Craniofacial Resection of Advanced Juvenile Nasopharyngeal Angiofibroma

ORIGINAL ARTICLE Craniofacial Resection of Advanced Juvenile Nasopharyngeal Angiofibroma

Christina Bales, BA; Mark Kotapka, MD; Laurie A. Loevner, MD; Mouwafak Al-Rawi, MD; Gregory Weinstein, MD; Robert Hurst, MD; Randal S. Weber, MD

Objective: To describe the results of a craniofacial ap- Main Outcome Measures: Intraoperative and post- proach to resection of stage IIIB juvenile nasopharyn- operative morbidity. geal angiofibroma, performed by an integrated skull base surgical team. Results: The average operating time was 12 hours 47 minutes. Estimated blood loss ranged from 700 to 1750 Design: A retrospective case-series review was con- mL (mean, 1120 mL), with 2 patients requiring intra- ducted with postoperative follow-up ranging from 28 to operative transfusion. Patients were hospitalized for 63 months. a mean duration of 5.6 days. Long-term morbidity includes facial dysesthesia, nasal crusting, and malodor- Setting: Operations were performed at a tertiary medi- ous nasal discharge. No patients sustained stroke, ocu- cal center. lomotor dysfunction, vision loss, or auditory impair- ment. At most recent follow-up, which ranges from 28 Patients: A referred sample of 5 male patients, ranging to 63 months, tumor recurrence has been confirmed in in age from 10 to 23 years (mean, 15 years). 1 patient.

Interventions: All patients underwent resection of na- Conclusions: A combined craniofacial approach is ap- sopharyngeal angiofibromas with intracranial exten- propriate for juvenile nasopharyngeal angiofibroma that sion. The procedure involved an infratemporal fossa ap- extends intracranially. Complete tumor removal with ac- proach via zygomatic osteotomy and subtemporal ceptable morbidity can be expected. craniectomy. Anterior exposure was gained through a standard facial translocation. Dissection of the cavernous carotid artery was required in 3 patients. Arch Otolaryngol Head Neck Surg. 2002;128:1071-1078

UVENILE NASOPHARYNGEAL angio- ated with tumor regression and symptom fibroma (JNA) is a benign, highly relief.7,8 Nevertheless, use of these non- vascular tumor that typically surgical modalities in the pediatric popu- originates at the superior mar- lation introduces additional sources of gin of the sphenopalatine fora- morbidity, including secondary malig- men. It predominantly occurs in nancy, cranial neuropathy, brainstem com- J 2,6 adolescent males and accounts promise, and growth arrest. The devel- for 0.05% of all head and neck neo- opment of contemporary craniofacial plasms.1 Despite histologically benign fea- surgical approaches to JNA has initiated tures, JNA can cause significant morbid- a trend away from radiation and chemo- ity and occasional mortality through therapy.4 This report reviews the results From the University of aggressive submucosal spread to adja- of our experience with the craniofacial ap- Pennsylvania Medical School, cent tissues.2-5 Approximately 20% of cases proach to extensive angiofibromas (stage Philadelphia (Ms Bales); pose a considerable obstacle to clinical IIIB tumors), performed by an integrated and the Departments of management due to skull base penetra- skull base surgical team. Neurosurgery (Dr Kotapka), tion and involvement of vital intracranial Radiology (Drs Loevner structures.3 PARTICIPANTS AND METHODS and Hurst), and Otorhinolaryngology–Head and Until recently, intracranially inva- Between 1996 and 1999, 5 patients were evalu- Neck Surgery (Drs Al-Rawi, sive angiofibromas were considered inop- ated and treated with a combined craniofacial Weinstein, and Weber), erable, leaving radiation and chemo- resection for JNA in the University of Penn- University of Pennsylvania therapy as the only viable treatment sylvania (Philadelphia) Departments of Oto- Medical Center, Philadelphia. strategies.4,6 Both alternatives are associ- rhinolaryngology–Head and Neck Surgery and

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Figure 1. Hemicoronal and Weber-Fergusson incisions. Figure 2. Bone cuts associated with a standard facial translocation.

Neurosurgery. The patients were white males, ranging in age Under an operating microscope, the dura was dissected from 10 to 23 years (mean, 15 years) at the time of surgery. All from the floor of the middle cranial fossa. The floor was then cases represented initial tumor presentations, with the excep- entered with a high-speed pneumatic drill to sequentially ex- tion of 1 patient referred with a recurrent tumor 23 months pose the following structures: (1) the superior orbital fissure; after his initial surgery. Each patient presented with evidence (2) the foramen rotundum with the second division of the of intracranial extension as determined by imaging studies. Ini- trigeminal nerve (V2); (3) the foramen ovale with the third di- tial patient evaluation involved a comprehensive head and neck vision of the trigeminal nerve (V3) and the accessory menin- history and physical examination, including fiberoptic exami- geal artery; (4) the foramen spinosum with the middle men- nation of the nasopharynx. Radiological staging was obtained ingeal artery and vein; (5) the greater superficial petrosal nerve; by computed tomography and magnetic resonance imaging. and (6) the carotid canal. The middle meningeal vessels and Studies were reviewed by a neuroradiologist specializing in head the trigeminal nerve divisions were selectively divided to pro- and neck imaging. Angiography was performed to identify pri- vide access to the tumor extensions in the middle cranial fossa mary feeding vessels to the tumor and to selectively embolize and the cavernous sinus. Additional removal of bone medial them prior to surgery. All embolizations took place within 24 to the foramen rotundum provided a lateral approach to the hours of tumor resection. tumor within the sphenoid sinus. Likewise, drilling medial to Surgery was initiated with an infratemporal fossa approach the foramen ovale permitted access to the tumor abutting the via zygomatic osteotomy and subtemporal craniectomy (Figure 1). petrous carotid artery. In cases of orbital tumor extensions, an After complete exposure and dissection of the tumor from the sphe- orbitozygomatic osteotomy was performed to facilitate tumor noid sinus, the superior orbital fissure, the middle cranial fossa, dissection from the orbital fissures. and the lateral cavernous sinus, anterior exposure was gained After the superior aspect of the angiofibroma had been com- through a standard facial translocation (Figure 2). The additional pletely mobilized, the transfacial approach was initiated through exposure permitted tumor mobilization from the nasopharynx, a Weber-Fergusson incision that extended through the underly- the paranasal sinuses, the pterygopalatine fossa, the infratempo- ing bone. The resulting cheek flap was turned to expose the piri- ral fossa, and the medial cavernous sinus. form aperture, the inferior orbital rim, and the inferior aspect of After induction of anesthesia, a subarachnoid lumbar drain- the anterior maxillary wall. The orbital contents were then reflected age catheter was inserted to facilitate intraoperative brain relax- posteriorly to expose the lacrimal sac. The lacrimal sac was divided, ation. The patient was placed supine with his head extended and the orbital contents were retracted, and the soft tissues lining the rotated 60° away from the intracranial tumor bulk. A hemicoro- inner aspect of the piriform aperture were elevated. Multiple bone nal skin incision was initiated in the preauricular region and ex- cuts were then made to allow the anterior maxilla and overlying tended behind the hairline. A scalp flap was subsequently el- cheek flap to be laterally reflected as a vascularized osteoplastic evated to expose the underlying zygomatic bone and arch. The flap (Figure 3). Specifically, cuts were made (1) from the supe- flap, which extended from the zygomaticomaxillary suture to the rior piriform aperture to the inferomedial orbit; (2) across the in- external auditory canal, incorporated the temporal fat pad to pre- ferior aspect of the maxillary buttress; (3) across the anterior wall serve the frontal branch of the facial nerve. A zygomatic oste- of the maxilla; and (4) through the lateral orbit. The bone of the otomy was performed, and the temporalis muscle was reflected posterior maxillary wall was removed, and the internal maxillary inferiorly through the resulting defect. Single burr holes were artery was clamped and divided. The pterygoid plates were then drilled at the pterion and above the zygomatic root. A bone flap removed with a high-speed pneumatic drill from either the trans- was turned with a craniotome and stored in a covered basin of facial or the cranial approach. Working concomitantly from above sterile isotonic sodium chloride solution. Perimeter holes were and below the skull base, the tumor was dissected and removed then placed to allow the dura to be secured with suture. en bloc. Although the presence of a tumor pseudocapsule largely

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 obviated the need for frozen sections, they were used in isolated a buttress against which the packing was placed. The skin in- cases in which a plane of dissection could not be clearly identi- cision was closed in layers. fied. Specifically, multiple frozen sections were obtained from the eustachian tube region in 2 patients and from the pterygoid RESULTS musculature in 1 patient. Successive sections were sent when necessary to ensure that all operative site margins were free of The most common presenting symptom, reported by all tumor. 5 patients, was unilateral nasal obstruction. Three of 5 Upon achieving complete tumor removal and hemosta- patients also developed severe recurring epistaxis or eu- sis, the temporalis muscle was split. One half of the muscle was stachian tube dysfunction. Two of 5 patients experi- folded under the temporal lobe and placed into the sphenoid enced sinus pressure, rhinitis, and/or anosmia. Symp- sinus. The craniotomy flap was then restored to its anatomic position and secured with a plating system (Synthes 1.3; Syn- tom duration prior to presentation ranged from 1 to 9 thes, Paoli, Pa). The wound was passively drained, and the scalp months. Physical examination revealed a unilateral, fleshy, flap was closed in 2 layers. red mass obscuring the nasal cavity in 4 of 5 patients. Prior to closing the facial wound, an internal dacryocys- Tumor extension into the nasopharynx was demon- torhinostomy was performed. Specifically, the divided lacri- strated via fiberoptic nasopharyngoscopy in 3 of these mal sac was incised vertically and marsupialized with 2 su- patients, with 1 individual exhibiting posterior displace- tures. The facial bone flap was then replaced and secured with ment of the soft palate. Facial deformity in the cheek area miniplates. The entire nasal cavity was packed with gauze im- was also noted in 2 of the 5 patients. One of these indi- pregnated with antibiotic ointment. A Foley catheter served as viduals exhibited marked proptosis. Radiological evidence of tumor extension and sites of intracranial involvement for each patient are presented in Table 1 and representative imaging studies are shown in Figure 4 and Figure 5A-C. Skull base invasion and/or intracranial extension were demonstrated via computed tomography or magnetic resonance imaging in all 5 cases. All tumors extended into the cavernous sinus, and 3 tumors abutted the internal carotid artery. Invasion of the vidian canal was demonstrated in 4 patients. Orbital involvement with tumor extension through the inferior and/or superior orbital fissures was noted in 3 of 5 patients. Angiography showed that the majority of tumors were supplied by branches from both the internal and external carotid arteries. Branches of the external carotid artery included the internal maxillary and the accessory meningeal arteries, while those of the internal carotid artery included the mandibulovidian (Figure 5D) and the cavernous arteries. Preoperative embolization of external carotid artery branches was accomplished without complications in all patients. Thirty-minute balloon- occlusion tests of the internal carotid artery ipsilateral to the primary tumor were also conducted in 3 patients. This procedure was performed under fluoroscopic guid- Figure 3. Reflection of a vascularized anterior maxillary osteoplastic flap. ance following systemic heparinization. No patients ex-

Table 1. Summary of Patient Profiles and Tumor Characteristics*

Surgery Estimated Units Patient Age at Prior Duration, Blood of Blood Hospitalization Follow-up, No. Surgery, y Treatment Sites of Extension Blood Supply h:min Loss, mL Transfused Duration, d mo 1 23 No SS, ES, CCS, IF, MCF, IMA (U) 11:33 700 0 7 63 PPF, OF 2 14 Yes SS, MS, CCS, IF, MCF, AMA (U), CB and 13:23 1750 2 5 34 VC, PPF, ET MVB of ICA (U) 3 13 No SS, CCS, IF, MCF, VC, IMA (B), MVB of 15:23 1100 0 6 32 PPF, ET ICA (B) 4 10 No SS, MS, ES, CCS, IF, IMA (B), CB and 13:25 850 1 3 32 MCF, ACF, VC, PPF, OF MVB of ICA (U) 5 15 No SS, MS, ES, CCS, IF, IMA (U) 10:11 1200 0 7 28 MCF, VC, PPF, OF

*SS indicates sphenoid sinus; ES, ethmoid sinus; CCS, carotid cavernous sinus; IF, infratemporal fossa; MCF, middle cranial fossa; PPF, pterygopalatine fossa; OF, orbital fissure; IMA, internal maxillary artery; U, unilateral; MS, maxillary sinus; VC, vidian canal; ET, eustachian tube; AMA, accessory meningeal artery; CB, cavernous branch; MVB, mandibulovidian branch; ICA, internal carotid artery; B, bilateral; and ACF, anterior cranial fossa.

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 A plications. The most common complication is nasal crusting, found in 4 patients. Serous otitis media was present in 3 patients at the initial postoperative evaluation, but has resolved completely in all cases. Two patients developed facial anesthesia due to sectioning of the second division of the trigeminal nerve. One patient has loss of sensation in the second and third division territories, the other experiences a deficit only in the second division terri- tory. Additional isolated complications included tran- sient facial lymphedema, intermittent eye irritation, chronic sinusitis, and a persistent malodorous nasal discharge. Recurrent disease has been confirmed in 1 patient who presented 19 months after resection with com- plaints of epiphora and intermittent swelling in the medial canthal region. Magnetic resonance imaging revealed a cystic lesion that was centered in the pterygopalatine fossa and extended into the right orbit, the right sphenoid sinus, and the medial wall of the right B middle cranial fossa. Initial management included cyst aspiration, which failed to yield cerebrospinal or other fluid contents, and marsupialization through an endoscopic approach. Four months after this procedure, follow-up magnetic resonance imaging showed replace- ment of the cyst with enhancing solid tumor. Given the distribution of disease in the cavernous sinus and along the second division of the trigeminal nerve, the tumor was deemed unresectable, and the patient was referred for radiation treatment. Representative radiographic images are shown in Figure 6A and B. Follow-up of a second patient has also revealed an area suspicious for recurrence near the petrous carotid artery (Figure 7). This area has been monitored at 3-month intervals, with no evidence of change over the last 18 months. The stabil- ity of the lesion is most consistent with postoperative scarring. However, the region will continue to undergo ob- servation for evidence of recurrent disease.

Figure 4. A 10-year-old boy with juvenile nasopharyngeal angiofibroma. A, Axial T2-weighted (repetition time [TR], 2500 milliseconds; echo time COMMENT [TE], 90 milliseconds) magnetic resonance image with fat suppression. There is extension of the patient’s juvenile angiofibroma into the posterior The epidemiological profile, clinical presentation, and di- ethmoid air cells and sphenoid sinus (arrows). In addition, neoplasm has extended from the pterygopalatine fossa to the foramen rotundum agnostic evaluation of this sample are consistent with pre- (arrowheads), the inferior orbital fissure, and the masticator space (white viously published reports. Although isolated cases of JNA arrow) via the pterygomaxillary fissure. Neoplasm abuts the precavernous have been observed in adult and female populations, the right internal carotid artery (curved arrow). B, Enhanced fat-suppressed coronal T1-weighted (TR, 600 milliseconds; TE, 17 milliseconds) magnetic tumor typically occurs in pubescent boys. The classic pre- resonance image. This again shows neoplasm extending to the sphenoid sentation includes nasal obstruction, recurrent epistaxis, sinus (arrows) and foramen rotundum (arrowhead), with frank invasion of and the presence of a smooth, lobulated, red-gray mass the pterygoid bone and vidian canal (curved arrow). in the posterior nasal cavity or nasopharynx. Physical examination may reveal facial deformity, proptosis, palate hibited neurological changes during or after temporary expansion, serous otitis, and visual or auditory impair- occlusion. ment.9,10 Our patients were initially evaluated 1 to 9 months Complete tumor resection was accomplished through after the onset of these characteristic signs and symp- a craniofacial approach. A lumbar drain was placed prior toms. Their delay in presentation can be attributed to a to surgery in all patients. Operating time ranged from 10 tendency to associate the indolent symptoms of JNA with hours 11 minutes to 15 hours 23 minutes, with a mean more common disease entities, such as rhinitis, sinusitis, duration of 12 hours 47 minutes. The estimated blood loss and antrochoanal nasal polyps.11 ranged from 700 to 1750 mL and averaged 1120 mL. Trans- Characteristic routes of JNA invasion are anterior fusion was necessary in 2 patients, who each received up infiltration of the nasal cavity, anterolateral erosion of the to2Uofpacked red blood cells. Patients were hospital- posterior maxillary sinus wall, and/or anterosuperior de- ized for an average of 5.6 days following surgery. struction of the ethmoid air cells. Lateral growth leads Ongoing patient follow-up, which ranges from 28 to invasion of the pterygopalatine fossa, erosion of the to 63 months, has revealed minimal postoperative com- pterygoid plates, and potential extension into the infra-

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C D

Figure 5. A 13-year-old boy with juvenile nasopharyngeal angiofibroma with skull base extension and invasion of the left cavernous sinus. A, Axial unenhanced T1-weighted (repetition time [TR], 600 milliseconds; echo time [TE], 17 milliseconds) magnetic resonance image at the level of the skull base/clivus. Abnormal tissue is seen in the region of the left vidian canal (white arrow), and neoplasm is seen along the horizontal portion of the left petrous internal carotid artery (arrowheads). B, Axial enhanced fat-suppressed T1-weighted (TR, 600 milliseconds; TE, 17 milliseconds) magnetic resonance image obtained at the same level as panel A. Enhancing tumor is seen in the skull base at the left vidian canal (black arrow), and medial to and abutting the left petrous internal carotid artery (arrowheads). C, Axial T-weighted (TR, 2500 milliseconds; TE, 85 milliseconds) magnetic resonance image obtained at the level of the cavernous sinus demonstrates hypointense tumor in the left cavernous sinus (arrows). Again, tumor abuts the left internal carotid artery (curved arrow). D, Lateral cerebral angiogram obtained following selective catheterization of the proximal left internal carotid artery. This image demonstrates supply to the neoplasm from the mandibulovidian artery (arrows).

temporal fossa via the pterygomaxillary fissure. From the eral to the cavernous sinus. However, particularly ag- infratemporal fossa, JNA may invade the inferior orbital gressive angiofibromas may invade the cavernous sinus fissure and erode the greater wing of the sphenoid bone. and threaten multiple cranial nerves (III, IV, V [divi- The tumor may also exhibit intracranial extension. Spe- sions 1 and 2], and VI), the internal carotid artery, the cifically, JNA may enter the middle cranial fossa by de- hypophysis, the optic chiasm, and the lateral wall of the stroying the superior bony region triangulated by the fo- sphenoid sinus. A second pattern of intracranial destruc- ramen rotundum, foramen ovale, and foramen lacerum. tion may arise in cases of posterosuperior extension from Further extension from the middle cranial fossa tends to the tumor origin. In these situations, JNA spreads into respect the dural barrier, such that the tumor remains lat- the nasopharynx, erodes through the inferior wall of the

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T T

Figure 6. Enhanced fat-suppressed coronal T1-weighted magnetic resonance images showing tumor recurrence at the skull base and orbit. A, Tumor is present in the bed of the right pterygopalatine fossa (T) with extension into the orbit (arrow). B, Image obtained posterior to panel A showing tumor invading the skull base (arrows).

Table 2. Revised Tumor Staging System*

Tumor Stage Classification IA Tumor limited to posterior nares and/or nasopharyngeal vault IB Tumor involving the posterior nares and/or nasopharyngeal vault with involvement of at least 1 paranasal sinus IIA Minimal lateral extension into the pterygomaxillary fossa IIB Full occupation of the pterygomaxillary fossa with or without superior erosion of the orbital bones IIC Extension into the infratemporal fossa or extension posterior to the pterygoid plates IIIA Erosion of the base of skull (middle cranial fossa/base of pterygoids)—minimal intracranial extension IIIB Extensive intracranial extension with or without extension into the cavernous sinus

*Adapted from Radkowski et al.21

Figure 7. Enhanced fat-suppressed axial T1-weighted magnetic resonance We used the most recently revised system, introduced by image 12 months following surgery. New enhancing soft tissue (arrow) Radkowski et al12 in 1996 (Table 2). This system reflects consistent with possible tumor recurrence is noted just medial to the petrous the incremental rise in tumor recurrence observed at pro- portion of the left internal carotid artery. gressively higher levels of skull base/intracranial involvement. Based on this approach, all tumors in our series were sphenoid sinus, and enters the cavernous sinus from an classified as stage IIIB angiofibromas. inferomedial approach. Our preoperative assessment of patients was facili- Angiofibromas with significant intracranial extension tated by the development of modern imaging techniques. have historically been associated with increased rates of un- Since 1974, head computed tomographic scans have been controllable hemorrhage, neurological deficits, subtotal re- used to identify characteristic patterns of bone erosion by section, and recurrence.12,13 A recent analysis suggests that JNA.4 Axial and coronal sections permit visualization of tumors that infiltrate the infratemporal fossa, the sphenoid septal deviation, orbital fissure expansion, hard palate dis- sinus, the base of the pterygoids, the cavernous sinus, the placement, and erosion of the medial pterygoid plate, the foramen lacerum, and the anterior fossa are most vulner- maxillary sinus, and the basisphenoid.9,11,14 Computed to- able to incomplete excision and recurrence.13 All tumors in mographic studies may also reveal anterior bowing of the our series extended into at least 3 of these regions. posterior wall of the maxillary antrum. Otherwise known Since 1981, several classification systems have been as the Holman-Miller sign, this finding is classically— proposed to exploit the predictive value of site extension. though not exclusively—associated with JNA.

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 For more than a decade, the diagnostic contribu- currence.24 This approach provides wide lateral expo- tions of computed tomography have been supple- sure to the internal carotid artery and the cavernous si- mented by the use of magnetic resonance imaging. This nus. However, tumor that is medial to the abducens nerve technique had a dramatic impact on the evaluation of in- (VI) in the cavernous sinus is inaccessible and deemed tracranial extension in our series. Magnetic resonance im- unresectable.24 The infratemporal fossa approach mini- aging offers improved soft tissue resolution combined with mizes facial scarring, but creates a depression when the multiplanar capabilities. These features enhance the evalu- temporalis muscle is used to reconstruct operative de- ation of intracranial landmarks and facilitate the differ- fects of the middle cranial fossa floor. This depression entiation of tumor from the surrounding mucosa, brain can cause facial and orthodontic asymmetry in addition tissue, and paranasal sinus contents.1 Moreover, they al- to cosmetic deficits.2 Finally, the infratemporal fossa low a detailed assessment of spatial relationships be- approach involves subtotal petrosectomy, which neces- tween the tumor and vital intracranial structures. This sitates sacrifice of the pneumatic middle ear cleft and was especially critical in our assessment of tumor exten- eustachian tube. The procedure is therefore associated sion into the cavernous sinus.2,15 with unilateral conductive hearing loss in nearly all The vascular nature of JNA has made embolization cases.24 an important component of preoperative management. In contrast, a combined transfacial and infratempo- Although this procedure is not associated with reduc- ral fossa approach permits access to the sphenoid sinus, tions in intraoperative hemorrhage in all reports, it has cavernous sinus, anterior skull base, and nasopharynx. been shown to limit blood loss in patients with the level We performed facial translocations in combination with of advanced disease observed in this series.16 Early ex- the lateral approach in all 5 patients. The estimated av- perience has underscored the importance of performing erage blood loss in our patients was more than 50% lower embolization in the immediate preoperative period (1-2 than the mean blood loss reported for the infratemporal days prior to surgery), as premature vessel occlusion can fossa approach (1120 mL vs 2400 mL). This reduction promote collateralization and limit the efficacy of the pro- in intraoperative hemorrhage suggests that the com- cedure.17 We also performed temporary balloon- bined approach may diminish the need for transfusion occlusion tests of the internal carotid artery in select cases through improved tumor exposure and vessel control. of cavernous sinus involvement. This additional step al- This approach also promotes complete resection by maxi- lowed us to assess the potential impact of vessel ligation mizing exposure to the nasopharynx, the sphenoid si- during resection.11 nus, the pterygopalatine fossa, the infratemporal fossa, Surgical resection has been the preferred treatment and both the medial and lateral aspects of the cavernous for extracranial JNA since 1955.11 However, the man- sinus. Complete tumor excision was achieved in all 5 cases agement of tumors with significant intracranial exten- in this series. There has been confirmed evidence of tu- sion continues to be a source of considerable contro- mor recurrence in 1 patient, and follow-up of a second versy. Numerous alternatives to surgery include hormonal patient has revealed an area suspicious for recurrence that treatment, chemotherapy, and external-beam radiation will continue to be monitored. therapy. Despite promising preliminary studies, hor- Our technique has been associated with minimal monal treatment has received little attention,18 and che- postoperative complications to date. The most signifi- motherapy has been largely abandoned due to associ- cant morbidity has been diminished sensation in the dis- ated toxic effects.8 Radiation has been advocated as both tribution of the second and third divisions of the trigemi- a primary treatment and as an adjunct to surgery. Sup- nal nerve. Although 2 patients report some loss of facial port for this approach is primarily derived from a single sensation 32 months after surgery, prior studies suggest study of 55 patients treated for JNA with moderate-dose that sensation may improve over time. Indeed, facial an- radiation (3000 rad or 3500 rad [30 or 35 Gy]).7 In con- esthesia associated with V2 and V3 resection through the trast to prior reports of inadequate tumor control,19,20 infratemporal fossa approach has been shown to im- this series demonstrated an 80% initial control rate with prove by 25% to 50% in the majority of affected patients limited long-term complications.7 However, the well- after an average follow-up of 2 years.24 Additional com- known risks of radiation-induced secondary malignan- plications in our series, including nasal crusting, serous cies (eg, sarcoma and thyroid carcinoma) and growth otitis media, and chronic sinusitis, have completely or arrest have fueled ongoing skepticism about the use of partially resolved with appropriate management. While this approach in a primarily pediatric population. the majority of complications have been treated conser- Surgical excision of extensive intracranial JNA has his- vatively, 1 patient required permanent pressure equal- torically been associated with mortality.11 In recent de- ization tube placement. cades, advanced approaches to the skull base, improved Until recently, the application of craniofacial sur- imaging techniques, and selective arterial embolization have gery in the pediatric population has been limited by con- fostered more aggressive surgical resections. Multiple ap- cerns that facial growth would be disrupted by osteoto- proaches to intracranial angiofibromas have been de- mies. These concerns have not been substantiated. A scribed, including an infratemporal fossa approach,21 a total recent review of craniofacial procedures in children aged maxillectomy,21 a midfacial degloving approach,22 and an 3.5 to 14 years for multiple intracranial pathologies re- extended transcranial approach.23 ported no evidence of arrest in facial skeletal growth in Among these techniques, the infratemporal fossa ap- patients 1 to 5 years after surgery.25 Likewise, none of proach has been reported to remove all gross tumor in the patients in our series have demonstrated facial asym- 80% of patients and to have the lowest rate (6%) of re- metry at long-term follow-up.

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©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 In summary, extensive angiofibromas that invade 5. Wylie JP, Slevin NJ, Johnson RJ. Intracranial juvenile nasopharyngeal angiofibroma. Clin Oncol (R Coll Radiol). 1998;10:330-333. intracranially and extend to the cavernous sinus may be 6. Gantz B, Seid AB, Weber RS. Nasopharyngeal angiofibroma. Head Neck. 1992; safely resected through a combined approach. The tem- 14:67-71. poral craniectomy provides excellent exposure to the 7. Cummings BJ, Blend R, Keane T, et al. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. Laryngoscope. 1984;94(pt 1):1599-1605. carotid artery, cavernous sinus, and superior orbital fis- 8. Goepfert H, Cangir A, Lee YY. Chemotherapy for aggressive juvenile nasopha- sure, while the transfacial approach allows for complete ryngeal angiofibroma. Arch Otolaryngol. 1985;111:285-289. 9. Neel BH, Whicker JH, Devine KD, Weiland LH. Juvenile angiofibroma: review of removal of tumor in the nasopharynx, sphenoid sinus, 120 cases. Am J Surg. 1973;126:547-556. and medial cavernous sinus. Successful outcomes are 10. Witt TR, Shah JP, Sternberg SS. Juvenile nasopharyngeal angiofibroma: a 30 ultimately determined by careful patient selection, im- year clinical review. Am J Surg. 1983;146:521-525. 11. Bremer JM, Neel HB, DeSanto LW, Jones GC. Angiofibroma: treatment trends in aging that clearly delineates the anatomic extent of tu- 150 patients during 40 years. Laryngoscope. 1986;96:1321-1329. mor invasion, safe preoperative embolization, and the 12. Radkowski D, McGill T, Healy GB, Ohlms L, Jones DT. Angiofibroma: changes in collaboration of experienced head and neck and skull– staging and treatment. Arch Otolaryngol Head Neck Surg. 1996;122:122-129. 13. Herman P, Lot G, Chapot R, Salvan D, Huy P. Long-term follow up of juvenile naso- based surgery teams. pharyngealangiofibromas:analysisofrecurrence.Laryngoscope.1999;109:141-147. 14. Lund VJ, Lloyd GA, Howard DJ. Juvenile angiofibroma—imaging techniques in diagnosis. Rhinology. 1989;27:179-185. Accepted for publication February 13, 2002. 15. Deschler DG, Kaplan MJ, Boles R. Treatment of large juvenile nasopharyngeal The study was presented at the Fifth International angiofibroma. Otolaryngol Head Neck Surg. 1992;106:278-284. Conference on Head and Neck Cancer, San Francisco, Calif, 16. Moulin G, Chagnaud C, Gras R, et al. Juvenile nasopharyngeal angiofibroma: com- parison of blood loss during removal in embolized group versus non-embolized July 31, 2000. group. Cardiovasc Intervent Radiol. 1995;18:158-161. Corresponding author and reprints: Randal S. Weber, 17. Bryan RN, Sessions RB, Horowitz BL. Radiographic management of juvenile an- MD, Hospital of the University of Pennsylvania, Depart- giofibromas. AJNR Am J Neuroradiol. 1981;2:157-166. 18. Gates GA, Rice DH, Koopman CF, Schuller DE. Flutamide-induced regression of ment of Otorhinolaryngology–Head and Neck Surgery, 5 Rav- angiofibroma. Laryngoscope. 1992;102:641-644. din Bldg, 3400 Spruce St, Philadelphia, PA 19104 (e-mail: 19. DeCarvalho M, Andrade J, Rapoport A, et al. The nasopharyngeal angiofibroma. Arch Otolaryngol. 1980;106:234-236. [email protected]). 20. Jereb B, Anggard A, Baryd I. Juvenile nasopharyngeal angiofibroma: a clinical study of 69 cases. Acta Radiol. 1970;9:302-310. 21. Andrews JC, Fisch U, Valavanis A, Aeppli U, Makek MS. The surgical manage- REFERENCES ment of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. Laryngoscope. 1989;99:429-437. 1. Batsakis JG. Tumors of the Head and Neck: Clinical and Pathological Consider- 22. Close SG, Schaefer SD, Mickey BE, Manning SC. Surgical management of na- ations. 2nd ed. Baltimore, Md: Williams & Wilkins; 1979:296-300. sopharyngeal angiofibroma involving the cavernous sinus. Arch Otolaryngol Head 2. Fagan JJ, Snyderman CH, Carrau RL, Janecka IP. Nasopharyngeal angiofibro- Neck Surg. 1989;15:1091-1095. mas: selecting a surgical approach. Head Neck. 1997;19:391-399. 23. Goel A, Bhayani R, Sheode J. Technique of extended transcranial approach for 3. Kamel RH. Transnasal endoscopic surgery in juvenile nasopharyngeal angiofi- massive nasopharyngeal angiofibroma. Br J Neurosurg. 1994;8:593-597. broma. J Laryngol Otol. 1996;110:962-968. 24. Zhang M, Garvis W, Linder T, Fisch U. Update on the infratemporal fossa ap- 4. Unganont K, Byers RM, Weber RS, Callendar DL, Wolf PE, Goepfert H. Juvenile proaches to nasopharyngeal angiofibroma. Laryngoscope. 1998;108:1717-1723. nasopharyngeal angiofibroma: an update of therapeutic management. Head Neck. 25. Lang DA, Neil-Dwyer G, Evans BT, Honeybul S. Craniofacial access in children. 1996;18:60-66. Acta Neurchir (Wien). 1998;140:33-40.

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