journal of Clinical Neuroscience 17 (2010) 1423–1427
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Fig. 2. Anteroposterior (left) and lateral (right) preoperative angiography showing a vascular supply from proximal and terminal branches of the internal maxillary artery.
Fig. 3. Intraoperative photographs. (A) Early endoscopic view following binaural access and exposure of the pterygopalatine fossa. (B) Endoscopic picture following drilling of the clivus. Normal appearing bone is seen on the right side of the image, bony crevices are seen on the left from which numerous nodules of tumor have been removed. (C) Endoscopic view following identification of the vidian canal and vidian artery. A large tumor nodule is seen and was removed from the vidian canal. (D) Endoscopic view following the tumor resection. The proximal coagulated vidian artery, middle cranial fossa, and Eustachian tube orifice are visualized. Normal appearing fat in the pterygopalatine fossa is present on the left side of this image. C = clivus, ETO = Eustachian tube orifice, MCF = middle cranial fossa, PPF = pterygopalatine fossa, SS = sphenoid sinus, T = tumor, TN = tumor nodule, VA = vidian artery, VC = vidian canal.
resected from the superior portion of the Eustachian tube cartilage (Fig. 3). The numerous bony crevices along the clivus and skull base associated with tumor invasion were extensively drilled to remove microscopic disease. A gross total resection was achieved. The dura was exposed in several areas; there was no cerebrospinal fluid leak encountered. The rescue nasal septal flap was then replaced along the nasal septum and sutured into position. Bilateral Doyle splints were then placed and secured with a nylon stitch. The patient tolerated the procedure well and had no neurologic deficits postoperatively. His postoperative skull base MRI scan demonstrated a gross total resection (Fig. 4). He was discharged home on postoperative day 3. On writing, the patient was 4 months postsurgery with no radiographic or clinical evidence of recurrent disease.
3. Discussion Complete surgical extirpation is the treatment of choice for patients with JNA. The traditional surgical approaches to these lesions historically include transoral, transfacial or combined craniofacial approaches.8–14 Advances in endoscopic surgery have provided a minimally invasive means to gain excellent tumor visualization that allows for safe and effective resection. For early stage lesions limited to the nasal cavity, nasopharynx and paranasal sinuses, endoscopic approaches are currently considered the treatment of choice.15–20 For more extensive JNAs with skull base and/or intracranial involvement, the optimal surgical approach is less well defined. Although many advocate open skull base approaches or even radiotherapy for advanced lesions, there are several reports
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G.L. Gallia et al. / Journal of Clinical Neuroscience 17 (2010) 1423–1427
Fig. 4. Axial (upper) and coronal (lower) postoperative T1-weighted MRI after gadolinium contrast showing a gross total resection.
describing endoscopic resection of JNAs with skull base involvement. Roger et al. reported a series of 20 patients with a mean follow-up of 22 months. Nine patients had a Radkowski stage IIIa tumor. In all but one of these patients, complete eradication was possible.19 Nicolai et al. reported a series or 15 patients with JNA who were treated endoscopically.17 Four of these patients had advanced disease. Three patients had stage IIIa disease and one patient had stage IIIb according to the system described by Andrews et al.4, defined as tumor invading the infratemporal fossa or orbital region without intracranial involvement (a) or with extradural (parasellar) involvement (b), respectively. All advanced lesions were successfully resected without a recurrence with a mean of 39 months of follow-up. Only one patient of the 15 developed a recurrence and this patient had early stage disease at presentation. Onerci et al. reported a series of 12 patients with JNA treated endoscopically. Four patients were classed as Radkowski stage IIIA. Two of these four patients had minimal residual disease around the cavernous sinus, but showed no progression of disease over a 2 year follow-up with MRI scans. Two patients had no residual or recurrent tumor over a follow-up of 6 months.18 Midilli et al. reported a series of 42 patients, 12 of whom were endoscopically treated; one patient had Radkowski stage IIIA disease22 and Tosun et al. reported two patients with Radkowski IIIA tumors resected endoscopically.14 Hackman et al. recently updated the Pittsburgh experience with JNA.23 A total of 31 cases were identified with 15 treated using a purely endoscopic approach. The authors specifically looked at the subset of patients with skull base involvement defined as radiographic evidence of bone erosion of the skull base excluding the pterygoid plates, anterior wall of the sphenoid sinus, orbital walls and maxillary sinus walls (that is, Radkowski stage IIIA or higher). There were a total of 12 patients in this subgroup. Three of these patients underwent a purely endoscopic resection, two underwent an open procedure and six underwent a combined
approach. There was one recurrence noted in the skull base subset in a patient who underwent a combined procedure.23 There are numerous advantages to an endoscopic approach including better illumination and a magnified field of view. This can lead to more complete inspection of the resection cavity especially for the tumor fronds, which intercalate into the bony crevices of the skull base (Fig. 3). This benefit is also well recognized in endoscopic-assisted procedures. An additional advantage of endoscopic approaches includes the avoidance of facial incisions and facial osteotomies requiring bone plating. This avoids potential craniofacial growth alterations in this patient population. One potential disadvantage of endoscopic approaches includes the obscuration of the operative field with hemorrhage. Given the vascularity of these tumors, however, and the risk of intraoperative hemorrhage, many groups including ours utilize preoperative angiography and embolization. This has been shown to reduce the intraoperative blood loss. Another disadvantage of endoscopic surgery is the limitation of being a one-handed surgeon. Our endonasal endoscopic skull base surgeries are performed together with an otolaryngologist and neurosurgeon allowing for the use of threehanded and four-handed techniques. This allows the operating surgeon the equivalent technical ability as with an open microscopic approach. An important aspect not only of the current patient, but of all of our endonasal endoscopic skull base cases, is the preoperative radiographic evaluation and surgical planning. All patients receive a maxillofacial CT scan for evaluation of the bony anatomy. Additionally, our patients undergo high-resolution skull base imaging with submillimeter isotropic sequences. The exceptional detail obtained from these scans enables us to determine preoperatively the optimal surgical approach as well as the anatomic and pathologic details expected at the time of surgery. Additionally, both CT scans and MRI are used for image guidance for our endonasal endoscopic skull base procedures.
4. Conclusion In this report, we describe a patient with a JNA with skull base involvement. He underwent an expanded endonasal endoscopic approach for gross total resection. This report adds to the growing body of data supporting the use of endoscopic resection of carefully selected JNAs with skull base involvement. Preoperative high-resolution skull base imaging is essential for surgical planning.
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